Lupus and Pregnancy
Philip Samuels, MDSteven G. Gabbe, MD
While lupus can have profound effects on both mother and child during pregnancy, most women with lupus can conceive, have a successful pregnancy and a healthy baby. Pregnancy does not seem to affect the long-term course of their lupus. It is very important to remember that later pregnancies may affect lupus differently in the same patient.
There appears to be an increased risk of lupus flares during pregnancy, especially during the days surrounding childbirth. There is also an increased risk of miscarriage in patients with lupus. The miscarriages associated with lupus often occur in the third and fourth months of pregnancy and may not necessarily have any relationship to how mild or severe the mother's other symptoms are at that time. Currently research into possible causes for recurrent miscarriages is focusing on the lupus anticoagulant and the anti-cardiolipin antibody. Such antibodies are found in the blood of approximately fifteen percent of patients with lupus.
It appears that therapy with prednisone, aspirin, heparin, or gamma globulin either alone or used together may help reduce the number of miscarriages in these patients. Pregnant patients with lupus may have an increased chance for a premature delivery. This is due both to spontaneous premature labor and to emergency Caesarean section.
The Caesarean section is done if the mother's condition is deteriorating or the baby is in jeopardy. Patients with lupus may also have an increase in the possibility of having a stillborn child. This is especially true in patients who have severe kidney involvement. Infants bom to mothers with lupus may have rashes on their face, scalp, and chest. These rashes usually disappear by one year of age.
Once in a great while, these babies may develop a congenital heart block. In these cases, the baby's heart beats at a slower rate than normal before birth, and continues to do so after delivery. This disorder is associated with the presence of the anti-Ro (SS-A) antibody in mothers with lupus. If this problem is recognized early, however, most of these babies will do well after birth. Most patients with lupus can have a successful pregnancy. There are certain factors that must be watched and there are certain measures the pregnant patient herself can take to help assure that she has a healthy baby. Most importantly, a patient's lupus should be in clinical remission before conception.
If possible she should try to wait six months after her last flare of lupus before conceiving. If her condition is stable, the patient greatly increases her chances for a normal pregnancy. Before conception, the patient should have blood tests performed for the presence of the anti-Ro antibody, the lupus anticoagulant, and the anti-cardiolipin antibody. If the anti-Ro antibody is present, the patient should be watched closely for the possible development of fetal heart block. If the lupus anticoagulant or anti-cardiolipin antibody is present, the patient should probably receive steroid therapy throughout her pregnancy.
The obstetrician and rheumatologist must work together as a team to help the patient throughout her pregnancy. The patient's blood pressure should be checked frequently. Her kidney function should be checked monthly. Ultrasound exams should be used frequently during pregnancy in a patient with lupus to make certain that the fetus (unborn baby) is growing properly. Ultrasound is a technique which uses high frequency sound waves to photograph the fetus. This test is painless and safe. Another safe test involves use of a fetal heart rate monitor to observe the patterns of the fetal heart rate for 20-40 minutes.
In the last months of pregnancy, this test may be done frequently to assess the condition of the unborn baby. Finally, frequent blood tests will be performed to make certain that there are no flares of the lupus. Although having blood tests performed is a nuisance and is somewhat uncomfortable, it is very important that the doctor watch the level of certain antibodies in the blood during late pregnancy to be assured that the mother's condition is stable.
Upon leaming that they are pregnant, many women react by immediately stopping all medications. This should not be done without consulting both the obstetrician and rheumatologist. Steroids are the mainstay of treating lupus, and they and most medications used to treat lupus cause no serious problems for the fetus. Patients should choose an obstetrician who is familiar with lupus and who feels comfortable treating pregnant patients with this illness.
=========================================================== This information is for"informational purposes" and is not meant to be used for medical diagnosis. Alwaysconsult your physician on matters such as this.
Tuesday, August 29, 2006
Sex and Lupus
Sex and Lupus
Mary P. Brassell, MA, CRRN
Many couples experience some type of sexual difficulty when one partner has a chronic disease such as lupus. Sexuality may be affected by disease symptoms, medications and/or psychological concerns. Disease symptoms of lupus which may influence sexual activity include: oral ulcers, vaginal ulcers, arthritis, Sjogren's syndrome, and Raynaud's phenomenon. Each of these problems will be discussed in detail. Oral ulcers occur in about 10-15% of people with lupus.
They can interfere with pleasant oral sensations. A prescription mouthwash with antibiotics and/or steroids may be necessary and can help heal the ulcers. Remember, they do not last forever! Vaginal ulcers are present in less than 5% of patients with lupus. They are rarely painful but when they are they can interfere with intercourse. A prescribed steroid cream or other medication can be used to treat them. Alternate forms of sexual expression can also be used until healing has taken place. Joint pains or arthritis that often accompany lupus may respond to warm baths, a few warming up exercises, and taking anti-inflammatory medication an hour before sexual activity. The vaginal dryness associated with Sjogren's syndrome responds well to the use of a water soluble lubricant which is absorbed and does not have to be removed. (Vaseline-like ointments should be avoided since they can encourage infection.) Water soluble lubricants (e.g. K-Y jelly) are available over the counter at drugstores or supermarkets.
People who have Raynaud's phenomenon have a condition in which blood vessels constrict (become narrower) when they are exposed to cold. It can be very painful. Raynaud's can cause fingers and toes to change color (first white, then blue, then red). Here are some solutions: Avoid having sex in an air conditioned room. Wear socks if your feet are sensitive to cold. Try a warm bath (not hot) prior to sexual activity as it will help open the blood vessels. During sex, blood pools in the genital area and less blood goes to the fingers and toes. Take the bottom position - it helps avoid the pressure on hands and feet that can further reduce blood flow. Medications can certainly influence sex life. Tranquilizers (antianxiety agents), anti-hypertensives (drugs that lower high blood pressure), and corticosteroids (prednisone) can affect both sexual desire (libido) and performance.
Some anti-hypertensives decrease libido in men and women as well as produce temporary impotence. It is best to discuss these drugs with your doctor, who may be able to prescribe a different blood pressure drug that won't interfere with sexual performance. A small percentage of men and women have experienced libido changes while on steroids. If you experience any of these changes, talk with your doctor. Psychological factors can also have a powerful influence on a satisfying sex life. If the person with lupus has developed low self esteem, unhappiness about the way their body looks, feelings of worthlessness, depression, fatigue, and feelings of inadequacy, the sexual relationship will certainly be affected. The patient may withdraw from his/her partner.
The healthy partner may feel that this withdrawal means rejection and the loss of affection and love. If communication does not occur, the relationship may be in serious jeopardy. Sexual pleasure is an important aspect of any relationship. Some studies have reported that arthritis patients are free from joint pain for up to six hours after intercourse. Sex, therefore, seems to be therapeutic. Even if intercourse is not possible, other forms of sexual expression such as cuddling, holding, stroking, kissing, and closeness are all manifestations of sexual affection that can help reinforce your sense of self-worth and desirability.
Many publications contain various suggestions for different methods of sexual expression. The Arthritis Foundation has a publication titled "Living and Loving with Arthritis". It costs less than a dollar and can be obtained from the local branch of the Arthritis Foundation. This booklet contains information about sexual expression, illustrations of comfortable positions to assume during sexual activity, and some sound advice about coping with sexual challenges. Suggestions for improving a sexual relationship are offered. Sexuality is a part of life. It is a normal and usual activity of daily living. Whether sexual problems are physical or emotional or both, willingness by botb partners to discuss and search for solutions to sexual problems is essential.
Help in reaching solutions can be sought from a physician, from nurses in the field of arthritis and lupus, from psychologists, and/or from sex therapists. infon-nation is readily available at low cost. All that is required is that the patient and partner find the necessary person or publication. Sexuality is best thought of as another form of communication that helps couples enjoy each other and deepen their intimacy in a loving relationship. Sometimes if this communication does not happen, other parts of the relationship will suffer. Solutions can be achieved with a little effort by both patient and partner, but there must be an effort!
=========================================================== This information is for"informational purposes" and is not meant to be used for medical diagnosis. Alwaysconsult your physician on matters such as this.
Mary P. Brassell, MA, CRRN
Many couples experience some type of sexual difficulty when one partner has a chronic disease such as lupus. Sexuality may be affected by disease symptoms, medications and/or psychological concerns. Disease symptoms of lupus which may influence sexual activity include: oral ulcers, vaginal ulcers, arthritis, Sjogren's syndrome, and Raynaud's phenomenon. Each of these problems will be discussed in detail. Oral ulcers occur in about 10-15% of people with lupus.
They can interfere with pleasant oral sensations. A prescription mouthwash with antibiotics and/or steroids may be necessary and can help heal the ulcers. Remember, they do not last forever! Vaginal ulcers are present in less than 5% of patients with lupus. They are rarely painful but when they are they can interfere with intercourse. A prescribed steroid cream or other medication can be used to treat them. Alternate forms of sexual expression can also be used until healing has taken place. Joint pains or arthritis that often accompany lupus may respond to warm baths, a few warming up exercises, and taking anti-inflammatory medication an hour before sexual activity. The vaginal dryness associated with Sjogren's syndrome responds well to the use of a water soluble lubricant which is absorbed and does not have to be removed. (Vaseline-like ointments should be avoided since they can encourage infection.) Water soluble lubricants (e.g. K-Y jelly) are available over the counter at drugstores or supermarkets.
People who have Raynaud's phenomenon have a condition in which blood vessels constrict (become narrower) when they are exposed to cold. It can be very painful. Raynaud's can cause fingers and toes to change color (first white, then blue, then red). Here are some solutions: Avoid having sex in an air conditioned room. Wear socks if your feet are sensitive to cold. Try a warm bath (not hot) prior to sexual activity as it will help open the blood vessels. During sex, blood pools in the genital area and less blood goes to the fingers and toes. Take the bottom position - it helps avoid the pressure on hands and feet that can further reduce blood flow. Medications can certainly influence sex life. Tranquilizers (antianxiety agents), anti-hypertensives (drugs that lower high blood pressure), and corticosteroids (prednisone) can affect both sexual desire (libido) and performance.
Some anti-hypertensives decrease libido in men and women as well as produce temporary impotence. It is best to discuss these drugs with your doctor, who may be able to prescribe a different blood pressure drug that won't interfere with sexual performance. A small percentage of men and women have experienced libido changes while on steroids. If you experience any of these changes, talk with your doctor. Psychological factors can also have a powerful influence on a satisfying sex life. If the person with lupus has developed low self esteem, unhappiness about the way their body looks, feelings of worthlessness, depression, fatigue, and feelings of inadequacy, the sexual relationship will certainly be affected. The patient may withdraw from his/her partner.
The healthy partner may feel that this withdrawal means rejection and the loss of affection and love. If communication does not occur, the relationship may be in serious jeopardy. Sexual pleasure is an important aspect of any relationship. Some studies have reported that arthritis patients are free from joint pain for up to six hours after intercourse. Sex, therefore, seems to be therapeutic. Even if intercourse is not possible, other forms of sexual expression such as cuddling, holding, stroking, kissing, and closeness are all manifestations of sexual affection that can help reinforce your sense of self-worth and desirability.
Many publications contain various suggestions for different methods of sexual expression. The Arthritis Foundation has a publication titled "Living and Loving with Arthritis". It costs less than a dollar and can be obtained from the local branch of the Arthritis Foundation. This booklet contains information about sexual expression, illustrations of comfortable positions to assume during sexual activity, and some sound advice about coping with sexual challenges. Suggestions for improving a sexual relationship are offered. Sexuality is a part of life. It is a normal and usual activity of daily living. Whether sexual problems are physical or emotional or both, willingness by botb partners to discuss and search for solutions to sexual problems is essential.
Help in reaching solutions can be sought from a physician, from nurses in the field of arthritis and lupus, from psychologists, and/or from sex therapists. infon-nation is readily available at low cost. All that is required is that the patient and partner find the necessary person or publication. Sexuality is best thought of as another form of communication that helps couples enjoy each other and deepen their intimacy in a loving relationship. Sometimes if this communication does not happen, other parts of the relationship will suffer. Solutions can be achieved with a little effort by both patient and partner, but there must be an effort!
=========================================================== This information is for"informational purposes" and is not meant to be used for medical diagnosis. Alwaysconsult your physician on matters such as this.
Tuesday, August 01, 2006
Rehabilitation in Lupus
Rehabilitation in Lupus
Bertram Greenspun, DO
Rehabilitation medicine is the field of medicine that concerns itself not only with how the disease process has affected the individual but also with how the disease has changed the person's ability to maintain the lifestyle and roles in life which they had before the disease started.
The rehabilitation team concentrates on how to improve the person's functioning and independence to the highest possible level. Systemic Lupus Erythematosus (SLE) at times presents problems that can be helped by an individualized rehabilitation program. Among the body systems that can be affected by lupus (and may be helped by rehabilitation) are the central nervous system (CNS), the musculoskeletal system (muscles, bones and joints), and the vascular system (blood vessels).
The CNS can be involved in a variety of ways. Patients who develop stroke-like syndromes or problems of the spinal cord usually require an in-patient program in a comprehensive rehabilitation center. These problems often result in weakness of one or both arms or legs. Weakness can be so profound that it may result in paralysis. Under such circumstances, passive range of motion (ROM) exercises should be started by the physical therapist (PT). Family and friends may also be instructed by the physical therapist to perform ROM exercises for the patient. These exercises are done without the patient having to do any of the work. Since some degree of strength almost always returns when the stroke or the spinal cord damage resolves, it is vital not to allow contractures to develop.
Contractures happen when the joints are allowed to stiffen to the point where they will be unable to bend. Should this occur, even if muscle power returns it will do no good unless the stiffness is reduced or eliminated. This can take a very long time and even require surgery. It is much wiser and easier to prevent contractures in the first place than it is to correct them after the fact. As the patient improves, he/she can move the joint along with the physical therapist. Then the exercise is known as active-assistive ROM. When the patient can move the joint through the entire ROM, he/she no longer needs the assistance of another person and is instructed on how to do regular, active ROM exercises independently.
In addition to ROM exercises, strengthening exercises can be started under proper supervision as soon as is medically possible. The strengthening program should begin at a low intensity that is well within the capability of the patient. It is increased only gradually and slowly. The patient must learn to "listen" to his/her body. This means that if muscle pain or fatigue lasts for more than an hour to an hour and a half after a session, the level of exercise intensity should be temporarily reduced. if the discomfort or fatigue continues after each session the physician who prescribed the program should be notified.
Too much exercise, done too quickly, can easily make the situation worse. If the weakness is associated with acute inflammatory joint pain, isometric exercises can be helpful. Isometric exercises involve contracting the muscles without moving the involved joint. In this way strength can be maintained without triggering the intense pain associated with moving an acutely inflamed joint. Since isometric exercises can cause the blood pressure to rise they should be performed cautiously and without holding the breath, especially in the individual with hypertension. Almost all patients with lupus will, at some point in the disease process, have joint pain. There is often associated morning stiffness and joint swelling. Tenderness and painful movement are common. Deformities may result. Most of these problems can be dealt with on an out-patient basis.
An early morning shower will frequently shorten the duration of morning stiffness. Moist heat is quite helpful for most joint problems and is used prior to ROM exercises. This can be provided by using an inexpensive hydrocollator pack (a wan-n, moistened, sand-filled canvas bag). The moist heat helps to prevent or reduce joint pain caused by motion. For chronic pain, deep heat, such as is provided by ultrasound, can be applied by a PT.
For acute, severe joint pain, ice is most helpful. Bed rest is occasionally prescribed for widespread severe pain. In such cases, it is especially important to properly position the patient in bed to avoid the development of contractures of the joints. One of the most common mistakes is to put pillows under the knees. This forces the knees into a bent position and, along with them, the hips. To imagine the difficulties this can cause, try walking with your hips and knees bent. Both musculoskeletal and CNS manifestations can lead to problems in performing the activities of daily living (ADL) such as dressing, bathing, toileting, etc. An occupational therapist (OT) can be most helpful in suggesting ways to solve these problems.
The OT can provide tools such as button hooks and long-handled sponges to make these activities easier, and can fit splints to allow painful hands and arms to rest while keeping them positioned properly. The OT can educate the patient in joint conservation techniques (ways to protect the joints yet still get the task done). The OT can also aid in the selection of devices to make living at home less difficult (tub benches, raised toilet seats, stair glides, etc.) and can help evaluate the home environment from the point of view of the patient's safety. If mobility has become a problem as a result of joint or CNS involvement, the PT teaches transfer techniques (bed to chair, sit to stand,etc.) and ambulation training, including going up and down stairs. The physical therapist also teaches patients to make proper use of assistive devices such as canes and crutches and helps the patient to adapt to leg braces and splints.
As a result of lupus vasculitis (inflammation of the blood vessels), patients may develop ulcerations of the skin. Special exercises can then be used to help improve circulation and whirlpool treatments can be given to clean the ulcers by removing dead tissue. Rarely, as a result of ulcerations of the feet or ankles, or as a result of blood clots in the major leg arteries, amputation of part of a leg may become necessary.
There has been dramatic improvement in prosthetic (artificial) feet in the last three to four years, allowing many more normal activities such as running and jumping, and materials are now available that result in a much lighter limb. In each state, there is a government office concerned with helping individuals with disabilities obtain training or education so that they can return to gainful employment if their disability prevents them from resuming their previous vocation. This office is usually known as the Office of Vocational Rehabilitation. Social workers can be most helpful in directing people with lupus-related disabilities to the appropriate office or case worker.
The social worker is familiar with the many resources that can help the person with lupus obtain a wide variety of services. Social workers are available in medical clinics, hospitals and social agencies and are members of all rehabilitation teams. If lupus patients have to be hospitalized in a rehabilitation center they will encounter physicians responsible for directing their rehabilitative care. Such physicians are specialists in Physical Medicine and Rehabilitation and are called "physiatrists". In addition to the PT, the OT and the social worker, the rehabilitation team usually includes a number of other professionals. These are a rehabilitation nurse (who has had special training in working with patients disabled by different diseases) a psychiatrist and/or psychologist (to help with the emotional problems that may be present), a recreational therapist (to get patients involved in activities that they may have enjoyed previously or to introduce new interesting activities that make them aware that life can still be enjoyed) and a speech therapist (to help in problems with language or with swallowing difficulties). As well as working with the patient, all members of the rehabilitation team work with the patient's family and friends so that they know what to do and what not to do, when the patient goes home. In summary, the primary purpose of any rehabilitation program is to improve the functional independence of the individual.
Most of the patients requiring a rehabilitation program can be treated in an out-patient setting or at home. Only a small minority will require an intensive in-patient program. While no patients are cured, almost all can be helped.
=========================================================== This information is for"informational purposes" and is not meant to be used for medical diagnosis. Alwaysconsult your physician on matters such as this.
Bertram Greenspun, DO
Rehabilitation medicine is the field of medicine that concerns itself not only with how the disease process has affected the individual but also with how the disease has changed the person's ability to maintain the lifestyle and roles in life which they had before the disease started.
The rehabilitation team concentrates on how to improve the person's functioning and independence to the highest possible level. Systemic Lupus Erythematosus (SLE) at times presents problems that can be helped by an individualized rehabilitation program. Among the body systems that can be affected by lupus (and may be helped by rehabilitation) are the central nervous system (CNS), the musculoskeletal system (muscles, bones and joints), and the vascular system (blood vessels).
The CNS can be involved in a variety of ways. Patients who develop stroke-like syndromes or problems of the spinal cord usually require an in-patient program in a comprehensive rehabilitation center. These problems often result in weakness of one or both arms or legs. Weakness can be so profound that it may result in paralysis. Under such circumstances, passive range of motion (ROM) exercises should be started by the physical therapist (PT). Family and friends may also be instructed by the physical therapist to perform ROM exercises for the patient. These exercises are done without the patient having to do any of the work. Since some degree of strength almost always returns when the stroke or the spinal cord damage resolves, it is vital not to allow contractures to develop.
Contractures happen when the joints are allowed to stiffen to the point where they will be unable to bend. Should this occur, even if muscle power returns it will do no good unless the stiffness is reduced or eliminated. This can take a very long time and even require surgery. It is much wiser and easier to prevent contractures in the first place than it is to correct them after the fact. As the patient improves, he/she can move the joint along with the physical therapist. Then the exercise is known as active-assistive ROM. When the patient can move the joint through the entire ROM, he/she no longer needs the assistance of another person and is instructed on how to do regular, active ROM exercises independently.
In addition to ROM exercises, strengthening exercises can be started under proper supervision as soon as is medically possible. The strengthening program should begin at a low intensity that is well within the capability of the patient. It is increased only gradually and slowly. The patient must learn to "listen" to his/her body. This means that if muscle pain or fatigue lasts for more than an hour to an hour and a half after a session, the level of exercise intensity should be temporarily reduced. if the discomfort or fatigue continues after each session the physician who prescribed the program should be notified.
Too much exercise, done too quickly, can easily make the situation worse. If the weakness is associated with acute inflammatory joint pain, isometric exercises can be helpful. Isometric exercises involve contracting the muscles without moving the involved joint. In this way strength can be maintained without triggering the intense pain associated with moving an acutely inflamed joint. Since isometric exercises can cause the blood pressure to rise they should be performed cautiously and without holding the breath, especially in the individual with hypertension. Almost all patients with lupus will, at some point in the disease process, have joint pain. There is often associated morning stiffness and joint swelling. Tenderness and painful movement are common. Deformities may result. Most of these problems can be dealt with on an out-patient basis.
An early morning shower will frequently shorten the duration of morning stiffness. Moist heat is quite helpful for most joint problems and is used prior to ROM exercises. This can be provided by using an inexpensive hydrocollator pack (a wan-n, moistened, sand-filled canvas bag). The moist heat helps to prevent or reduce joint pain caused by motion. For chronic pain, deep heat, such as is provided by ultrasound, can be applied by a PT.
For acute, severe joint pain, ice is most helpful. Bed rest is occasionally prescribed for widespread severe pain. In such cases, it is especially important to properly position the patient in bed to avoid the development of contractures of the joints. One of the most common mistakes is to put pillows under the knees. This forces the knees into a bent position and, along with them, the hips. To imagine the difficulties this can cause, try walking with your hips and knees bent. Both musculoskeletal and CNS manifestations can lead to problems in performing the activities of daily living (ADL) such as dressing, bathing, toileting, etc. An occupational therapist (OT) can be most helpful in suggesting ways to solve these problems.
The OT can provide tools such as button hooks and long-handled sponges to make these activities easier, and can fit splints to allow painful hands and arms to rest while keeping them positioned properly. The OT can educate the patient in joint conservation techniques (ways to protect the joints yet still get the task done). The OT can also aid in the selection of devices to make living at home less difficult (tub benches, raised toilet seats, stair glides, etc.) and can help evaluate the home environment from the point of view of the patient's safety. If mobility has become a problem as a result of joint or CNS involvement, the PT teaches transfer techniques (bed to chair, sit to stand,etc.) and ambulation training, including going up and down stairs. The physical therapist also teaches patients to make proper use of assistive devices such as canes and crutches and helps the patient to adapt to leg braces and splints.
As a result of lupus vasculitis (inflammation of the blood vessels), patients may develop ulcerations of the skin. Special exercises can then be used to help improve circulation and whirlpool treatments can be given to clean the ulcers by removing dead tissue. Rarely, as a result of ulcerations of the feet or ankles, or as a result of blood clots in the major leg arteries, amputation of part of a leg may become necessary.
There has been dramatic improvement in prosthetic (artificial) feet in the last three to four years, allowing many more normal activities such as running and jumping, and materials are now available that result in a much lighter limb. In each state, there is a government office concerned with helping individuals with disabilities obtain training or education so that they can return to gainful employment if their disability prevents them from resuming their previous vocation. This office is usually known as the Office of Vocational Rehabilitation. Social workers can be most helpful in directing people with lupus-related disabilities to the appropriate office or case worker.
The social worker is familiar with the many resources that can help the person with lupus obtain a wide variety of services. Social workers are available in medical clinics, hospitals and social agencies and are members of all rehabilitation teams. If lupus patients have to be hospitalized in a rehabilitation center they will encounter physicians responsible for directing their rehabilitative care. Such physicians are specialists in Physical Medicine and Rehabilitation and are called "physiatrists". In addition to the PT, the OT and the social worker, the rehabilitation team usually includes a number of other professionals. These are a rehabilitation nurse (who has had special training in working with patients disabled by different diseases) a psychiatrist and/or psychologist (to help with the emotional problems that may be present), a recreational therapist (to get patients involved in activities that they may have enjoyed previously or to introduce new interesting activities that make them aware that life can still be enjoyed) and a speech therapist (to help in problems with language or with swallowing difficulties). As well as working with the patient, all members of the rehabilitation team work with the patient's family and friends so that they know what to do and what not to do, when the patient goes home. In summary, the primary purpose of any rehabilitation program is to improve the functional independence of the individual.
Most of the patients requiring a rehabilitation program can be treated in an out-patient setting or at home. Only a small minority will require an intensive in-patient program. While no patients are cured, almost all can be helped.
=========================================================== This information is for"informational purposes" and is not meant to be used for medical diagnosis. Alwaysconsult your physician on matters such as this.
Lupus in Childhood
Lupus in Childhood
Phyllis Slutsky, MEd, RNBarbara E. Ostrov, MD
About 20-25% of all lupus occurs in children. While the exact number of cases is not known, it is not a rare disorder. Girls have lupus about three to seven times more often than boys. Research is being done to study many possible causes of lupus.
Several studies have shown that there is a tendency for lupus to run in families. It is known that there are certain inherited factors called HLA types which make a person more likely to develop lupus. (See Chapter 4.) There is also an inherited lack of immune system proteins (serum complements or immunoglobulins) which increase the risk for developing lupus in some people.
When a susceptible person is exposed to a "trigger" such as a virus or other environmental factor, lupus can develop. A particular kind of lupus may develop in infants of mothers who have SLE. This is called "neonatal lupus" and is associated with an anti-Ro antibody in the mother. An infant with neonatal lupus may have a rash on the face, scalp, or chest. Very rarely, these infants may also be born with an abnormally slow heart rhythm (congenital heart block).
During the first six months to one year, the rash disappears but the slow heart rhythm can be serious and may require the placement of a pacemaker. Systemic lupus erythematosus in childhood can range from mild to severe and affects each individual differently. The usual course of lupus includes periods of "flare-ups" (when symptoms are active) and remissions (when symptoms go away).
Stressors such as infections (which may be more frequent in childhood) and hormonal changes may lead to a flare of symptoms. This may explain why lupus is more common in girls after puberty and may flare up during menstruation and pregnancy. In children, as in adults, sun exposure can bring on symptoms of lupus. Serious complications of lupus are most common when organs such as kidneys, heart or the central nervous system are involved.
The most common time to find out what body systems are involved is at the time that lupus is first diagnosed. As time goes on, the likelihood decreases that new body systems will become involved. Diagnosing lupus involves a thorough physical examination and often many laboratory tests.
There are guidelines which have been developed by the American College of Rheumatology which are used to diagnose lupus. (See Table 1) Four of their eleven selected symptoms and/or laboratory tests (criteria) must be present to make a definite diagnosis. Symptoms of lupus may start in a variety of ways.
A child may be very ill or have only mild symptoms. The most common symptoms in children are rash, fever, fatigue, and joint pain and swelling. Decreased appetite and weight loss are particularly common in children during the active phase of the disease. Inflammation of the kidneys (nephritis), inflammation of the heart covering (pericarditis), enlargement of the liver and spleen, and a low blood count (cytopenia) may be more frequent in children than in adults. Many children have rashes that can appear on the face or anywhere on the body. Ulcers of the mouth and nose are common in children with lupus. Hair loss (alopecia) may range from gradual thinning and change in texture, to loss of large amounts of hair when combing.
Central nervous system symptoms (affecting the brain and spinal column) can occur and may cause headaches, seizures, or a change in memory or thinking ability. Visual changes can also occur, making it important to have frequent checks by an eye doctor. Changes in mood or behavior can occur in children with lupus. These can be an understandable reaction to having an illness. Feelings of sadness or irritability do not necessarily mean there is central nervous system involvement. However, it is important to keep a careful watch on a child's behavior to decide if these things are caused by the lupus or are due to the child's reaction to the illness.
Diagnostic tests such as spinal taps, EEGS, and brain x-rays and scans can be helpful in deciding what is caused by lupus and what is due to psychosocial causes. The kidneys can be affected by lupus. A urinalysis may reveal problems which can then be further investigated with other tests. It is common to collect urine for 24 hours to do further studies. A kidney biopsy is sometimes necessary to determine how seriously the kidney is affected.
This test is used as a guideline for treatment and for monitoring changes over time. The treatment of lupus depends on what part of the body is affected. Being careful about sun exposure is important for all children. Planning the child's schedule to avoid exposure during the peak time of 12:00-3:00 P.M. each day is recommended. Children should be included in working out the schedule so that they understand the importance of any restrictions and will be more willing to cooperate. Another important aspect of treatment is to be alert for any signs of a developing infection.
Families should be aware that fever and increased tiredness or shortness of breath could be a sign of either a "flare-up" of lupus or possibly an infection. The doctor may need to evaluate the child when fever develops. There are a variety of medications used to treat lupus. Many children are treated with a non-steroidal anti-inflammatory drug (NSAID) which helps reduce inflammation and joint pain. Antimalarial drugs such as Plaquenil are sometimes used to treat the skin rashes of lupus. Steroids are sometimes necessary to treat lupus. The goal when using steroids is to use the lowest possible amount needed.
As symptoms improve, smaller and smaller doses are gradually given until the medication can be stopped altogether. When high doses of steroids are used over a long period of time, side effects can occur such as eye problems, and a decrease in the growth of the child. Giving steroids every other day helps to lower the chances of harmful side effects. Some other drugs used mainly when kidneys are affected by lupus are Cytoxan and Imuran. These work to control the reaction of a child's immune system so that it causes less harmful effects on the body. Recent studies have shown that these drugs can be used safely in children and can control the more serious effects of lupus on internal organs. A chronic illness like lupus will of course have a large effect on a child's life.
Often a child misses school during "flares". It is important to make contact with the school as soon as possible and stay in contact during the absent period. A school counselor or nurse can make arrangements to help the child. Some children may need home or hospital tutoring while others may be able to do make-up work on their own. This depends on how sick they are and how long they will be out of school. Because lupus symptoms come and go, arrangements may need to be made before a child is sick so that tutoring can start right away if needed. Many rheumatology centers that treat lupus have a health care team that includes a social worker and a nurse who can help the family with getting services needed for their child. Often children with lupus (like those with other chronic conditions) should have an Individualized Education Plan (IEP) .
This is an evaluation of all of the child's educational, psychosocial and physical needs which is done by a "study team" in each school district. The IEP identifies what each child needs in order to function well at school. Every child in the USA is entitled to this plan due to a government law (PL#94 -142). Children with lupus are also entitled to vocational planning services in order to prepare for school or job training for their future employment.
Each state has an Office of Vocational Rehabilitation (OVR) which offers career counseling that takes into account a child's physical abilities. Any illness in a family member is likely to cause reactions in other members of the family. These can help or hurt successful coping. There is often an increased burden on the family when caring for a sick child. This can also mean lost work time for parents and added financial problems due to medical expenses. Studies have been done to measure the effects on a family of a child with chronic illness. The families that coped best were those that did not place their child in a sick role, and so did not limit the child's activities in daily life.
Families based around a good quality marriage, and with good support from family, friends and/or religious groups cope better with a chronic illness. Support groups for parents of children with lupus exist through the AJAO (American juvenile Arthritis Organization) which is a part of the Arthritis Foundation. The large number of teenage and young adult women with lupus raises some special concerns. A major problem for teenagers is the change in appearance, often on the face, caused by lupus.
The characteristic rash and puffiness of lupus, or the acne caused by steroids cause the child to wonder, "How do I look to the world?" It is important to discuss this as openly as possible with the teenager, as these issues are a major source of stress and worry. Concerns about sexuality and pregnancy are also often uppermost in teenagers' minds when they become ill. Standard oral contraceptives which contain the hormone estrogen may cause an increase in symptoms of lupus and therefore are not recommended.
Sometimes the mini-pill, which contains only the hormone progesterone, is safe in lupus patients. Diaphragms and condoms are fine, and all of these options should be reviewed and discussed. It is recommended that a woman not become pregnant during an active period of lupus. Lupus has not been found to affect the ability to get pregnant, but miscarriages are more common when the disease is active. It is very possible to become pregnant and have a healthy baby, but careful planning with the doctor for the best time to get pregnant is advised. There are many obstetricians available who specialize in "high risk" pregnancies. Because of the many issues and adjustments necessary for a teenager with lupus, individual counseling may be necessary and can be helpful to some adolescents.
Support groups are an ideal way for teens to get the peer support and acceptance they need. Teens who attend these groups report a decrease in feelings of isolation and are better able to accept their illness. There has been a remarkable improvement in the prognosis of children with lupus during the past thirty years. This has happened due to improvements in diagnosis and treatment. Most importantly, children and their families need to seek careful and competent medical treatment and to cultivate the support of their health care team, their school and their community.
=========================================================== This information is for"informational purposes" and is not meant to be used for medical diagnosis. Alwaysconsult your physician on matters such as this.
Phyllis Slutsky, MEd, RNBarbara E. Ostrov, MD
About 20-25% of all lupus occurs in children. While the exact number of cases is not known, it is not a rare disorder. Girls have lupus about three to seven times more often than boys. Research is being done to study many possible causes of lupus.
Several studies have shown that there is a tendency for lupus to run in families. It is known that there are certain inherited factors called HLA types which make a person more likely to develop lupus. (See Chapter 4.) There is also an inherited lack of immune system proteins (serum complements or immunoglobulins) which increase the risk for developing lupus in some people.
When a susceptible person is exposed to a "trigger" such as a virus or other environmental factor, lupus can develop. A particular kind of lupus may develop in infants of mothers who have SLE. This is called "neonatal lupus" and is associated with an anti-Ro antibody in the mother. An infant with neonatal lupus may have a rash on the face, scalp, or chest. Very rarely, these infants may also be born with an abnormally slow heart rhythm (congenital heart block).
During the first six months to one year, the rash disappears but the slow heart rhythm can be serious and may require the placement of a pacemaker. Systemic lupus erythematosus in childhood can range from mild to severe and affects each individual differently. The usual course of lupus includes periods of "flare-ups" (when symptoms are active) and remissions (when symptoms go away).
Stressors such as infections (which may be more frequent in childhood) and hormonal changes may lead to a flare of symptoms. This may explain why lupus is more common in girls after puberty and may flare up during menstruation and pregnancy. In children, as in adults, sun exposure can bring on symptoms of lupus. Serious complications of lupus are most common when organs such as kidneys, heart or the central nervous system are involved.
The most common time to find out what body systems are involved is at the time that lupus is first diagnosed. As time goes on, the likelihood decreases that new body systems will become involved. Diagnosing lupus involves a thorough physical examination and often many laboratory tests.
There are guidelines which have been developed by the American College of Rheumatology which are used to diagnose lupus. (See Table 1) Four of their eleven selected symptoms and/or laboratory tests (criteria) must be present to make a definite diagnosis. Symptoms of lupus may start in a variety of ways.
A child may be very ill or have only mild symptoms. The most common symptoms in children are rash, fever, fatigue, and joint pain and swelling. Decreased appetite and weight loss are particularly common in children during the active phase of the disease. Inflammation of the kidneys (nephritis), inflammation of the heart covering (pericarditis), enlargement of the liver and spleen, and a low blood count (cytopenia) may be more frequent in children than in adults. Many children have rashes that can appear on the face or anywhere on the body. Ulcers of the mouth and nose are common in children with lupus. Hair loss (alopecia) may range from gradual thinning and change in texture, to loss of large amounts of hair when combing.
Central nervous system symptoms (affecting the brain and spinal column) can occur and may cause headaches, seizures, or a change in memory or thinking ability. Visual changes can also occur, making it important to have frequent checks by an eye doctor. Changes in mood or behavior can occur in children with lupus. These can be an understandable reaction to having an illness. Feelings of sadness or irritability do not necessarily mean there is central nervous system involvement. However, it is important to keep a careful watch on a child's behavior to decide if these things are caused by the lupus or are due to the child's reaction to the illness.
Diagnostic tests such as spinal taps, EEGS, and brain x-rays and scans can be helpful in deciding what is caused by lupus and what is due to psychosocial causes. The kidneys can be affected by lupus. A urinalysis may reveal problems which can then be further investigated with other tests. It is common to collect urine for 24 hours to do further studies. A kidney biopsy is sometimes necessary to determine how seriously the kidney is affected.
This test is used as a guideline for treatment and for monitoring changes over time. The treatment of lupus depends on what part of the body is affected. Being careful about sun exposure is important for all children. Planning the child's schedule to avoid exposure during the peak time of 12:00-3:00 P.M. each day is recommended. Children should be included in working out the schedule so that they understand the importance of any restrictions and will be more willing to cooperate. Another important aspect of treatment is to be alert for any signs of a developing infection.
Families should be aware that fever and increased tiredness or shortness of breath could be a sign of either a "flare-up" of lupus or possibly an infection. The doctor may need to evaluate the child when fever develops. There are a variety of medications used to treat lupus. Many children are treated with a non-steroidal anti-inflammatory drug (NSAID) which helps reduce inflammation and joint pain. Antimalarial drugs such as Plaquenil are sometimes used to treat the skin rashes of lupus. Steroids are sometimes necessary to treat lupus. The goal when using steroids is to use the lowest possible amount needed.
As symptoms improve, smaller and smaller doses are gradually given until the medication can be stopped altogether. When high doses of steroids are used over a long period of time, side effects can occur such as eye problems, and a decrease in the growth of the child. Giving steroids every other day helps to lower the chances of harmful side effects. Some other drugs used mainly when kidneys are affected by lupus are Cytoxan and Imuran. These work to control the reaction of a child's immune system so that it causes less harmful effects on the body. Recent studies have shown that these drugs can be used safely in children and can control the more serious effects of lupus on internal organs. A chronic illness like lupus will of course have a large effect on a child's life.
Often a child misses school during "flares". It is important to make contact with the school as soon as possible and stay in contact during the absent period. A school counselor or nurse can make arrangements to help the child. Some children may need home or hospital tutoring while others may be able to do make-up work on their own. This depends on how sick they are and how long they will be out of school. Because lupus symptoms come and go, arrangements may need to be made before a child is sick so that tutoring can start right away if needed. Many rheumatology centers that treat lupus have a health care team that includes a social worker and a nurse who can help the family with getting services needed for their child. Often children with lupus (like those with other chronic conditions) should have an Individualized Education Plan (IEP) .
This is an evaluation of all of the child's educational, psychosocial and physical needs which is done by a "study team" in each school district. The IEP identifies what each child needs in order to function well at school. Every child in the USA is entitled to this plan due to a government law (PL#94 -142). Children with lupus are also entitled to vocational planning services in order to prepare for school or job training for their future employment.
Each state has an Office of Vocational Rehabilitation (OVR) which offers career counseling that takes into account a child's physical abilities. Any illness in a family member is likely to cause reactions in other members of the family. These can help or hurt successful coping. There is often an increased burden on the family when caring for a sick child. This can also mean lost work time for parents and added financial problems due to medical expenses. Studies have been done to measure the effects on a family of a child with chronic illness. The families that coped best were those that did not place their child in a sick role, and so did not limit the child's activities in daily life.
Families based around a good quality marriage, and with good support from family, friends and/or religious groups cope better with a chronic illness. Support groups for parents of children with lupus exist through the AJAO (American juvenile Arthritis Organization) which is a part of the Arthritis Foundation. The large number of teenage and young adult women with lupus raises some special concerns. A major problem for teenagers is the change in appearance, often on the face, caused by lupus.
The characteristic rash and puffiness of lupus, or the acne caused by steroids cause the child to wonder, "How do I look to the world?" It is important to discuss this as openly as possible with the teenager, as these issues are a major source of stress and worry. Concerns about sexuality and pregnancy are also often uppermost in teenagers' minds when they become ill. Standard oral contraceptives which contain the hormone estrogen may cause an increase in symptoms of lupus and therefore are not recommended.
Sometimes the mini-pill, which contains only the hormone progesterone, is safe in lupus patients. Diaphragms and condoms are fine, and all of these options should be reviewed and discussed. It is recommended that a woman not become pregnant during an active period of lupus. Lupus has not been found to affect the ability to get pregnant, but miscarriages are more common when the disease is active. It is very possible to become pregnant and have a healthy baby, but careful planning with the doctor for the best time to get pregnant is advised. There are many obstetricians available who specialize in "high risk" pregnancies. Because of the many issues and adjustments necessary for a teenager with lupus, individual counseling may be necessary and can be helpful to some adolescents.
Support groups are an ideal way for teens to get the peer support and acceptance they need. Teens who attend these groups report a decrease in feelings of isolation and are better able to accept their illness. There has been a remarkable improvement in the prognosis of children with lupus during the past thirty years. This has happened due to improvements in diagnosis and treatment. Most importantly, children and their families need to seek careful and competent medical treatment and to cultivate the support of their health care team, their school and their community.
=========================================================== This information is for"informational purposes" and is not meant to be used for medical diagnosis. Alwaysconsult your physician on matters such as this.
Friday, June 09, 2006
Dental Problems and the Lupus Patient
Dental Problems and the Lupus Patient
Jay A. Denbo, DDS
COMMUNICATION, SELF-EXAMINATION, and PREVENTION are the keys to controlling dental problems for the lupus patient. Lupus patients have specific dental problems because of the disease process and the medications needed to control lupus symptoms.
Communication
Communication between patient, physician, and dentist is essential. Lupus patients should visit the dentist at regular intervals, usually every two to six months. The patients must tell the dentist about their current and past medical history. The dentist should also be supplied with a complete list of medications the patient is taking. The dentist also needs to know the dosage and any side-effects of all the medications. The patient's physician should in turn be made aware of any dental problems and the proposed treatment.
If the lupus patient needs dental surgery, open communication between physician and dentist is especially important. The surgical procedure may well require a change in the dosage of any steroid medications and may require prophylactic (preventive) antibiotics if the patient has certain kinds of lupus heart disease (the kind that affects the valves of the heart). In such cases it may be necessary for the dentist to consult the patient's physician to decide on the best management. Post-operative examinations for the lupus patient must be more frequent and extend over a longer period of time than would be the case for the non-lupus dental patient.
Self-examination
Self-examination of the mouth should regularly be performed by lupus patients. Periodontal disease, which causes the greatest amount of tooth loss in adults and affects almost everyone, usually does not cause pain and may not give warning until significant tissue breakdown has occurred. However, there are signs and symptoms of periodontal disease which the patient can detect with self-examination. There are also certain procedures the patient can perform to prevent and help control periodontal disease. Periodontal disease is a group of inflammatory conditions which affect the gums (gingiva) and supporting bone around each tooth. The most common periodontal disorders are gingivitis (inflammation of the gingiva or gums) and periodontitis (inflammation affecting the bone under the gingiva). The presence of any of the following signs and symptoms may indicate the presence of gingivitis.
Jay A. Denbo, DDS
COMMUNICATION, SELF-EXAMINATION, and PREVENTION are the keys to controlling dental problems for the lupus patient. Lupus patients have specific dental problems because of the disease process and the medications needed to control lupus symptoms.
Communication
Communication between patient, physician, and dentist is essential. Lupus patients should visit the dentist at regular intervals, usually every two to six months. The patients must tell the dentist about their current and past medical history. The dentist should also be supplied with a complete list of medications the patient is taking. The dentist also needs to know the dosage and any side-effects of all the medications. The patient's physician should in turn be made aware of any dental problems and the proposed treatment.
If the lupus patient needs dental surgery, open communication between physician and dentist is especially important. The surgical procedure may well require a change in the dosage of any steroid medications and may require prophylactic (preventive) antibiotics if the patient has certain kinds of lupus heart disease (the kind that affects the valves of the heart). In such cases it may be necessary for the dentist to consult the patient's physician to decide on the best management. Post-operative examinations for the lupus patient must be more frequent and extend over a longer period of time than would be the case for the non-lupus dental patient.
Self-examination
Self-examination of the mouth should regularly be performed by lupus patients. Periodontal disease, which causes the greatest amount of tooth loss in adults and affects almost everyone, usually does not cause pain and may not give warning until significant tissue breakdown has occurred. However, there are signs and symptoms of periodontal disease which the patient can detect with self-examination. There are also certain procedures the patient can perform to prevent and help control periodontal disease. Periodontal disease is a group of inflammatory conditions which affect the gums (gingiva) and supporting bone around each tooth. The most common periodontal disorders are gingivitis (inflammation of the gingiva or gums) and periodontitis (inflammation affecting the bone under the gingiva). The presence of any of the following signs and symptoms may indicate the presence of gingivitis.
- Bleeding gums: Gingiva which bleeds on eating or brushing teeth, or bleeds without any obvious cause.
- Swollen gums: Enlarged gingiva which is not bound tightly around each tooth.
- Red Gums: Gingiva which is red in color. Dark colored gingiva due to naturally occurring melanin (the substance which colors the gums) is not an indication of inflammation.
- Sensitive gums: Gingiva which is painful to brushing. Untreated gingivitis can progress into the tissues under the gingiva and cause the bone which supports the teeth to become weakened and resorb.
Periodontitis is a form of dental disease which is marked by bone loss and will eventually lead to loosening or loss of teeth. It is usually painless and may have the same signs and symptoms as gingivitis. If any signs or symptoms of gingivitis or periodontitis are noted, the tupus patient should tell his/her dentist. A dentist can accurately diagnose and treat periodontat disease.
Prevention
Prevention is the best treatment for dental disease. The only way to prevent periodontal disease is by effective tooth brushing techniques. Putting toothpaste on a toothbrush and swishing the brush around your mouth for 15 to 30 seconds once or twice a day is not effective tooth brushing. Tooth brushing must clean all five surfaces of each tooth. The most critical area of the tooth to clean is immediately adjacent or next to the gingiva or gums. Therefore, the junction between the gum and the tooth should be very carefully brushed. The area between the teeth cannot be cleaned by a toothbrush. Dental floss, dental tape, rubber tips, special (interproximal) brushes and Stimulators are used to remove the dental plaque which is deposited between adjacent teeth. It should take approximately three minutes to brush your teeth thoroughly. Plaque which causes dental caries (cavities) and periodontal disease accumulates on all tooth surfaces and must be removed daily. Any areas of gingival sensitivity, tooth sensitivity, or gingival bleeding require effective brushing or the sensitivity and bleeding will get worse. If it hurts or if it bleeds, it should be brushed harder.
Lupus patients should check the inside of their mouths regularly for any red or irritated areas. If such areas are noticed, these should be brought to the attention of the physician and dentist. Approximately 25% of lupus patients have these kinds of oral problems, which are usually accompanied by a skin irritation or facial rash. Oral problems in lupus are also found on the lips and on the tissue inside the cheeks. Patients with active oral problems should not use denture powder or denture paste to hold in removable dentures. if toothpaste irritates the mouth, baking soda and water should be substituted for it.
=========================================================== This information is for "informational purposes" and is not meant to be used for medical diagnosis. Always consult your physician on matters such as this.
With COMMUNICATION, SELF-EXAMINATION, and PREVENTION, the lupus patient can keep dental problems under control.
Avascular Necrosis of Bone and Lupus
Avascular Necrosis of Bone and Lupus
Marvin E. Steinberg, MD
Avascular necrosis of bone (AVN) is a disorder in which an avascular (lacking in blood supply) area of bone undergoes necrosis (dies). AVN is not common, but it is encountered in a certain number of patients with lupus. The area most often affected is the hip and in particular the upper part of the thigh bone (the femoral head) which makes up the ball of this ball-and-socket joint. This problem primarily affects younger adults. In 50 to 60 percent of cases it occurs in both hips. The goal for treatment of AVN is to save the natural hip joint if possible and not to have to replace the affected femoral head with an artificial joint. In order to accomplish this, early diagnosis is very important. Other bones may also be affected by AVN, but much less often than the hip. (These include the knee, the shoulder, and rarely the small bones of the wrist and the foot or ankle.) The following discussion will, therefore, focus on the hip.
Causes
Many things can cause AVN. One of the most common causes is a fracture through the thigh bone which results in tearing of the blood vessels that nourish the femoral head. AVN can also occur without a fracture or other major injury. These same blood vessels can be blocked by blood clots (thrombi or emboli). They can also be blocked by fat droplets which form in the circulating blood, by clumps of abnormal red blood cells (as in sickle cell disease), and occasionally by nitrogen bubbles (which form in individuals such as deep sea divers who work under different atmospheric pressures). AVN can also result from inflammation or narrowing of the arteries, and from increased pressure outside the blood vessels. it is frequently seen with excess use of alcohol. A very small percentage of people who use corticosteroids for prolonged periods of time will also develop it. Why this is so is unclear. It may be that certain individuals are especially sensitive to steroids and form circulating fat droplets as a result. Although some authorities are of the opinion that the blood vessel changes in lupus itself can result in AVN, there are almost no cases of AVN reported in patients with lupus who have not been treated with steroids.
Within a few hours after the blood circulation to the bone is blocked, the cells in the bone marrow and the bone begin to die. The body then makes an attempt to repair the damage. During this repair process the pressure within the bone begins to build. In approximately 80 percent of cases, a steady progression of damage takes place. The dead area of bone becomes weakened and begins to collapse. This starts in the soft bone underneath the surface of the joint. Eventually, however, the joint surface itself becomes involved, and actual flattening of the normally round femoral head results. The cartilage of the joint is subjected to abnormal stresses and undergoes gradual degeneration (breakdown). Since this cartilage gets its nourishment from the fluid within the joint and not from the blood supply of the underlying bone, it remains alive for quite some time after the initial degeneration begins. In the later stages, cartilage damage ultimately leads to advanced degenerative arthritis involving the entire hip joint.
Clinical Course
At first the patient with AVN has no symptoms. Later the buildup of pressure within the femoral head may cause a mild and vague type of pain. Once collapse of the joint surface occurs, the pain usually increases dramatically and may become severe. Some patients however have only mild discomfort in spite of significant involvement of the joint. As the process continues, most patients develop a limp and note some decrease in motion of the hip joint.
Early on, routine x-rays in AVN are entirely normal. As the softer bone below the joint surface begins to collapse, a fluid filled space is left which shows up as a dark semi-circle or "crescent sign" on the x-ray. Later actual flattening of the normally round femoral head may be seen. Thinning of the cartilage of the joint results in narrowing of the joint space. This will show on an x-ray. Still later the characteristic picture of advanced degenerative joint disease is seen on the x-ray and may be accompanied by complete loss of the joint space, the formation of spurs (or osteophytes), large cysts, and areas of dense bone.
There are other special imaging techniques which are frequently used to diagnose AVN in addition to plain x-rays. Often these techniques show clear changes in the bone before such changes can be detected on the routine x-ray. These include bone scans, computerized tomography (CAT) scans, and magnetic resonance imaging (MRI). During the last few years, MRI has proven to be the single best method for the early diagnosis of AVN. The MRI does not use x-rays but uses magnetic waves to show very early changes in the marrow of the bone, bone itself, and other tissues in and around the hip joint. It is a very safe technique and is both very sensitive and very specific for AVN.
Treatment
In 80 or 90 percent of cases of AVN, the condition will progress even if we restrict activities and limit weight bearing on the joint by having the patient use canes or crutches. For this reason, it is usually best not to try to treat the hip "conservatively" if it is important to save the joint. (The shoulder and knee do better with "conservative management" than the hip does, and this is usually the treatment of choice for these joints.)
Although there is no completely effective method for preventing early AVN of the hip from progressing, there are a number of surgical procedures which give better results than conservative medical management. These should be very seriously considered during the early stages of AVN, before there has been any collapse of the joint. They include drilling small or large holes to relieve the pressure, bone grafting, osteotomy (cutting across the bone to change its position), and electrical stimulation. There have been some encouraging reports about the use of electrical stimulation, but this technique is still in a somewhat experimental stage. Another new procedure which seems quite promising is the use of a bone graft containing its own blood vessels which can be attached to the arteries and veins in the region of the hip. Although none of these surgical treatments gives consistent or completely satisfactory results, progress is being made, and the use of one of these techniques will usually give better results than non-operative management, as mentioned earlier.
Once there has been definite flattening of the femoral head, these early surgical interventions are seldom of much value. At this stage, patients should be treated conservatively with measures designed to decrease their pain and preserve function of the hip. Such measures include restricted activities, use of a cane, and non-steroidal anti-inflammatory drugs or mild pain relievers.
When pain and disability have progressed to the point that conservative methods of treatment are no longer effective in relieving symptoms, reconstructive surgery should be considered. There are two commonly used procedures: The replacement of only the upper end of the femur (the "ball") with an "endoprosthesis" and the replacement of both the ball and the socket with a "total hip replacement". Of these two operations, the use of a total hip replacement seems to give the most consistent and durable results. It leads to complete or nearly complete relief of pain and relatively normal function in 90 to 95 percent of patients. With modern techniques and devices these artificial hips should continue to function for at least ten to fifteen years in the majority of patients. In the younger individual they will rarely last a lifetime, but when they do wear out, they can be replaced.
Summary
AVN is a rather uncommon complication in patients with lupus and is probably related to the need to use corticosteroids rather than to the underlying disease itself. The area most frequently involved is the hip and specifically the femoral head. Although the condition may heal spontaneously (without any treatment) in 10 to 20 percent of diagnosed cases, most of the time it will get worse without specific treatment. The goal is therefore to diagnose this condition as early as possible and to use any one of a number of surgical procedures which may prevent or slow down its progression. Although the results with the present surgical methods of treatment are not as good as we would like, they are generally better than simply relying on symptomatic treatment. We remain optimistic that some of the newer methods will give better results. When the condition has become fairly advanced, such preventative measures are of little value and patients are treated symptomatically for as long as possible. When sufficient pain and disability have developed, reconstructive surgery is usually needed. Of the available measures, total hip replacement gives the best results. This procedure can allow patients to resume a relatively normal lifestyle with little pain or disability.
=========================================================== This information is for "informational purposes" and is not meant to be used for medical diagnosis. Always consult your physician on matters such as this.
Marvin E. Steinberg, MD
Avascular necrosis of bone (AVN) is a disorder in which an avascular (lacking in blood supply) area of bone undergoes necrosis (dies). AVN is not common, but it is encountered in a certain number of patients with lupus. The area most often affected is the hip and in particular the upper part of the thigh bone (the femoral head) which makes up the ball of this ball-and-socket joint. This problem primarily affects younger adults. In 50 to 60 percent of cases it occurs in both hips. The goal for treatment of AVN is to save the natural hip joint if possible and not to have to replace the affected femoral head with an artificial joint. In order to accomplish this, early diagnosis is very important. Other bones may also be affected by AVN, but much less often than the hip. (These include the knee, the shoulder, and rarely the small bones of the wrist and the foot or ankle.) The following discussion will, therefore, focus on the hip.
Causes
Many things can cause AVN. One of the most common causes is a fracture through the thigh bone which results in tearing of the blood vessels that nourish the femoral head. AVN can also occur without a fracture or other major injury. These same blood vessels can be blocked by blood clots (thrombi or emboli). They can also be blocked by fat droplets which form in the circulating blood, by clumps of abnormal red blood cells (as in sickle cell disease), and occasionally by nitrogen bubbles (which form in individuals such as deep sea divers who work under different atmospheric pressures). AVN can also result from inflammation or narrowing of the arteries, and from increased pressure outside the blood vessels. it is frequently seen with excess use of alcohol. A very small percentage of people who use corticosteroids for prolonged periods of time will also develop it. Why this is so is unclear. It may be that certain individuals are especially sensitive to steroids and form circulating fat droplets as a result. Although some authorities are of the opinion that the blood vessel changes in lupus itself can result in AVN, there are almost no cases of AVN reported in patients with lupus who have not been treated with steroids.
Within a few hours after the blood circulation to the bone is blocked, the cells in the bone marrow and the bone begin to die. The body then makes an attempt to repair the damage. During this repair process the pressure within the bone begins to build. In approximately 80 percent of cases, a steady progression of damage takes place. The dead area of bone becomes weakened and begins to collapse. This starts in the soft bone underneath the surface of the joint. Eventually, however, the joint surface itself becomes involved, and actual flattening of the normally round femoral head results. The cartilage of the joint is subjected to abnormal stresses and undergoes gradual degeneration (breakdown). Since this cartilage gets its nourishment from the fluid within the joint and not from the blood supply of the underlying bone, it remains alive for quite some time after the initial degeneration begins. In the later stages, cartilage damage ultimately leads to advanced degenerative arthritis involving the entire hip joint.
Clinical Course
At first the patient with AVN has no symptoms. Later the buildup of pressure within the femoral head may cause a mild and vague type of pain. Once collapse of the joint surface occurs, the pain usually increases dramatically and may become severe. Some patients however have only mild discomfort in spite of significant involvement of the joint. As the process continues, most patients develop a limp and note some decrease in motion of the hip joint.
Early on, routine x-rays in AVN are entirely normal. As the softer bone below the joint surface begins to collapse, a fluid filled space is left which shows up as a dark semi-circle or "crescent sign" on the x-ray. Later actual flattening of the normally round femoral head may be seen. Thinning of the cartilage of the joint results in narrowing of the joint space. This will show on an x-ray. Still later the characteristic picture of advanced degenerative joint disease is seen on the x-ray and may be accompanied by complete loss of the joint space, the formation of spurs (or osteophytes), large cysts, and areas of dense bone.
There are other special imaging techniques which are frequently used to diagnose AVN in addition to plain x-rays. Often these techniques show clear changes in the bone before such changes can be detected on the routine x-ray. These include bone scans, computerized tomography (CAT) scans, and magnetic resonance imaging (MRI). During the last few years, MRI has proven to be the single best method for the early diagnosis of AVN. The MRI does not use x-rays but uses magnetic waves to show very early changes in the marrow of the bone, bone itself, and other tissues in and around the hip joint. It is a very safe technique and is both very sensitive and very specific for AVN.
Treatment
In 80 or 90 percent of cases of AVN, the condition will progress even if we restrict activities and limit weight bearing on the joint by having the patient use canes or crutches. For this reason, it is usually best not to try to treat the hip "conservatively" if it is important to save the joint. (The shoulder and knee do better with "conservative management" than the hip does, and this is usually the treatment of choice for these joints.)
Although there is no completely effective method for preventing early AVN of the hip from progressing, there are a number of surgical procedures which give better results than conservative medical management. These should be very seriously considered during the early stages of AVN, before there has been any collapse of the joint. They include drilling small or large holes to relieve the pressure, bone grafting, osteotomy (cutting across the bone to change its position), and electrical stimulation. There have been some encouraging reports about the use of electrical stimulation, but this technique is still in a somewhat experimental stage. Another new procedure which seems quite promising is the use of a bone graft containing its own blood vessels which can be attached to the arteries and veins in the region of the hip. Although none of these surgical treatments gives consistent or completely satisfactory results, progress is being made, and the use of one of these techniques will usually give better results than non-operative management, as mentioned earlier.
Once there has been definite flattening of the femoral head, these early surgical interventions are seldom of much value. At this stage, patients should be treated conservatively with measures designed to decrease their pain and preserve function of the hip. Such measures include restricted activities, use of a cane, and non-steroidal anti-inflammatory drugs or mild pain relievers.
When pain and disability have progressed to the point that conservative methods of treatment are no longer effective in relieving symptoms, reconstructive surgery should be considered. There are two commonly used procedures: The replacement of only the upper end of the femur (the "ball") with an "endoprosthesis" and the replacement of both the ball and the socket with a "total hip replacement". Of these two operations, the use of a total hip replacement seems to give the most consistent and durable results. It leads to complete or nearly complete relief of pain and relatively normal function in 90 to 95 percent of patients. With modern techniques and devices these artificial hips should continue to function for at least ten to fifteen years in the majority of patients. In the younger individual they will rarely last a lifetime, but when they do wear out, they can be replaced.
Summary
AVN is a rather uncommon complication in patients with lupus and is probably related to the need to use corticosteroids rather than to the underlying disease itself. The area most frequently involved is the hip and specifically the femoral head. Although the condition may heal spontaneously (without any treatment) in 10 to 20 percent of diagnosed cases, most of the time it will get worse without specific treatment. The goal is therefore to diagnose this condition as early as possible and to use any one of a number of surgical procedures which may prevent or slow down its progression. Although the results with the present surgical methods of treatment are not as good as we would like, they are generally better than simply relying on symptomatic treatment. We remain optimistic that some of the newer methods will give better results. When the condition has become fairly advanced, such preventative measures are of little value and patients are treated symptomatically for as long as possible. When sufficient pain and disability have developed, reconstructive surgery is usually needed. Of the available measures, total hip replacement gives the best results. This procedure can allow patients to resume a relatively normal lifestyle with little pain or disability.
=========================================================== This information is for "informational purposes" and is not meant to be used for medical diagnosis. Always consult your physician on matters such as this.
Monday, April 24, 2006
Lupus and the Eye
Lupus and the Eye
Stephen H. Sinclair, MDErnesto L. Collazo, MD
Systemic lupus erythematosus is a disease which can involve every organ in the human body. It is no surprise, therefore, that it can involve not only the eyes but also the orbit (eye socket) around the eyes and the eyelids that house them. It may even involve the nerve connections from the eye to the brain. Before we can discuss eye problems in lupus, it is first necessary to understand the structure of the human eye. The eye can be compared to a photographic camera.
Stephen H. Sinclair, MDErnesto L. Collazo, MD
Systemic lupus erythematosus is a disease which can involve every organ in the human body. It is no surprise, therefore, that it can involve not only the eyes but also the orbit (eye socket) around the eyes and the eyelids that house them. It may even involve the nerve connections from the eye to the brain. Before we can discuss eye problems in lupus, it is first necessary to understand the structure of the human eye. The eye can be compared to a photographic camera.
The camera contains a lens which focuses an image onto the film, located against the back wall of the camera. The eye is arranged in a similar fashion. It is protected by the eyelids which act as windshield wipers, spreading tears over the surface of the eye (the cornea) to keep the cornea moist and clear. The lids have a smooth inner lining known as the conjunctiva. The conjunctiva is essentially a membrane which also covers the front portion of the eyeball preventing objects such as dirt and contact lenses from getting behind the eye. The front surface of the eye is covered by the cornea, a transparent window similar to the crystal of a watch. Behind the cornea is the iris which may be of various colors (brown, blue, green, etc.) and acts similarly to the diaphragm of the camera by controlling the amount of light that enters the eye.
Behind the iris is the lens which, like the lens of the camera, focuses the image on the retina at the back of the eye. It is not composed of glass but rather is a bag of clear protein. The eye's outer wall or sclera, the white of the eye, provides structural strength. The inside layer of the eye, known as the retina, is the light sensitive layer that is similar to the film of the camera. It lies flat against the back inside wall of the eye. The nerve impulses which it produces are transmitted to the visual part of the brain through the optic nerve in the back of the eye.
Skin and Eyelids
Lupus can produce inflammatory lesions (or sores) of the eyelids. The lesions may be small and individual ("discoid") or they may b more widespread causing swelling, irritation and redness of the eyelids, and sometimes loss of the eyelashes. Over time these lesion, if untreated, may cause scarring which can distort the eyelids and prevent them from performing their function of blinking and wiping the front of the eye. Treatment usually consists of systemic (taken internally) or topical (applied locally) corticosteroids to decrease inflammation.
Conjunctiva
The conjunctiva may also become inflamed causing redness and tearing, although this does not occur often. The tear glands are located in the conjunctiva. When lupus affects the conjunctiva, causing inflammation and scarring, it may reduce tear production resulting in dryness of the eye (which is often associated with mouth and vaginal dryness and is known as sicca or Sjogren's Syndrome). With reduced wetting, the cornea can lose its clarity and become roughened and irritable as well as prone to infection (ulcers). Rarely inflammation of the sclera or iris may also occur. Again systemic or topical (eye drops in this case) corticosteroids are used to reduce the inflammation. In addition artificial tear preparations and ointments can be used to reduce the dryness and subsequent irritation. Infection or ulcers of the cornea require prompt and careful treatment with antibiotics.
Retina
By far the most common effect of systemic lupus erythematostis on the eye is injury to the blood vessels that supply the retina. Small retinal hemorrhages can result, as well as occlusions or blockages of the smaller retinal blood vessels. This causes damage to the retinal tissue due to lack of an adequate blood supply. Infrequently, larger arteries may also be occluded leading to loss of function in large areas of the retina. When this involves the center of the retina, known as the macula, a large area of central vision is lost. The optic nerve may also suffer from blockages of the blood vessels which supply it, resulting in loss of vision in the entire eye. In rare cases the blockages of blood vessels in the retina may be so severe and widespread that the retina may, in response to the blockages, attempt to develop new vessels. This process is termed "neovascularization." Such new vessels grow abnormally out from the surface of the retina into the central vitreous gel (the substance that fills the inside of the eye). They are prone to bleeding when stretched or pulled by the gel, resulting in severe vision loss. Although these abnormal blood vessels are rare in patients who are on adequate treatment for lupus, all lupus patients should be followed with periodic eye examinations because the blockages and abnormal vessel growth may occur without the patient noticing symptoms. If blockages or neovascularization are detected early, they may be treated with a laser. This procedure is usually done as an outpatient.
Central Nervous System
Involvement of the central nervous system is uncommon but, when it occurs, may be due to blood vessel inflammation and blockage within the brain. The resulting damage may affect the visual system in a variety of ways. These include defects or gray areas of the visual field in one or both eyes, eye movement abnormalities which can lead to double vision, disturbances in the function of the pupils, and damage to the optic nerve leading to loss of vision in one eye.
Complications of Systemic Drugs
Treatments for reducing the systemic inflammation of lupus (inflammation which can affect any area of the body) are vitally important to the well-being of the patient and also lessen the effect of inflammation on the eye, eyelids, or orbit. Some of the drugs which are used in the treatment of systemic lupus, however, can have side effects on the eyes. The long-term use of steroids, either systemic or topical, may cause the formation of cataracts. A cataract is a change in the lens of the eye which can affect the ability of the lens to focus. It can produce opacities or defects that create glare and haziness of vision. Most mild cataracts are treated with corrective eyeglasses or special lens filters. When the glare or haziness become such a problem that patients can no longer see well enough to go about their daily lives, then the lens with the cataract can be removed surgically and replaced with an artificial lens which restores the vision.
In addition, corticosteroids can lead to the development of' glaucoma, a condition in which an increase in the pressure within the eye causes damage to the optic nerve and an irreversible loss of' vision. This condition can be treated with medications to lower the pressure within the eye, therefore preventing further damage to the optic nerve. Periodic eye examinations are vital to the early detection and treatment of glaucoma. The use of antimalarials has become widespread in the treatment of lupus patients. One of the first of these, chloroquine, was found to have a significant toxic effect on the eye. Over the years the drug has a tendency to accumulate in the layer between the retina and the sclera, and in some cases this leads to severe destruction of the central retina with central vision loss.
A drug similar to chloroquine is more commonly used today. It is called hydroxychloroquine (Plaquenil). This drug is apparently not as toxic and vision loss is extremely rare, even with relatively high doses taken over several years. Lupus patients who are on Plaquenil are still checked carefully with periodic vision and field testing and evaluation of the retina. If toxic side effects to the retina do occur, they are detected early, and the drug can be stopped without any further retinal damage and no injury to vision.
=========================================================== This information is for "informational purposes" and is not meant to be used for medical diagnosis. Always consult your physician on matters such as this.
Lupus and Anemia
Lupus and Anemia
Douglas B. Cines, MD
Anemia is the condition in which an individual has less than the normal number of red cells in the blood. Anemia occurs frequently in patients with lupus and some of these patients may require special attention. How do you know if you are anemic? Patients with anemia may have no symptoms or may feel fatigued because not enough red blood cells are available to carry oxygen to muscles and other tissues. However, similar feelings of fatigue may be caused by lupus itself. Occasionally, patients who are anemic may feel dizzy, experience a rapid heart beat or chest discomfort. These symptoms are more likely to develop following exercise when the body's demand for oxygen is increased.
If you experience increasing fatigue or related symptoms, your physician may check to see if you are anemic. Your doctor may use the term "hematocrit" to describe the number of red cells in your blood. The hematocrit refers to the proportion of whole blood occupied by the red cells themselves. Usually about 40% of the blood is composed of red cells, but in patients with anemia, this proportion may be reduced to 20-30%.If you are found to be anemic, your physician may order other tests to find out the cause. There are four common causes of anemia that patients with lupus experience.
Anemia of Chronic Illness. Normally, as red blood cells age, approximately 1% of them are removed from the blood each day. The bone marrow, where all blood cells are made, replaces these cells with new ones. When lupus is active, many patients develop anemia because the bone marrow produces fewer red blood cells than are needed. The exact reason why fewer cells are produced is unknown, but in some way this is related to the tissue inflammation caused by lupus. In most patients, this form of anemia is mild and produces no symptoms. This form of anemia does not respond to changes in diet, vitamins, etc., but improves when the lupus has been controlled.
Iron Deficiency Anemia. Sometimes anemia is caused by iron deficiency. Iron deficiency is also a common cause of anemia in healthy women who do not have lupus. It usually results from loss of iron over many years due to normal menstrual bleeding. Medications used to treat lupus, such as aspirin, may cause irritation of the stomach lining leading to bleeding and iron deficiency. If you are anemic, you should get a blood test to determine if you are iron deficient before taking iron pills, because iron has no beneficial effect if the anemia is caused bv another problem.
Anemia of Kidney Disease. Some patients with lupus develop kidney damage. The kidney makes a hormone called erythropoietin that is needed by the bone marrow to produce red blood cells.
If the kidney is damaged and does not produce this hormone, the bone marrow will produce fewer red blood cells. Your doctor will be able to tell by urine and blood tests if kidney damage is responsible for the anemia. If kidney function improves with treatment, the production of erythropoietin increases and the anemia improves.
Autoimmune Hemolytic Anemia. Anemia may also result from the production of an antibody that binds to an individual's own red blood cells. 'I'his form of anemia, called autoimmune hemolytic anemia (AHA), occurs in approximately 5% of patients with lupus. An antibody is a type of protein made by the body, usually in response to infection. Patients with lupus commonly produce antibodies that bind to their own cells. In some cases, these antibodies bind to the red cells. These red cells coated with antibodies are quickly removed from the blood and destroyed by white blood cells, called macrophages, that normally destroy antibody coated viruses or bacteria as part of the body's defenses against infection. If the production of new red cells cannot keep up with this destruction, the patient becomes anemic.
Your physician can tell if you have autoimmune hemolytic anemia by looking at your blood under the microscope. If you do, the physician will see both red cells whose shape has been altered by these macrophages and a large number of newly produced, young red blood cells. The diagnosis of AHA is confirmed using the direct antiglobulin test, or Coombs' test, which measures both the presence and type of antibody bound to the red cell. The treatment of AHA depends upon its severity. If the anemia is mild, there may be no need for treatment. In one variety of uncommon AHA, called "cold-agglutinin disease", simple measures such as preventing exposure to cold may be sufficient. Periodic transfusions of warmed blood are often useful for patients with cold-agglutinin disease who have mild symptoms.
Patients with the more common, "warm-antibody" type AHA often require additional measures to correct the anemia. In most cases, the doctor will recommend the drug, prednisone, which both decreases the rate of red cell destruction and the amount of antibody produced. Sometimes prednisone must be taken for several weeks before its effect becomes apparent. Patients who do not respond to prednisone, or who require high doses of prednisone to control the anemia, may be advised to have an operation to remove their spleen. The reason why this procedure is so often recommended is that in many patients, most of the red cells to which antibodies have become attached are trapped and destroyed by macrophages within the spleen. In some patients the hormone, Danazol, or other drugs such as cyclophosphamide (Cytoxan) may also be recommended.
Transfusions are used only as a temporary measure since the transfused red cells will also quickly become coated with antibodies and will be destroyed. AHA can be managed Successfully in practically all patients with systemic lupus, but the specific recommendations for treatment vary among different patients for several reasons. First, the doctor must be sure of the diagnosis. Other causes of anemia may also be present and may contribute to the anemia. Second, the doctor must determine if the anemia is actually contributing to the patient's symptoms, since one of the major reasons to treat a patient is to improve how she/he feels. Third, the amount of medicine needed to treat AHA may vary from time to time. Patients vary widely in their ability to tolerate anemia and their ability to tolerate each form of treatment. Therefore, treatments must be custom tailored to fit each individual.
=========================================================== This information is for "informational purposes" and is not meant to be used for medical diagnosis. Always consult your physician on matters such as this.
Douglas B. Cines, MD
Anemia is the condition in which an individual has less than the normal number of red cells in the blood. Anemia occurs frequently in patients with lupus and some of these patients may require special attention. How do you know if you are anemic? Patients with anemia may have no symptoms or may feel fatigued because not enough red blood cells are available to carry oxygen to muscles and other tissues. However, similar feelings of fatigue may be caused by lupus itself. Occasionally, patients who are anemic may feel dizzy, experience a rapid heart beat or chest discomfort. These symptoms are more likely to develop following exercise when the body's demand for oxygen is increased.
If you experience increasing fatigue or related symptoms, your physician may check to see if you are anemic. Your doctor may use the term "hematocrit" to describe the number of red cells in your blood. The hematocrit refers to the proportion of whole blood occupied by the red cells themselves. Usually about 40% of the blood is composed of red cells, but in patients with anemia, this proportion may be reduced to 20-30%.If you are found to be anemic, your physician may order other tests to find out the cause. There are four common causes of anemia that patients with lupus experience.
Anemia of Chronic Illness. Normally, as red blood cells age, approximately 1% of them are removed from the blood each day. The bone marrow, where all blood cells are made, replaces these cells with new ones. When lupus is active, many patients develop anemia because the bone marrow produces fewer red blood cells than are needed. The exact reason why fewer cells are produced is unknown, but in some way this is related to the tissue inflammation caused by lupus. In most patients, this form of anemia is mild and produces no symptoms. This form of anemia does not respond to changes in diet, vitamins, etc., but improves when the lupus has been controlled.
Iron Deficiency Anemia. Sometimes anemia is caused by iron deficiency. Iron deficiency is also a common cause of anemia in healthy women who do not have lupus. It usually results from loss of iron over many years due to normal menstrual bleeding. Medications used to treat lupus, such as aspirin, may cause irritation of the stomach lining leading to bleeding and iron deficiency. If you are anemic, you should get a blood test to determine if you are iron deficient before taking iron pills, because iron has no beneficial effect if the anemia is caused bv another problem.
Anemia of Kidney Disease. Some patients with lupus develop kidney damage. The kidney makes a hormone called erythropoietin that is needed by the bone marrow to produce red blood cells.
If the kidney is damaged and does not produce this hormone, the bone marrow will produce fewer red blood cells. Your doctor will be able to tell by urine and blood tests if kidney damage is responsible for the anemia. If kidney function improves with treatment, the production of erythropoietin increases and the anemia improves.
Autoimmune Hemolytic Anemia. Anemia may also result from the production of an antibody that binds to an individual's own red blood cells. 'I'his form of anemia, called autoimmune hemolytic anemia (AHA), occurs in approximately 5% of patients with lupus. An antibody is a type of protein made by the body, usually in response to infection. Patients with lupus commonly produce antibodies that bind to their own cells. In some cases, these antibodies bind to the red cells. These red cells coated with antibodies are quickly removed from the blood and destroyed by white blood cells, called macrophages, that normally destroy antibody coated viruses or bacteria as part of the body's defenses against infection. If the production of new red cells cannot keep up with this destruction, the patient becomes anemic.
Your physician can tell if you have autoimmune hemolytic anemia by looking at your blood under the microscope. If you do, the physician will see both red cells whose shape has been altered by these macrophages and a large number of newly produced, young red blood cells. The diagnosis of AHA is confirmed using the direct antiglobulin test, or Coombs' test, which measures both the presence and type of antibody bound to the red cell. The treatment of AHA depends upon its severity. If the anemia is mild, there may be no need for treatment. In one variety of uncommon AHA, called "cold-agglutinin disease", simple measures such as preventing exposure to cold may be sufficient. Periodic transfusions of warmed blood are often useful for patients with cold-agglutinin disease who have mild symptoms.
Patients with the more common, "warm-antibody" type AHA often require additional measures to correct the anemia. In most cases, the doctor will recommend the drug, prednisone, which both decreases the rate of red cell destruction and the amount of antibody produced. Sometimes prednisone must be taken for several weeks before its effect becomes apparent. Patients who do not respond to prednisone, or who require high doses of prednisone to control the anemia, may be advised to have an operation to remove their spleen. The reason why this procedure is so often recommended is that in many patients, most of the red cells to which antibodies have become attached are trapped and destroyed by macrophages within the spleen. In some patients the hormone, Danazol, or other drugs such as cyclophosphamide (Cytoxan) may also be recommended.
Transfusions are used only as a temporary measure since the transfused red cells will also quickly become coated with antibodies and will be destroyed. AHA can be managed Successfully in practically all patients with systemic lupus, but the specific recommendations for treatment vary among different patients for several reasons. First, the doctor must be sure of the diagnosis. Other causes of anemia may also be present and may contribute to the anemia. Second, the doctor must determine if the anemia is actually contributing to the patient's symptoms, since one of the major reasons to treat a patient is to improve how she/he feels. Third, the amount of medicine needed to treat AHA may vary from time to time. Patients vary widely in their ability to tolerate anemia and their ability to tolerate each form of treatment. Therefore, treatments must be custom tailored to fit each individual.
=========================================================== This information is for "informational purposes" and is not meant to be used for medical diagnosis. Always consult your physician on matters such as this.
Sunday, March 05, 2006
Neurological Complications of Lupus
Terry D. Heiman-Patterson, MD
LEARNING ABOUT LUPUS: A USER FRIENDLY GUIDEFrom the Lupus Foundation of Delaware Valley, Inc.Edited by: Mary E. Moore, Ph.D., M.D., Carolyn H. McGrory, MS, RN, Robert S. Rosenthal, M.D.
Systemic lupus etythematosus (SLE) is a multisystem disorder that frequently affects the nervous system. In fact, of all the connective tissue disorders, lupus has the highest incidence of nervous system complications. These neurologic complications may occur in 30-75 percent of patients. In order to understand the variety of complications, one must have some notion of what makes up the nervous system.
The nervous system contains the brain, brain stem, spinal cord, nerves outside the brain and spinal column (peripheral nerves), and muscle. The brain has centers for speech, movement, sensation, and also more complex functions such as thinking, memory, and personality. The brain relays commands and messages to other areas of the nervous system and receives information from these areas, as well. The brain blends together all this information so that persons can interact with their environment. Between the brain and the spinal cord is the brain stem. The brain stem contains nerve cells that control the muscles of eye movement, facial expression, speaking, swallowing, hearing and balance. The spinal cord contains nerve cells that supply impulses to and from the muscles (i.e., innervate the muscles), and that receive sensory information from the skin and joints.
Muscles attach to bones and when they contract they enable a person to move. Information travels from the brain to the brain stem and spinal cord where contact is made with the nerve cells, (i.e. the cranial and peripheral nerves) innervating muscles of the head and arms and legs. Sensory information is also relayed from these areas through contacts in the spinal cord and brain stem to the sensory centers of the brain. There are several possible ways that lupus can damage the nervous system. First, the blood vessels of the brain may themselves be damaged, leading to poor blood flow, to death of brain tissue (strokes), or to bleeding (hemorrhage). There may be an immune system attack on the nervous system because of antibodies that are produced. These can cause problems directly by attacking nerve cells (antineuronal antibodies) or indirectly by causing blood clots in the brain (lupus anticoagulants or anticardiolipin antibodies).
When lupus affects the nervous system, the type of symptom and the distribution of complaints will depend on what area of the nervous system is involved and on what sensory and motor pathways are damaged. When there is more general involvement of the brain, a patient may demonstrate personality changes and psychiatric disorders. Actual psychosis (severe mental illness) can occur, or mood changes such as depression, or even an unnatural feeling of well-being (euphoria) can occur. Other patients may experience anxiety that is out of proportion to the problems they are experiencing. in all these psychiatric disorders, thinking and memory are normal. However, some patients may actually have loss of intellectual skills (i.e., dementia), or experience confusion and altered consciousness, (sleepiness and even coma). Although confusion and altered consciousness may occur because of direct involvement of the brain by lupus, it can also occur because of drugs used in the treatment of lupus (i.e. steroids) or because of other complications from lupus (i.e. infection, kidney failure).
In these instances, withdrawal of the drug, treatment of the infection, or management of the other systemic complications will help to clear the confusion and improve alertness. Another complication due to brain involvement is having seizures or convulsions. These can occur in up to 20% of patients. In patients with kidney failure due to lupus, the rate of seizures rises to almost 50 percent. Lupus can also cause strokes in which there is a sudden onset of neurologic symptoms. Strokes can be due to bleeding into the brain or to death of brain tissue because of lack of blood flow. These problems occur if there is inflammation of blood vessels, clotting of blood within vessels, or not enough clotting of blood causing patients to hemorrage more readily. Patients with strokes may have a variety of symptoms, including loss of sensation on one side of the body, loss of movement (paralysis) on one side of the body, problems with verbal expression, or problems with vision.
The exact symptoms will depend on what area of the brain is damaged. Headaches can occur in 20-25 percent of lupus patients. These are most like migraine headaches that are pounding, associated with nausea and vomiting, and cause photophobia (light hurting the eyes). Other much rarer complications due to brain damage relate to problems with coordinated movements. Brain stem involvement with lupus also causes a variety of symptoms. These are primarily due to involvement of the nerves to the muscles of the head and of the sensory pathways from the face. Problems with movement of the eyes can cause double vision. Inflammation of the nerve for vision can cause blurring of vision.
Sensation or movement of the face can be altered, causing numbness or tingling or weakness of the face. In addition, other symptoms of brain stem involvement include hearing problems, slurred speech, and trouble swallowing. Since the longer nerve pathways that carry motor and sensory information between the brain and spinal cord may also be damaged in the brain stem, there may be sensory and motor symptoms, not only of the head and face, but also in the arms and legs. Spinal cord involvement is very rare. When it does occur, it is called "myelopathy" and patients may not be able to move their arms and legs depending on what section of the spinal cord is involved. In addition, sensation is also changed below the area of the spinal cord that is involved. The patient may not feel pain or temperature properly. Other sensations may also be affected such as the ability to feel a vibration or to sense the body's position.
Lupus can imitate inflammation of the spinal cord that is usually due to other diseases such as multiple sclerosis. These symptoms require careful examination to determine their cause. The peripheral nervous system includes the rest of the motor and sensory nerves and the muscles. It includes the nerves that come from the spinal cord to all parts of the body. The peripheral motor nerves carry motor information (originating in the brain) from the spinal cord to the muscle. The muscle will then contract so movement can occur. The sensory fibers carry sensory information from the skin, joints, and muscle to the spinal cord, where it can then be relayed back to the brain. Damage to the peripheral nerves occurs in 10-15 percent of patients with lupus and can result in numbness (pins and needles sensation) of the feet and hands, an inability to feel a pin prick, temperature change, vibration, or change in position, and weakness. If this peripheral nerve damage is severe, patients may inadvertently hurt themselves without knowing it.
If this becomes a frequent occurrence, it can lead to infections, ulcerations, and joint problems. Sometimes individual peripheral nerves to the arm or leg can be damaged. This results in more local sensation and movement problems. Finally, some types of sensation may be more affected than others. If position sense is altered, difficulties with walking may result. This is due to an inability to sense where the feet are being placed. In addition to peripheral nerve involvement, the muscle can also be involved with inflammation or other nonspecific damage. This occurs in 5-30 percent of patients. Patients with muscle problems will experience weakness of the thighs and shoulders. They may have trouble arising from chairs or going up stairs. There will be complaints related to combing the hair and putting things up on a shelf. There may also be some muscle tenderness.
When patients with lupus are clearly weak, other factors should also be considered, i.e. steroid-induced weakness and weakness due to other problems from lupus. Kidney disease, poor nutrition, or immobility can all lead to muscle weakness. When neurologic complications begin, the first step is to identify lupus as the causative factor and make sure all other possible causes are considered (i.e., infection, anemia, kidney failure, drug toxicities). If they are present, these other causes should then be treated appropriately. On the other hand, if the complication is primarily due to lupus, then treatment of the lupus itself is necessary.
=========================================================== This information is for "informational purposes" and is not meant to be used for medical diagnosis. Always consult your physician on matters such as this.
Terry D. Heiman-Patterson, MD
LEARNING ABOUT LUPUS: A USER FRIENDLY GUIDEFrom the Lupus Foundation of Delaware Valley, Inc.Edited by: Mary E. Moore, Ph.D., M.D., Carolyn H. McGrory, MS, RN, Robert S. Rosenthal, M.D.
Systemic lupus etythematosus (SLE) is a multisystem disorder that frequently affects the nervous system. In fact, of all the connective tissue disorders, lupus has the highest incidence of nervous system complications. These neurologic complications may occur in 30-75 percent of patients. In order to understand the variety of complications, one must have some notion of what makes up the nervous system.
The nervous system contains the brain, brain stem, spinal cord, nerves outside the brain and spinal column (peripheral nerves), and muscle. The brain has centers for speech, movement, sensation, and also more complex functions such as thinking, memory, and personality. The brain relays commands and messages to other areas of the nervous system and receives information from these areas, as well. The brain blends together all this information so that persons can interact with their environment. Between the brain and the spinal cord is the brain stem. The brain stem contains nerve cells that control the muscles of eye movement, facial expression, speaking, swallowing, hearing and balance. The spinal cord contains nerve cells that supply impulses to and from the muscles (i.e., innervate the muscles), and that receive sensory information from the skin and joints.
Muscles attach to bones and when they contract they enable a person to move. Information travels from the brain to the brain stem and spinal cord where contact is made with the nerve cells, (i.e. the cranial and peripheral nerves) innervating muscles of the head and arms and legs. Sensory information is also relayed from these areas through contacts in the spinal cord and brain stem to the sensory centers of the brain. There are several possible ways that lupus can damage the nervous system. First, the blood vessels of the brain may themselves be damaged, leading to poor blood flow, to death of brain tissue (strokes), or to bleeding (hemorrhage). There may be an immune system attack on the nervous system because of antibodies that are produced. These can cause problems directly by attacking nerve cells (antineuronal antibodies) or indirectly by causing blood clots in the brain (lupus anticoagulants or anticardiolipin antibodies).
When lupus affects the nervous system, the type of symptom and the distribution of complaints will depend on what area of the nervous system is involved and on what sensory and motor pathways are damaged. When there is more general involvement of the brain, a patient may demonstrate personality changes and psychiatric disorders. Actual psychosis (severe mental illness) can occur, or mood changes such as depression, or even an unnatural feeling of well-being (euphoria) can occur. Other patients may experience anxiety that is out of proportion to the problems they are experiencing. in all these psychiatric disorders, thinking and memory are normal. However, some patients may actually have loss of intellectual skills (i.e., dementia), or experience confusion and altered consciousness, (sleepiness and even coma). Although confusion and altered consciousness may occur because of direct involvement of the brain by lupus, it can also occur because of drugs used in the treatment of lupus (i.e. steroids) or because of other complications from lupus (i.e. infection, kidney failure).
In these instances, withdrawal of the drug, treatment of the infection, or management of the other systemic complications will help to clear the confusion and improve alertness. Another complication due to brain involvement is having seizures or convulsions. These can occur in up to 20% of patients. In patients with kidney failure due to lupus, the rate of seizures rises to almost 50 percent. Lupus can also cause strokes in which there is a sudden onset of neurologic symptoms. Strokes can be due to bleeding into the brain or to death of brain tissue because of lack of blood flow. These problems occur if there is inflammation of blood vessels, clotting of blood within vessels, or not enough clotting of blood causing patients to hemorrage more readily. Patients with strokes may have a variety of symptoms, including loss of sensation on one side of the body, loss of movement (paralysis) on one side of the body, problems with verbal expression, or problems with vision.
The exact symptoms will depend on what area of the brain is damaged. Headaches can occur in 20-25 percent of lupus patients. These are most like migraine headaches that are pounding, associated with nausea and vomiting, and cause photophobia (light hurting the eyes). Other much rarer complications due to brain damage relate to problems with coordinated movements. Brain stem involvement with lupus also causes a variety of symptoms. These are primarily due to involvement of the nerves to the muscles of the head and of the sensory pathways from the face. Problems with movement of the eyes can cause double vision. Inflammation of the nerve for vision can cause blurring of vision.
Sensation or movement of the face can be altered, causing numbness or tingling or weakness of the face. In addition, other symptoms of brain stem involvement include hearing problems, slurred speech, and trouble swallowing. Since the longer nerve pathways that carry motor and sensory information between the brain and spinal cord may also be damaged in the brain stem, there may be sensory and motor symptoms, not only of the head and face, but also in the arms and legs. Spinal cord involvement is very rare. When it does occur, it is called "myelopathy" and patients may not be able to move their arms and legs depending on what section of the spinal cord is involved. In addition, sensation is also changed below the area of the spinal cord that is involved. The patient may not feel pain or temperature properly. Other sensations may also be affected such as the ability to feel a vibration or to sense the body's position.
Lupus can imitate inflammation of the spinal cord that is usually due to other diseases such as multiple sclerosis. These symptoms require careful examination to determine their cause. The peripheral nervous system includes the rest of the motor and sensory nerves and the muscles. It includes the nerves that come from the spinal cord to all parts of the body. The peripheral motor nerves carry motor information (originating in the brain) from the spinal cord to the muscle. The muscle will then contract so movement can occur. The sensory fibers carry sensory information from the skin, joints, and muscle to the spinal cord, where it can then be relayed back to the brain. Damage to the peripheral nerves occurs in 10-15 percent of patients with lupus and can result in numbness (pins and needles sensation) of the feet and hands, an inability to feel a pin prick, temperature change, vibration, or change in position, and weakness. If this peripheral nerve damage is severe, patients may inadvertently hurt themselves without knowing it.
If this becomes a frequent occurrence, it can lead to infections, ulcerations, and joint problems. Sometimes individual peripheral nerves to the arm or leg can be damaged. This results in more local sensation and movement problems. Finally, some types of sensation may be more affected than others. If position sense is altered, difficulties with walking may result. This is due to an inability to sense where the feet are being placed. In addition to peripheral nerve involvement, the muscle can also be involved with inflammation or other nonspecific damage. This occurs in 5-30 percent of patients. Patients with muscle problems will experience weakness of the thighs and shoulders. They may have trouble arising from chairs or going up stairs. There will be complaints related to combing the hair and putting things up on a shelf. There may also be some muscle tenderness.
When patients with lupus are clearly weak, other factors should also be considered, i.e. steroid-induced weakness and weakness due to other problems from lupus. Kidney disease, poor nutrition, or immobility can all lead to muscle weakness. When neurologic complications begin, the first step is to identify lupus as the causative factor and make sure all other possible causes are considered (i.e., infection, anemia, kidney failure, drug toxicities). If they are present, these other causes should then be treated appropriately. On the other hand, if the complication is primarily due to lupus, then treatment of the lupus itself is necessary.
=========================================================== This information is for "informational purposes" and is not meant to be used for medical diagnosis. Always consult your physician on matters such as this.
Lupus and the Kidney
Lupus and the Kidney
Alan G. Wasserstein, M.D.
LEARNING ABOUT LUPUS: A USER FRIENDLY GUIDEFrom the Lupus Foundation of Delaware Valley, Inc.Edited by: Mary E. Moore, Ph.D., M.D., Carolyn H. McGrory, MS, RN, Robert S. Rosenthal, M.D.
Lupus does not always cause kidney disease, but when it does, kidney involvement can be one of its most significant problems. In all of these cases, the kidney problems are due to the production of abnormal autoantibodies. These antibodies are directed against the patient's own tissue, for example, against DNA, the material of which the genes are made. The formation of immune complexes (combinations of these antibodies joining with normal body substances) appears to set up an inflammatory reaction in the kidney.
Several serious clinical disorders can result. The kidney may leak large amounts of protein, a condition called the nepbrotic syndrome. In the nephrotic syndrome, the ability of the kidneys to remove salt and water from the body is impaired. Excess fluid accumulates in the legs and abdomen and around the eyes, causing discomfort and inconvenience. Patients can not fit into their clothes or shoes. Diuretics are usually prescribed to help eliminate the excess fluid by increasing urination. The most feared complication is kidney failure. Kidney failure may or may not be preceded by nephrotic syndrome. Minor abnormalities in the results of urine or blood tests often provide the clue that damage to the kidney exists. Such abnormalities include excessive amounts of protein in the urine, red blood cells in the urine, or a slight loss of kidney function indicated by a rise in the serum creatinine (a blood study).
Sometimes kidney damage is more severe than the clinical symptoms reveal. A kidney biopsy should be done early if such problems are suspected. If kidney damage caused by lupus is diagnosed and treated early, the treatment is usually more effective. There is no doubt that the biopsy is the best way to determine the extent of disease and the need for treatment. After the kidney has been located with ultrasound or a CAT scan, a biopsy is done with the patient lying on his or her abdomen. Only a local anesthetic is required. A tiny cylinder of kidney tissue is withdrawn with a special needle. This tissue is examined under a microscope. Since there is a risk of bleeding from the kidney, the patient must stay in the hospital for observation for 12 hours or overnight following the biopsy.
A treatment plan is developed on the basis of the biopsy. Mild abnormalities are present in practically all lupus patients, even those with no other clinical or laboratory evidence of kidney involvement. These do not require treatment. Corticosteroids, such as prednisone, have been the basis of treatment for a long time. Their use may result in some improvement in lupus kidney disease, but the improvement is not as long lasting as we would like. (They are very effective, however, for other lupus problems such as arthritis, rash, fever, etc.) A significant advance in the treatment of lupus-related kidney disease has been the use of immunosuppressive drugs, specifically Cytoxan (cyclophosphamide).
Immunosuppressive drugs reduce the production of antibodies which cause inflammation. Cytoxan is usually given in combination with prednisone. In many cases it has led to clear improvement in lupus kidney disease. This has been demonstrated by observing improvement in biopsy specimens, reduction in the amount of protein in the urine, and increase in kidney function. Cytoxan is a toxic drug, however, which has many side effects. These include a decrease in the white blood cell count which makes a patient more susceptible to bacterial and other infections. It can also cause bleeding from the bladder (hemorrhagic cystitis), hair loss, sterility, and, years later, some patients may even develop cancer from its use. Sometimes kidney failure occurs very rapidly in patients with lupus (acute renal failure). in these cases, extremely large doses of corticosteroids and/or Cytoxan given intravenously (pulse therapy) may be helpful.
Patients who do not respond to treatment may progress to kidney failure, requiring dialysis or a kidney transplant. Although these are unfortunate outcomes, both dialysis and kidney transplants may have favorable results. Patients who have kidney failure which is advanced enough to require dialysis develop a natural immunosuppression and their lupus improves. As a result, dialysis patients with lupus do as well as other dialysis patients without systemic disease. Similarly, there has been success with kidney transplants in lupus patients. For the best outcome, a patient's lupus should be in remission at the time of the transplant. Kidneys for transplantation may come from either relatives or organ donors. In order to minimize the chance of rejection, the tissue type of the donated kidney should match the tissue type of the patient as closely as possible.
Cyclosporine, a new immunosuppressive drug given to kidney transplant patients, has significantly improved the success rate of transplants. Once the new kidney has been accepted by the body, recurrence of lupus in the kidney is rare.
=========================================================== This information is for "informational purposes" and is not meant to be used for medical diagnosis. Always consult your physician on matters such as this.
Alan G. Wasserstein, M.D.
LEARNING ABOUT LUPUS: A USER FRIENDLY GUIDEFrom the Lupus Foundation of Delaware Valley, Inc.Edited by: Mary E. Moore, Ph.D., M.D., Carolyn H. McGrory, MS, RN, Robert S. Rosenthal, M.D.
Lupus does not always cause kidney disease, but when it does, kidney involvement can be one of its most significant problems. In all of these cases, the kidney problems are due to the production of abnormal autoantibodies. These antibodies are directed against the patient's own tissue, for example, against DNA, the material of which the genes are made. The formation of immune complexes (combinations of these antibodies joining with normal body substances) appears to set up an inflammatory reaction in the kidney.
Several serious clinical disorders can result. The kidney may leak large amounts of protein, a condition called the nepbrotic syndrome. In the nephrotic syndrome, the ability of the kidneys to remove salt and water from the body is impaired. Excess fluid accumulates in the legs and abdomen and around the eyes, causing discomfort and inconvenience. Patients can not fit into their clothes or shoes. Diuretics are usually prescribed to help eliminate the excess fluid by increasing urination. The most feared complication is kidney failure. Kidney failure may or may not be preceded by nephrotic syndrome. Minor abnormalities in the results of urine or blood tests often provide the clue that damage to the kidney exists. Such abnormalities include excessive amounts of protein in the urine, red blood cells in the urine, or a slight loss of kidney function indicated by a rise in the serum creatinine (a blood study).
Sometimes kidney damage is more severe than the clinical symptoms reveal. A kidney biopsy should be done early if such problems are suspected. If kidney damage caused by lupus is diagnosed and treated early, the treatment is usually more effective. There is no doubt that the biopsy is the best way to determine the extent of disease and the need for treatment. After the kidney has been located with ultrasound or a CAT scan, a biopsy is done with the patient lying on his or her abdomen. Only a local anesthetic is required. A tiny cylinder of kidney tissue is withdrawn with a special needle. This tissue is examined under a microscope. Since there is a risk of bleeding from the kidney, the patient must stay in the hospital for observation for 12 hours or overnight following the biopsy.
A treatment plan is developed on the basis of the biopsy. Mild abnormalities are present in practically all lupus patients, even those with no other clinical or laboratory evidence of kidney involvement. These do not require treatment. Corticosteroids, such as prednisone, have been the basis of treatment for a long time. Their use may result in some improvement in lupus kidney disease, but the improvement is not as long lasting as we would like. (They are very effective, however, for other lupus problems such as arthritis, rash, fever, etc.) A significant advance in the treatment of lupus-related kidney disease has been the use of immunosuppressive drugs, specifically Cytoxan (cyclophosphamide).
Immunosuppressive drugs reduce the production of antibodies which cause inflammation. Cytoxan is usually given in combination with prednisone. In many cases it has led to clear improvement in lupus kidney disease. This has been demonstrated by observing improvement in biopsy specimens, reduction in the amount of protein in the urine, and increase in kidney function. Cytoxan is a toxic drug, however, which has many side effects. These include a decrease in the white blood cell count which makes a patient more susceptible to bacterial and other infections. It can also cause bleeding from the bladder (hemorrhagic cystitis), hair loss, sterility, and, years later, some patients may even develop cancer from its use. Sometimes kidney failure occurs very rapidly in patients with lupus (acute renal failure). in these cases, extremely large doses of corticosteroids and/or Cytoxan given intravenously (pulse therapy) may be helpful.
Patients who do not respond to treatment may progress to kidney failure, requiring dialysis or a kidney transplant. Although these are unfortunate outcomes, both dialysis and kidney transplants may have favorable results. Patients who have kidney failure which is advanced enough to require dialysis develop a natural immunosuppression and their lupus improves. As a result, dialysis patients with lupus do as well as other dialysis patients without systemic disease. Similarly, there has been success with kidney transplants in lupus patients. For the best outcome, a patient's lupus should be in remission at the time of the transplant. Kidneys for transplantation may come from either relatives or organ donors. In order to minimize the chance of rejection, the tissue type of the donated kidney should match the tissue type of the patient as closely as possible.
Cyclosporine, a new immunosuppressive drug given to kidney transplant patients, has significantly improved the success rate of transplants. Once the new kidney has been accepted by the body, recurrence of lupus in the kidney is rare.
=========================================================== This information is for "informational purposes" and is not meant to be used for medical diagnosis. Always consult your physician on matters such as this.
Sunday, January 22, 2006
The Skin, Sunlight and Lupus
LEARNING ABOUT LUPUS: A USER FRIENDLY GUIDEFrom the Lupus Foundation of Delaware Valley, Inc.Edited by: Mary E. Moore, Ph.D., M.D., Carolyn H. McGrory, MS, RN, Robert S. Rosenthal, M.D.
The Skin, Sunlight and Lupus
Warren R. Heymann, MD
Sensitivity to the sun from exposure to ultraviolet light (either sunlight or artificial sources) is one of the symptoms the American College of Rheumatology uses to diagnose systemic lupus erythematosus. This chapter will review the effects of lupus on the skin and will explore what is known about the relationship between ultraviolet light and lupus. it will also discuss what the lupus patient should do to avoid the damaging effects of the sun.
The cause of lupus is unknown. Many factors may be involved in the clinical picture of lupus in a particular individual. Exposure to sunlight may play an important role either in the start of lupus in some patients or in causing flares of existing disease in others. It is important to realize that many lupus patients are not negatively affected by the sun. For this reason lupus has been called a "photoaggravated" disorder or one which may be, but is not always, made worse by the sun. Lupus represents a wide range of disease. At the less severe end of the range is discoid (chronic cutaneous) lupus.
Generally, patients with discoid lupus have few problems which affect the whole body. Most patients with discoid lupus have skin problems on the head, scalp, and neck. First these appear as red, swollen plaques (a raised circular patch of skin) while later the patients experience thinning of the skin, scarring, changes in skin color and "plugging" of the hair follicles.
Aggravation of discoid lupus by the sun is quite variable but may occur in up to 50% of patients. The more severe form of lupus is systemic lupus erythematosus. Symptoms affecting the skin are the "butterfly" rash on the nose and cheeks, hives, a net-like pattern of redness on the arms and legs ("livedo reticularis"), dilated blood vessels of the finger and toe nail folds ("periungual telangiectasias"), hair loss and ulcers of the lining (mucous membrane) of the mouth, nose, or the vagina. Sun sensitivity may occur in lupus after even mild sun exposure.
It is also possible to find sun sensitivity in a less severe form of lupus called subacute cutaneous lupus. The skin findings in this condition may resemble psoriasis or have a ring-like (annular) shape. These lesions are found in areas exposed to sunlight (i.e.. face. upper chest, upper back, and sides of the arms). Systemic findings are mild and are usually limited to joint and muscle pains. it should be emphasized that because lupus involves a range of symptoms, an individual may have any of the skin or systemic features.
Although it is evident that lupus may be negatively affected by ultraviolet radiation, little is known about how this actually occurs, Most experts agree that ultraviolet B radiation, the "sunburn" rays, (290-320 nm) are most important. There is some evidence, however, that ultraviolet A radiation, the "tanning" rays, (320-400 nm) may also result in sun sensitivity reactions. The theory behind this is that ultraviolet light may alter the DNA (deoxyribonucleic acid) of the uppermost layer of the skin (epidermal cells) so that DNA causes an immune response. DNA is the material of which genes are made. DNA which is not exposed to the sun's rays does not cause this immune response, while sun-exposed DNA does.
It is also possible that sunburn may act as a non-specific trauma or injury. Other environmental agents such as cold, heat, and wind may also aggravate lupus or cause it to begin in a previously well person. Individuals affected by lupus should learn to protect their skin from the sun. By practicing common sense measures, the sun need not be strictly avoided and the out-of-doors may be enjoyed. The midday sun should be avoided (between 11 A.M. and 2 P.M.). Use of a broad-rimmed hat or staying in the shade is encouraged, and the use of sunscreens should become a daily habit. A sunscreen with a sun protection factor (SPF) of at least 15 is recommended for the summer months.
Remember that snow reflects sunlight and if the individual likes to be outside throughout the year, use of sunscreens should be continued in all seasons. Tanning parlors are absolutely taboo for the lupus patient. It is apparent that ultraviolet rays may affect all forms of lupus, especially systemic lupus erythematosus. Although it is not understood exactly how this occurs, it is clear that by fairly simple precautions the damaging effects of sunlight on patients with lupus may be diminished or avoided altogether.
=========================================================== This information is for "informational purposes" and is not meant to be used for medical diagnosis. Always consult your physician on matters such as this.
The Skin, Sunlight and Lupus
Warren R. Heymann, MD
Sensitivity to the sun from exposure to ultraviolet light (either sunlight or artificial sources) is one of the symptoms the American College of Rheumatology uses to diagnose systemic lupus erythematosus. This chapter will review the effects of lupus on the skin and will explore what is known about the relationship between ultraviolet light and lupus. it will also discuss what the lupus patient should do to avoid the damaging effects of the sun.
The cause of lupus is unknown. Many factors may be involved in the clinical picture of lupus in a particular individual. Exposure to sunlight may play an important role either in the start of lupus in some patients or in causing flares of existing disease in others. It is important to realize that many lupus patients are not negatively affected by the sun. For this reason lupus has been called a "photoaggravated" disorder or one which may be, but is not always, made worse by the sun. Lupus represents a wide range of disease. At the less severe end of the range is discoid (chronic cutaneous) lupus.
Generally, patients with discoid lupus have few problems which affect the whole body. Most patients with discoid lupus have skin problems on the head, scalp, and neck. First these appear as red, swollen plaques (a raised circular patch of skin) while later the patients experience thinning of the skin, scarring, changes in skin color and "plugging" of the hair follicles.
Aggravation of discoid lupus by the sun is quite variable but may occur in up to 50% of patients. The more severe form of lupus is systemic lupus erythematosus. Symptoms affecting the skin are the "butterfly" rash on the nose and cheeks, hives, a net-like pattern of redness on the arms and legs ("livedo reticularis"), dilated blood vessels of the finger and toe nail folds ("periungual telangiectasias"), hair loss and ulcers of the lining (mucous membrane) of the mouth, nose, or the vagina. Sun sensitivity may occur in lupus after even mild sun exposure.
It is also possible to find sun sensitivity in a less severe form of lupus called subacute cutaneous lupus. The skin findings in this condition may resemble psoriasis or have a ring-like (annular) shape. These lesions are found in areas exposed to sunlight (i.e.. face. upper chest, upper back, and sides of the arms). Systemic findings are mild and are usually limited to joint and muscle pains. it should be emphasized that because lupus involves a range of symptoms, an individual may have any of the skin or systemic features.
Although it is evident that lupus may be negatively affected by ultraviolet radiation, little is known about how this actually occurs, Most experts agree that ultraviolet B radiation, the "sunburn" rays, (290-320 nm) are most important. There is some evidence, however, that ultraviolet A radiation, the "tanning" rays, (320-400 nm) may also result in sun sensitivity reactions. The theory behind this is that ultraviolet light may alter the DNA (deoxyribonucleic acid) of the uppermost layer of the skin (epidermal cells) so that DNA causes an immune response. DNA is the material of which genes are made. DNA which is not exposed to the sun's rays does not cause this immune response, while sun-exposed DNA does.
It is also possible that sunburn may act as a non-specific trauma or injury. Other environmental agents such as cold, heat, and wind may also aggravate lupus or cause it to begin in a previously well person. Individuals affected by lupus should learn to protect their skin from the sun. By practicing common sense measures, the sun need not be strictly avoided and the out-of-doors may be enjoyed. The midday sun should be avoided (between 11 A.M. and 2 P.M.). Use of a broad-rimmed hat or staying in the shade is encouraged, and the use of sunscreens should become a daily habit. A sunscreen with a sun protection factor (SPF) of at least 15 is recommended for the summer months.
Remember that snow reflects sunlight and if the individual likes to be outside throughout the year, use of sunscreens should be continued in all seasons. Tanning parlors are absolutely taboo for the lupus patient. It is apparent that ultraviolet rays may affect all forms of lupus, especially systemic lupus erythematosus. Although it is not understood exactly how this occurs, it is clear that by fairly simple precautions the damaging effects of sunlight on patients with lupus may be diminished or avoided altogether.
=========================================================== This information is for "informational purposes" and is not meant to be used for medical diagnosis. Always consult your physician on matters such as this.
Genetics and Lupus
Genetics and Lupus
Raphael J. DeHoratius, MD
The most frequently asked question about lupus is, "Is lupus inherited?" The answer to this question is both "yes" and "no"! Genes, those parts of our individual make-up that we inherit from our parents, are important in the development of lupus, but the answer is much more complicated than a simple "yes". Estimates are that from four to six or more genes must be combined for a person to inherit a susceptibility to acquire lupus.
It is nearly impossible to inherit all the genes necessary to develop lupus from a single parent, since an individual's genes come from both parents. This is one very important reason why it is unusual for lupus to occur in multiple generations of a family. If only some of the lupus genes are inherited, a person may not have lupus but may test positive for some of the immunologic tests, such as the antinuclear antibody (ANA).
A positive ANA occurs in up to one third of healthy family members of lupus patients.Genetic information is coded in chromosomes which are located in humans in a tiny part of the center (nucleus) of each cell. Humans have 46 chromosomes, each of which is made up of thousands of genes. Each chromosome is divided into a long and short arm. Most of the important genes in systemic lupus erythematosus are located on the short arm of chromosome #6. The genes on chromosome #6 have many complex functions.
Some regulate complement components (proteins important in acute and chronic inflammation and in the formation of immune complexes). When these complement components are missing, a milder form of lupus, which usually lacks kidney involvement, may develop. Complement genes are important but they are not the whole story in the development of this form of lupus.
For example, many susceptible individuals who lack these genes for complement never develop lupus at all.Another important area on the short arm of chromosome #6 is the HLA (human leukocyte antigen) region. It is located next to the area for complement genes. The HLA area has been very thoroughly studied since it is used to match donors genetically to recipients for organ transplants. it is further divided into smaller regions called HLA-A, HLA-B, HLA-C, HLA-DR, HLA-DQ. In lupus patients there is an increased frequency of the HLA genes called Al, B8, Dr2, or Dr-3 and DQ1.Associations between genes and diseases such as lupus are established by comparing lupus patients to a normal or "control" population.
Particular HLA markers found in white lupus patients (on whom the majority of studies have focused) have not been shown to be present in black patients or Japanese patients with lupus. The reasons for these differences are not clear. There may be other as yet unknown genes or there may also be important genes on other chromosomes which play a part in making a person susceptible to developing lupus.
The newest research methods now being used to study genetics come from the field of molecular biology. They are redefining the way in which we look at the genetics of disease. When methods of molecular biology are used to study the HLA system in various diseases, we are finding that what looked like a specific HLA type, by our current standard tests, in reality is slightly different and Much more complex. This methodology should lead to new important findings, both in genetics and in lupus.
Another way of studying the genetics of lupus is by looking at families in which lupus occurs in more than one member. Familial cases are reported in approximately 10% of the lupus population. The most thoroughly studied family association is between twins. If one of a pair of identical twins (twins with exactly the same genes) has lupus, the other will develop it more than two thirds (69%) of the time. If a fraternal or non-identical twin (a twin with genes no more similar to his twin than to any other brother or sister) has lupus, the other twin has only a 5% chance of developing it. It is obvious that genetics are important, since the frequency of developing lupus is so much higher in identical twins than in fraternal twins when one of the twins already has lupus.
Genetic factors cannot be the only answers, however, or susceptible identical twins would both develop lupus 100% of the time. Environmental factors, therefore, must also be important. It appears that some people are genetically predisposed to develop lupus but then must be exposed to the proper environmental triggers in order to have the disease.In summary, heredity is involved in the development of lupus but it is rare to have more than one family member who has lupus.
Much is known about the genetics of lupus, yet even more needs to be discovered. It is only through careful family studies using molecular biological techniques that the answer to the genetic riddle of systemic lupus erythematosus and the relationship between heredity and environment will be solved.
=========================================================== This information is for "informational purposes" and is not meant to be used for medical diagnosis. Always consult your physician on matters such as this.
Raphael J. DeHoratius, MD
The most frequently asked question about lupus is, "Is lupus inherited?" The answer to this question is both "yes" and "no"! Genes, those parts of our individual make-up that we inherit from our parents, are important in the development of lupus, but the answer is much more complicated than a simple "yes". Estimates are that from four to six or more genes must be combined for a person to inherit a susceptibility to acquire lupus.
It is nearly impossible to inherit all the genes necessary to develop lupus from a single parent, since an individual's genes come from both parents. This is one very important reason why it is unusual for lupus to occur in multiple generations of a family. If only some of the lupus genes are inherited, a person may not have lupus but may test positive for some of the immunologic tests, such as the antinuclear antibody (ANA).
A positive ANA occurs in up to one third of healthy family members of lupus patients.Genetic information is coded in chromosomes which are located in humans in a tiny part of the center (nucleus) of each cell. Humans have 46 chromosomes, each of which is made up of thousands of genes. Each chromosome is divided into a long and short arm. Most of the important genes in systemic lupus erythematosus are located on the short arm of chromosome #6. The genes on chromosome #6 have many complex functions.
Some regulate complement components (proteins important in acute and chronic inflammation and in the formation of immune complexes). When these complement components are missing, a milder form of lupus, which usually lacks kidney involvement, may develop. Complement genes are important but they are not the whole story in the development of this form of lupus.
For example, many susceptible individuals who lack these genes for complement never develop lupus at all.Another important area on the short arm of chromosome #6 is the HLA (human leukocyte antigen) region. It is located next to the area for complement genes. The HLA area has been very thoroughly studied since it is used to match donors genetically to recipients for organ transplants. it is further divided into smaller regions called HLA-A, HLA-B, HLA-C, HLA-DR, HLA-DQ. In lupus patients there is an increased frequency of the HLA genes called Al, B8, Dr2, or Dr-3 and DQ1.Associations between genes and diseases such as lupus are established by comparing lupus patients to a normal or "control" population.
Particular HLA markers found in white lupus patients (on whom the majority of studies have focused) have not been shown to be present in black patients or Japanese patients with lupus. The reasons for these differences are not clear. There may be other as yet unknown genes or there may also be important genes on other chromosomes which play a part in making a person susceptible to developing lupus.
The newest research methods now being used to study genetics come from the field of molecular biology. They are redefining the way in which we look at the genetics of disease. When methods of molecular biology are used to study the HLA system in various diseases, we are finding that what looked like a specific HLA type, by our current standard tests, in reality is slightly different and Much more complex. This methodology should lead to new important findings, both in genetics and in lupus.
Another way of studying the genetics of lupus is by looking at families in which lupus occurs in more than one member. Familial cases are reported in approximately 10% of the lupus population. The most thoroughly studied family association is between twins. If one of a pair of identical twins (twins with exactly the same genes) has lupus, the other will develop it more than two thirds (69%) of the time. If a fraternal or non-identical twin (a twin with genes no more similar to his twin than to any other brother or sister) has lupus, the other twin has only a 5% chance of developing it. It is obvious that genetics are important, since the frequency of developing lupus is so much higher in identical twins than in fraternal twins when one of the twins already has lupus.
Genetic factors cannot be the only answers, however, or susceptible identical twins would both develop lupus 100% of the time. Environmental factors, therefore, must also be important. It appears that some people are genetically predisposed to develop lupus but then must be exposed to the proper environmental triggers in order to have the disease.In summary, heredity is involved in the development of lupus but it is rare to have more than one family member who has lupus.
Much is known about the genetics of lupus, yet even more needs to be discovered. It is only through careful family studies using molecular biological techniques that the answer to the genetic riddle of systemic lupus erythematosus and the relationship between heredity and environment will be solved.
=========================================================== This information is for "informational purposes" and is not meant to be used for medical diagnosis. Always consult your physician on matters such as this.
Saturday, January 07, 2006
LEARNING ABOUT LUPUS: Laboratory Tests in Lupus
LEARNING ABOUT LUPUS: A USER FRIENDLY GUIDE
From the Lupus Foundation of Delaware Valley, Inc.
Edited by: Mary E. Moore, Ph.D., M.D., Carolyn H. McGrory, MS, RN, Robert S. Rosenthal, M.D.
Laboratory Tests in Lupus
Burton Zweiman, MD
Most physicians with a lot of experience in the care of lupus patients can make the diagnosis of systemic lupus erythematosus based upon certain symptoms and findings when they take a medical history and do a physical examination. Sometimes, however, these physical symptoms or clinical clues are not well defined early in the course of lupus and, therefore, certain laboratory tests are useful in making the diagnosis.
Many people ask, "is there a laboratory test that can diagnose lupus in all patients?" Despite much research in this area, the answer must still be "No". There is no one test which can do this. The laboratory test performed most often is the immunofluorescence test for antinuclear antibodies (ANA). This is often simply called the ANA test. It has almost entirely replaced the older LE cell test which is more time consuming to perform and is less accurate. The ANA test is a very sensitive one. Over ninety-five percent of untreated lupus patients have high ANA levels in their blood.
A small number of individuals have an unusual lupus disorder with a negative ANA test, a rash and a high sensitivity to sunlight. For most people, however, an ANA test with negative results in several repeated tests performed in a good laboratory is strong evidence against the diagnosis of systemic lupus. The ANA test is not specific for lupus. This means that people with other diseases can also have a high ANA. Elevated blood ANA levels are found in a number of other disorders including some with symptoms similar to lupus. Sometimes this leads to confusion and a diagnosis of lupus is made in someone with another disorder just because the ANA test is abnormal. Much research has been done to expand and refine the ANA test in order to help find a test more specific for lupus. Although the ANA levels in the blood are generally higher in untreated lupus patients than in patients with other diseases, this is not always so.
A diagnosis of lupus, therefore, cannot be made only on the basis of a high ANA. It is helpful to know in diagnosing lupus that the ANA is actually a group of antibodies directed against different parts of cells within our bodies. in laboratories with special equipment, these antibodies can now be detected individually. Some of these individual antibodies are very specific for the diagnosis of lupus. Some of these are listed below:
Anti-ds (double-stranded) DNA antibodies. These are substances which react to the material which makes up the genes found in cells. Double stranded DNA also plays a key role in the growth and multiplication of these cells. increased blood levels of antidsDNA antibodies are found in about 70% of lupus patients, and found very infrequently in other disorders. However, the dsDNA used in the test must be prepared very carefully so that it does not contain single stranded DNA. Antibodies against this single stranded DNA are commonly found in disorders other than lupus, and can confuse the results of the test. Anti-dsDNA antibodies are more commonly found in lupus patients whose disease is active, particularly when the disease involves the kidneys or the central nervous system.
Anti-Sm (Smith) antibodies. These antibodies are named after the patient, a person named Smith, in whom they were first found. They react with another part of the cell nucleus, the central part of the cell, and are found in about 30% of lupus patients. Anti-Sm antibodies are found very rarely in disorders other than lupus.
Anti-Ro (or SS-A) antibodies. These are found in some lupus patients, particularly those with a certain type of sun-sensitivity rash. If a pregnant lupus patient has anti-Ro antibodies, it is more likely that her baby will have a certain type of congenital heart disorder.
Anti-Ro antibodies are also found in a disorder called Sjogren's syndrome. Sometimes lupus can occur together with Sjogren's syndrome. Antihistone antibodies. These antibodies are found in the blood of many lupus patients and are directed against a protein which is frequently attached to the DNA (the material of which genes are made) within the cell nucleus. These antibodies are of particular interest because they are also found in the blood of some people who have high ANA tests caused by taking certain medications. Recent evidence suggests that the antibodies in lupus patients react with different histones than do the antibodies in these individuals with high ANA tests related to medications.
Anti-RNP (ribonucleoprotein) antibodies. These antibodies occur commonly in lupus and some other disorders. Certain individuals (mainly women) develop a group of symptoms that do not point strongly to lupus or to one of the other connective tissue inflammatory diseases, but rather to a combination of several of these diseases. This has been called "mixed connective tissue disease" or "overlap syndrome". Anti-RNP antibody levels are often very high in this disorder.
Several other antibody tests are often performed when trying to make a diagnosis of lupus because they help tell the difference between lupus and certain other diseases. Antiscleroderma 70 (Scl70) antibody is found in one form of scleroderma. Anticentromere antibodies are found in another form of scleroderma. Anti-PM-1 and anti-Jo-1 antibodies are found in poliomyelitis. Rheumatoid factor (RF) is often found in the blood of patients with rheumatoid arthritis, a condition which can sometimes be confused with lupus.
Rheumatoid factor may also be found in the blood of about 20% of lupus patients, and in a number of other disorders. The levels of certain non-antibody proteins in the blood, called complement components, may be low in lupus patients, particularly when the disease is active. Low complement levels are not very helpful in diagnosing lupus, however, because they can be found in other diseases as well. Complement levels are more useful both in following the disease activity and the response to treatment of individual lupus patients. There are many laboratory tests which measure and identify whether or not specific organ systems are affected by luptis. These can be very valuable in the care of the individual lupus patient.
These will be discussed in other chapters dealing with involvement of individual organ systems in lupus but several examples are described briefly here.Kidney involvement in lupus can be determined by measuring the amount of protein found in the urine during a 24 hour period. The 24 hour urine collection can also be used to find out if there is any decrease in the filtering function of the kidneys and to check for growth of bacteria if an infection of the urinary tract is suspected. If enough bacteria grow when the urine is cultured, these bacteria can also be tested to help determine the best way to treat the infection.
In certain situations it may be necessary to perform a kidney biopsy to find out what type of involvement of the kidney is present. This involves inserting a needle into the kidney under local anesthesia (somewhat like having your jaw numbed to have dental work performed). One or more small pieces of kidney tissue are removed and looked at under the microscope.
Involvement of the central nervous system occurs commonly in lupus, but is often difficult to diagnose. The cerebrospinal fluid, (fluid which is present around the spinal cord and the brain), is abnormal in about 50% of those patients with central nervous system involvement in lupus. X-rays and magnetic resonance imaging (MRI) signals are now also being used to help diagnose involvement of the nervous system in lupus. Other blood tests can also be helpful for lupus patients. Anemia or a low blood count is frequently the result of disease activity. Levels in the blood of leukocytes (white blood cells), and platelets (small blood cells which are part of the clotting mechanism) may also be lower in active lupus. This happens so often that these particular abnormal findings are considered one of the criteria on which the diagnosis of lupus is based. Occasionally the platelet count is so low that a bleeding and bruising tendency may be present. In some individuals with lupus, there are abnormal blood proteins that appear to be antibodies against something in the platelets.
Platelets are involved in normal blood clotting. When these abnormal proteins are present certain blood clotting tests are abnormal (a lupus anticoagulant is detected) or the test for an antibody (anticardiolipin) is positive. What is surprising is that individuals with these antibodies do not have a bleeding tendency.
If anything, there is a tendency to develop blood clots. Women with these antibodies may have spontaneous abortions (miscarriages) if they become pregnant. This is discussed in more detail in Chapter 18 on Lupus and Pregnancy. These antibodies can also result in a false positive test for syphilis. An individual with such antibodies who is tested for syphilis when applying for a marriage license or job, or during routine admission to the hospital, may be told that the test is positive even though they do not in fact have syphilis. The reason for this is that these antibodies against platelets closely resemble the antibody that produces the positive test for syphilis.
Even though these patients do not have syphilis, their blood reacts to the test. Fortunately, this false-positive test can now be distinguished from a true-positive test for syphilis by another, more specific test for syphilis. If a person has repeated normal results using current very sensitive ANA blood tests, this is very strong evidence against a diagnosis of lupus. There is no test which is as specific for lupus as we would like. Results obtained from a group of tests can help the experienced physician distinguish lupus from other conditions with similar symptoms. In some ways they can also help to measure the response to treatment and whether or not disease activity is increasing.
Repeated measurements of certain blood tests may also help determine whether problems with the lupus patient or the fetus are developing during pregnancy. Intensive research is leading to new tests of the immune system. Hopefully, these will lead to easier diagnoses and improved treatment.
===========================================================
This information is for "informational purposes" and is not meant to be used for medical diagnosis. Always consult your physician on matters such as this.
From the Lupus Foundation of Delaware Valley, Inc.
Edited by: Mary E. Moore, Ph.D., M.D., Carolyn H. McGrory, MS, RN, Robert S. Rosenthal, M.D.
Laboratory Tests in Lupus
Burton Zweiman, MD
Most physicians with a lot of experience in the care of lupus patients can make the diagnosis of systemic lupus erythematosus based upon certain symptoms and findings when they take a medical history and do a physical examination. Sometimes, however, these physical symptoms or clinical clues are not well defined early in the course of lupus and, therefore, certain laboratory tests are useful in making the diagnosis.
Many people ask, "is there a laboratory test that can diagnose lupus in all patients?" Despite much research in this area, the answer must still be "No". There is no one test which can do this. The laboratory test performed most often is the immunofluorescence test for antinuclear antibodies (ANA). This is often simply called the ANA test. It has almost entirely replaced the older LE cell test which is more time consuming to perform and is less accurate. The ANA test is a very sensitive one. Over ninety-five percent of untreated lupus patients have high ANA levels in their blood.
A small number of individuals have an unusual lupus disorder with a negative ANA test, a rash and a high sensitivity to sunlight. For most people, however, an ANA test with negative results in several repeated tests performed in a good laboratory is strong evidence against the diagnosis of systemic lupus. The ANA test is not specific for lupus. This means that people with other diseases can also have a high ANA. Elevated blood ANA levels are found in a number of other disorders including some with symptoms similar to lupus. Sometimes this leads to confusion and a diagnosis of lupus is made in someone with another disorder just because the ANA test is abnormal. Much research has been done to expand and refine the ANA test in order to help find a test more specific for lupus. Although the ANA levels in the blood are generally higher in untreated lupus patients than in patients with other diseases, this is not always so.
A diagnosis of lupus, therefore, cannot be made only on the basis of a high ANA. It is helpful to know in diagnosing lupus that the ANA is actually a group of antibodies directed against different parts of cells within our bodies. in laboratories with special equipment, these antibodies can now be detected individually. Some of these individual antibodies are very specific for the diagnosis of lupus. Some of these are listed below:
Anti-ds (double-stranded) DNA antibodies. These are substances which react to the material which makes up the genes found in cells. Double stranded DNA also plays a key role in the growth and multiplication of these cells. increased blood levels of antidsDNA antibodies are found in about 70% of lupus patients, and found very infrequently in other disorders. However, the dsDNA used in the test must be prepared very carefully so that it does not contain single stranded DNA. Antibodies against this single stranded DNA are commonly found in disorders other than lupus, and can confuse the results of the test. Anti-dsDNA antibodies are more commonly found in lupus patients whose disease is active, particularly when the disease involves the kidneys or the central nervous system.
Anti-Sm (Smith) antibodies. These antibodies are named after the patient, a person named Smith, in whom they were first found. They react with another part of the cell nucleus, the central part of the cell, and are found in about 30% of lupus patients. Anti-Sm antibodies are found very rarely in disorders other than lupus.
Anti-Ro (or SS-A) antibodies. These are found in some lupus patients, particularly those with a certain type of sun-sensitivity rash. If a pregnant lupus patient has anti-Ro antibodies, it is more likely that her baby will have a certain type of congenital heart disorder.
Anti-Ro antibodies are also found in a disorder called Sjogren's syndrome. Sometimes lupus can occur together with Sjogren's syndrome. Antihistone antibodies. These antibodies are found in the blood of many lupus patients and are directed against a protein which is frequently attached to the DNA (the material of which genes are made) within the cell nucleus. These antibodies are of particular interest because they are also found in the blood of some people who have high ANA tests caused by taking certain medications. Recent evidence suggests that the antibodies in lupus patients react with different histones than do the antibodies in these individuals with high ANA tests related to medications.
Anti-RNP (ribonucleoprotein) antibodies. These antibodies occur commonly in lupus and some other disorders. Certain individuals (mainly women) develop a group of symptoms that do not point strongly to lupus or to one of the other connective tissue inflammatory diseases, but rather to a combination of several of these diseases. This has been called "mixed connective tissue disease" or "overlap syndrome". Anti-RNP antibody levels are often very high in this disorder.
Several other antibody tests are often performed when trying to make a diagnosis of lupus because they help tell the difference between lupus and certain other diseases. Antiscleroderma 70 (Scl70) antibody is found in one form of scleroderma. Anticentromere antibodies are found in another form of scleroderma. Anti-PM-1 and anti-Jo-1 antibodies are found in poliomyelitis. Rheumatoid factor (RF) is often found in the blood of patients with rheumatoid arthritis, a condition which can sometimes be confused with lupus.
Rheumatoid factor may also be found in the blood of about 20% of lupus patients, and in a number of other disorders. The levels of certain non-antibody proteins in the blood, called complement components, may be low in lupus patients, particularly when the disease is active. Low complement levels are not very helpful in diagnosing lupus, however, because they can be found in other diseases as well. Complement levels are more useful both in following the disease activity and the response to treatment of individual lupus patients. There are many laboratory tests which measure and identify whether or not specific organ systems are affected by luptis. These can be very valuable in the care of the individual lupus patient.
These will be discussed in other chapters dealing with involvement of individual organ systems in lupus but several examples are described briefly here.Kidney involvement in lupus can be determined by measuring the amount of protein found in the urine during a 24 hour period. The 24 hour urine collection can also be used to find out if there is any decrease in the filtering function of the kidneys and to check for growth of bacteria if an infection of the urinary tract is suspected. If enough bacteria grow when the urine is cultured, these bacteria can also be tested to help determine the best way to treat the infection.
In certain situations it may be necessary to perform a kidney biopsy to find out what type of involvement of the kidney is present. This involves inserting a needle into the kidney under local anesthesia (somewhat like having your jaw numbed to have dental work performed). One or more small pieces of kidney tissue are removed and looked at under the microscope.
Involvement of the central nervous system occurs commonly in lupus, but is often difficult to diagnose. The cerebrospinal fluid, (fluid which is present around the spinal cord and the brain), is abnormal in about 50% of those patients with central nervous system involvement in lupus. X-rays and magnetic resonance imaging (MRI) signals are now also being used to help diagnose involvement of the nervous system in lupus. Other blood tests can also be helpful for lupus patients. Anemia or a low blood count is frequently the result of disease activity. Levels in the blood of leukocytes (white blood cells), and platelets (small blood cells which are part of the clotting mechanism) may also be lower in active lupus. This happens so often that these particular abnormal findings are considered one of the criteria on which the diagnosis of lupus is based. Occasionally the platelet count is so low that a bleeding and bruising tendency may be present. In some individuals with lupus, there are abnormal blood proteins that appear to be antibodies against something in the platelets.
Platelets are involved in normal blood clotting. When these abnormal proteins are present certain blood clotting tests are abnormal (a lupus anticoagulant is detected) or the test for an antibody (anticardiolipin) is positive. What is surprising is that individuals with these antibodies do not have a bleeding tendency.
If anything, there is a tendency to develop blood clots. Women with these antibodies may have spontaneous abortions (miscarriages) if they become pregnant. This is discussed in more detail in Chapter 18 on Lupus and Pregnancy. These antibodies can also result in a false positive test for syphilis. An individual with such antibodies who is tested for syphilis when applying for a marriage license or job, or during routine admission to the hospital, may be told that the test is positive even though they do not in fact have syphilis. The reason for this is that these antibodies against platelets closely resemble the antibody that produces the positive test for syphilis.
Even though these patients do not have syphilis, their blood reacts to the test. Fortunately, this false-positive test can now be distinguished from a true-positive test for syphilis by another, more specific test for syphilis. If a person has repeated normal results using current very sensitive ANA blood tests, this is very strong evidence against a diagnosis of lupus. There is no test which is as specific for lupus as we would like. Results obtained from a group of tests can help the experienced physician distinguish lupus from other conditions with similar symptoms. In some ways they can also help to measure the response to treatment and whether or not disease activity is increasing.
Repeated measurements of certain blood tests may also help determine whether problems with the lupus patient or the fetus are developing during pregnancy. Intensive research is leading to new tests of the immune system. Hopefully, these will lead to easier diagnoses and improved treatment.
===========================================================
This information is for "informational purposes" and is not meant to be used for medical diagnosis. Always consult your physician on matters such as this.
Systemic Lupus Erythematosus LEARNING ABOUT LUPUS: A USER FRIENDLY GUIDE
Systemic Lupus Erythematosus LEARNING ABOUT LUPUS: A USER FRIENDLY GUIDE From the Lupus Foundation of Delaware Valley, Inc.
Edited by: Mary E. Moore, Ph.D., M.D., Carolyn H. McGrory, MS, RN, Robert S. Rosenthal, M.D.
Systemic lupus erythematosus (SLE or lupus) is a disease which can affect many parts of the body, can be both acute and chronic, and can cause many different symptoms. The disease can range from very mild to very severe. Among adults, nine females will have lupus for every one male. Lupus most commonly affects people between the ages of twenty and forty and occurs, therefore, most frequently in women during their child-bearing years. Three times as many black as white women are affected. Children and teens can also get lupus.
During the first ten years of life girls will have lupus three to seven times more often than boys. (Childhood lupus is the subject of Chapter 15.)The cause of lupus is not known. The immune system, which is nature's way of protecting us from infection and cancer, works improperly in patients with lupus. Certain white blood cells, the T lymphocytes, aid in control of the immune system. In lupus, these cells fail to fulfill their regulatory function and the immune system becomes active when it is not supposed to. What starts this has not been discovered.
It appears that some foreign invader, some trigger or triggers from the outside must enter the body and set this process in motion. For years the search has gone on in vain to find a virus which could act as such a trigger. We do know that sunlight and some chemicals appear to trigger lupus in some patients, though not in all. We also know that some people have an inherited tendency to develop lupus. Something in their genetic makeup makes them especially sensitive to an environmental trigger. (Chapter 4 discusses recent advances in the genetics of lupus.)To aid in the diagnosis of lupus, the American College of Rheumatology has listed various criteria (guidelines based on symptoms and laboratory tests) to be used in identifying the disease (Table 1). The list contains the problems that are most typical of lupus as compared to other similar diseases.
It is not meant to include all, or even the most common problems a patient with lupus may experience. Persons with lupus most frequently suffer from fatigue, fevers, muscle and joint aching and stiffness, swollen glands, and generalized feelings of being unwell. None of these are listed as criteria for diagnosing SLE, however, because they occur in many other diseases as well. The first of the criteria involve the skin. The best known of the skin problems in lupus is the "butterfly" rash. This is a red rash over the cheeks and the bridge of the nose (the malar area). Several other skin-related problems which commonly occur are sun sensitivity, loss of hair, red plaques (slightly raised patches of skin that have a definite border) associated with scaling and plugging of the hair follicles ("discoid" rash), hives, dryness and ulceration of the mucous membrane (mouth, nose, and vagina), and dilated and broken blood vessels. (These are discussed in more detail in Chapter 5 and referred to in Chapters 12 and Chapter 14.) The circulation of blood to the skin and the underlying tissues is sometimes temporarily decreased in lupus, this is known as Raynaud's phenomenon.
The decrease is most commonly caused by cold temperatures but can also result from psychological stress. The tips of the fingers, the tips of the toes, and occasionally the nose and the ears are affected. These areas first turn white, then blue, and finally, as the circulation returns, red. If the circulation is reduced over a long period of time, the skin may break down and ulcerations (sores) appear.Almost all lupus patients have joint pains at some time during their illness, and many have joint inflammation (swelling, warmth, redness, pain) or arthritis. The type of arthritis associated with lupus is similar to that seen in rheumatoid arthritis but is usually not as severe and does not cause a wearing away of the bone with resulting deformity. It most commonly affects the small joints of the hands, the wrists, the knees, and the feet. Sometimes patients with lupus, especially those on corticosteroids, have involvement of the bone due to a loss of blood supply.
When this occurs in the bone near a joint, degenerative arthritis may result. (See Chapter 13.)Serositis is the inflammation of the delicate tissues which cover certain internal organs and line body compartments. It is quite common in lupus, most often occurring as inflammation of the covering of the lung and the lining of its compartment in the chest (pleuritis), and as inflammation of the covering of the heart (pericarditis). Movement of the involved tissues may cause chest pain, and the doctor can often hear a characteristic "rub" when listening over the inflamed areas with a stethoscope. Abdominal pain in lupus is sometimes caused by inflammation of the covering of the intestines and the lining of the abdominal cavity (peritonitis), and can imitate pancreatitis, diverticulitis, appendicitis, etc. Fluid sometimes accumulates as a result of serositis and may have to be withdrawn by a needle. In the case of pericarditis, an operation to make an opening in the covering of the heart (a "Pericardial window") may be necessary to relieve pressure on the heart from this fluid.One of the most feared complications of lupus is involvement of the kidney.
It is present to some degree in about 50% of cases. It can vary from very mild involvement which causes the patient no symptoms, to severe disease leading to kidney failure and the need for dialysis or kidney transplantation. Doctors detect early kidney problems by discovering protein or blood cells in the urine. They frequently ask the patient to collect 24 hour urine samples so as to be able to make better estimates of the degree to which the kidney is functioning. At times it is necessary to obtain a biopsy of the kidney to look for the presence of antibodies and kidney cell damage. (Lupus and the kidney is further discussed in Chapter 6.)The central nervous system (CNS) may become involved by systemic lupus erythematosus. Such involvement may cause a variety of disorders including seizures, psychosis, paralysis and personality disorders.
Since very similar problems might be the result of infection, hardening of the arteries, or mental illness, etc., and since there is no one test for central nervous system lupus, these disorders may be difficult for the physician to diagnose. (Neurologic problems seen in lupus are discussed in detail in Chapter 7, neuropsychological testing is discussed is Chapter 8 and depression in lupus is discussed in Chapter 10.)One of the frequent disorders in lupus is a decrease in the number of white blood cells, especially the lymphocytes. Since some of the drugs used to treat lupus can also cause a decrease in the white blood cell count, monitoring the safe use of these drugs can, at times, be difficult.
The platelet is another blood cell frequently decreased in lupus. Platelets play a key role in blood clotting and having too few platelets can lead to serious bleeding disorders. Anemia, a decrease in the number of red blood cells, is almost always present in cases of active lupus. It may result from bleeding, from a bone marrow which is depressed by illness and therefore does not produce enough red blood cells, or from the destruction of red blood cells by antibodies. (More about the anemia of lupus in Chapter 11.) When the number of certain T lymphocytes is decreased, the B lymphocytes of the lupus patient are stimulated to manufacture many different antibodies. Among these are antibodies called "autoantibodies" which target the lupus patient's own tissues. The body, in essence, attacks itself. In addition to the widely known antinuclear antibody (ANA), there are many other antibodies, the presence of which aid in the diagnosis of lupus. (See Chapter 2 for a discussion of ANA, anti-DNA, anti-Sm, anti-Ro, anticardiolipin, etc.)Certain drugs in common use may cause antinuclear antibodies to develop and, therefore, cause a mild form of lupus known as drug-induced lupus.
Drug-induced lupus is characterized by muscle pain and arthritis, fever, and pleuritis. Kidney and central nervous system involvement typically do not occur. Drugs which have been associated with drug-induced lupus include antibiotics, certain thyroid medication, and drugs used to control blood pressure, seizures, and heart rhythm. Two of the most common offenders are hydralazine (Apresoline) and procainamide (Pronestyl). Because of these drug effects, it is important to know what medications a patient diagnosed as having lupus has been taking.Pregnancy is the occasion for special concern in the patient with systemic lupus erythematosus.
While pregnancy does not appear to affect the overall life expectancy of the lupus patient, lupus may flare during pregnancy and there is an increase in premature delivery and in stillbirth among lupus patients. Therefore, it is very important for the woman with lupus to seek medical help early in her pregnancy from an obstetrician who is experienced in dealing with high-risk pregnancies. (This topic is covered in Chapter 18.) Lupus may be difficult at times to diagnose and to distinguish from other connective tissues diseases and the symptoms may actually overlap with some of them. Rheumatoid arthritis involves a similar, though usually more severe, arthritis with morning stiffness.
Patients with rheumatoid arthritis may have a positive ANA in addition to a positive test for an antibody known as the rheumatoid factor. Scleroderma (systemic sclerosis) typically is associated with Raynaud's phenomenon, arthritis and a positive ANA but is characterized by the development of remarkably tight skin. Dermatomyositis involves a facial rash and may be associated with arthritis and lung disease, but profound muscle weakness also occurs. Overlapping symptoms of lupus, derrnatomyositis, and scleroderma is not uncommon. Some of these overlap cases (including those referred to as mixed connective tissue disease) respond well to treatment and do not develop significant kidney or neurologic disease.Some patients with lupus require very little treatment.
They may need only steroid skin creams or ointments and sunscreens. Other patients may require the use of simple pain medications and mild anti-inflammatory medicines such as aspirin and NSAIDS (nonsteroidal anti-inflammatory drugs) for muscle pain or arthritis. Drugs such as hydroxychloroquine (an anti-malarial medication) are used for patients with arthritis and skin disease. Adrenal corticosteroids (steroids) given by mouth are usually reserved for the more serious cases such as those with kidney or central nervous system disorders, certain blood cell problems, or serositis. To supplement steroids or to replace them when they don't work, immunosuppressive drugs, which were originally developed for use in cancer chemotherapy but have also been found to be helpful in treating lupus, are used. (These and other treatments are dealt with further in Chapter 3.)
In addition to medical treatment, there are other important considerations in the treatment of lupus. Clubs and support groups are available to offer education and psychological help. (See Chapter 19.) Physical and occupational therapy and other methods used by rehabilitation medicine can provide pain relief and can help to maintain functioning (discussed in Chapter 16). Psychotherapy and counselling can be an important addition to other forms of treatment and are available from different sources including psychiatrists, psychologists, and social workers. (These are mentioned in connection with depression in Chapter 10 and with sexual dysfunction in Chapter 17.)
Finally, one of the most helpful aspects of the treatment program is a good doctor-patient relationship. This will be the subject of an entire chapter later on (Chapter 20).Over the years, we have had more and more success in the treatment of lupus. Studies done at Johns Hopkins University in 1954 showed a survival rate less than 50% after four years. Several large studies done in the 1980's, however, revealed an approximate 87% five year survival and an approximate 78% ten year survival. A smaller study from Holland published in 1989 found an even better ten year survival of 87%! There is good reason to believe that this upward trend will continue in the future as we learn more about the disease and how to control it.
=======================================================
This information is for "informational purposes" and is not meant to be used for medical diagnosis. Always consult your physician on matters such as this.
Edited by: Mary E. Moore, Ph.D., M.D., Carolyn H. McGrory, MS, RN, Robert S. Rosenthal, M.D.
Systemic lupus erythematosus (SLE or lupus) is a disease which can affect many parts of the body, can be both acute and chronic, and can cause many different symptoms. The disease can range from very mild to very severe. Among adults, nine females will have lupus for every one male. Lupus most commonly affects people between the ages of twenty and forty and occurs, therefore, most frequently in women during their child-bearing years. Three times as many black as white women are affected. Children and teens can also get lupus.
During the first ten years of life girls will have lupus three to seven times more often than boys. (Childhood lupus is the subject of Chapter 15.)The cause of lupus is not known. The immune system, which is nature's way of protecting us from infection and cancer, works improperly in patients with lupus. Certain white blood cells, the T lymphocytes, aid in control of the immune system. In lupus, these cells fail to fulfill their regulatory function and the immune system becomes active when it is not supposed to. What starts this has not been discovered.
It appears that some foreign invader, some trigger or triggers from the outside must enter the body and set this process in motion. For years the search has gone on in vain to find a virus which could act as such a trigger. We do know that sunlight and some chemicals appear to trigger lupus in some patients, though not in all. We also know that some people have an inherited tendency to develop lupus. Something in their genetic makeup makes them especially sensitive to an environmental trigger. (Chapter 4 discusses recent advances in the genetics of lupus.)To aid in the diagnosis of lupus, the American College of Rheumatology has listed various criteria (guidelines based on symptoms and laboratory tests) to be used in identifying the disease (Table 1). The list contains the problems that are most typical of lupus as compared to other similar diseases.
It is not meant to include all, or even the most common problems a patient with lupus may experience. Persons with lupus most frequently suffer from fatigue, fevers, muscle and joint aching and stiffness, swollen glands, and generalized feelings of being unwell. None of these are listed as criteria for diagnosing SLE, however, because they occur in many other diseases as well. The first of the criteria involve the skin. The best known of the skin problems in lupus is the "butterfly" rash. This is a red rash over the cheeks and the bridge of the nose (the malar area). Several other skin-related problems which commonly occur are sun sensitivity, loss of hair, red plaques (slightly raised patches of skin that have a definite border) associated with scaling and plugging of the hair follicles ("discoid" rash), hives, dryness and ulceration of the mucous membrane (mouth, nose, and vagina), and dilated and broken blood vessels. (These are discussed in more detail in Chapter 5 and referred to in Chapters 12 and Chapter 14.) The circulation of blood to the skin and the underlying tissues is sometimes temporarily decreased in lupus, this is known as Raynaud's phenomenon.
The decrease is most commonly caused by cold temperatures but can also result from psychological stress. The tips of the fingers, the tips of the toes, and occasionally the nose and the ears are affected. These areas first turn white, then blue, and finally, as the circulation returns, red. If the circulation is reduced over a long period of time, the skin may break down and ulcerations (sores) appear.Almost all lupus patients have joint pains at some time during their illness, and many have joint inflammation (swelling, warmth, redness, pain) or arthritis. The type of arthritis associated with lupus is similar to that seen in rheumatoid arthritis but is usually not as severe and does not cause a wearing away of the bone with resulting deformity. It most commonly affects the small joints of the hands, the wrists, the knees, and the feet. Sometimes patients with lupus, especially those on corticosteroids, have involvement of the bone due to a loss of blood supply.
When this occurs in the bone near a joint, degenerative arthritis may result. (See Chapter 13.)Serositis is the inflammation of the delicate tissues which cover certain internal organs and line body compartments. It is quite common in lupus, most often occurring as inflammation of the covering of the lung and the lining of its compartment in the chest (pleuritis), and as inflammation of the covering of the heart (pericarditis). Movement of the involved tissues may cause chest pain, and the doctor can often hear a characteristic "rub" when listening over the inflamed areas with a stethoscope. Abdominal pain in lupus is sometimes caused by inflammation of the covering of the intestines and the lining of the abdominal cavity (peritonitis), and can imitate pancreatitis, diverticulitis, appendicitis, etc. Fluid sometimes accumulates as a result of serositis and may have to be withdrawn by a needle. In the case of pericarditis, an operation to make an opening in the covering of the heart (a "Pericardial window") may be necessary to relieve pressure on the heart from this fluid.One of the most feared complications of lupus is involvement of the kidney.
It is present to some degree in about 50% of cases. It can vary from very mild involvement which causes the patient no symptoms, to severe disease leading to kidney failure and the need for dialysis or kidney transplantation. Doctors detect early kidney problems by discovering protein or blood cells in the urine. They frequently ask the patient to collect 24 hour urine samples so as to be able to make better estimates of the degree to which the kidney is functioning. At times it is necessary to obtain a biopsy of the kidney to look for the presence of antibodies and kidney cell damage. (Lupus and the kidney is further discussed in Chapter 6.)The central nervous system (CNS) may become involved by systemic lupus erythematosus. Such involvement may cause a variety of disorders including seizures, psychosis, paralysis and personality disorders.
Since very similar problems might be the result of infection, hardening of the arteries, or mental illness, etc., and since there is no one test for central nervous system lupus, these disorders may be difficult for the physician to diagnose. (Neurologic problems seen in lupus are discussed in detail in Chapter 7, neuropsychological testing is discussed is Chapter 8 and depression in lupus is discussed in Chapter 10.)One of the frequent disorders in lupus is a decrease in the number of white blood cells, especially the lymphocytes. Since some of the drugs used to treat lupus can also cause a decrease in the white blood cell count, monitoring the safe use of these drugs can, at times, be difficult.
The platelet is another blood cell frequently decreased in lupus. Platelets play a key role in blood clotting and having too few platelets can lead to serious bleeding disorders. Anemia, a decrease in the number of red blood cells, is almost always present in cases of active lupus. It may result from bleeding, from a bone marrow which is depressed by illness and therefore does not produce enough red blood cells, or from the destruction of red blood cells by antibodies. (More about the anemia of lupus in Chapter 11.) When the number of certain T lymphocytes is decreased, the B lymphocytes of the lupus patient are stimulated to manufacture many different antibodies. Among these are antibodies called "autoantibodies" which target the lupus patient's own tissues. The body, in essence, attacks itself. In addition to the widely known antinuclear antibody (ANA), there are many other antibodies, the presence of which aid in the diagnosis of lupus. (See Chapter 2 for a discussion of ANA, anti-DNA, anti-Sm, anti-Ro, anticardiolipin, etc.)Certain drugs in common use may cause antinuclear antibodies to develop and, therefore, cause a mild form of lupus known as drug-induced lupus.
Drug-induced lupus is characterized by muscle pain and arthritis, fever, and pleuritis. Kidney and central nervous system involvement typically do not occur. Drugs which have been associated with drug-induced lupus include antibiotics, certain thyroid medication, and drugs used to control blood pressure, seizures, and heart rhythm. Two of the most common offenders are hydralazine (Apresoline) and procainamide (Pronestyl). Because of these drug effects, it is important to know what medications a patient diagnosed as having lupus has been taking.Pregnancy is the occasion for special concern in the patient with systemic lupus erythematosus.
While pregnancy does not appear to affect the overall life expectancy of the lupus patient, lupus may flare during pregnancy and there is an increase in premature delivery and in stillbirth among lupus patients. Therefore, it is very important for the woman with lupus to seek medical help early in her pregnancy from an obstetrician who is experienced in dealing with high-risk pregnancies. (This topic is covered in Chapter 18.) Lupus may be difficult at times to diagnose and to distinguish from other connective tissues diseases and the symptoms may actually overlap with some of them. Rheumatoid arthritis involves a similar, though usually more severe, arthritis with morning stiffness.
Patients with rheumatoid arthritis may have a positive ANA in addition to a positive test for an antibody known as the rheumatoid factor. Scleroderma (systemic sclerosis) typically is associated with Raynaud's phenomenon, arthritis and a positive ANA but is characterized by the development of remarkably tight skin. Dermatomyositis involves a facial rash and may be associated with arthritis and lung disease, but profound muscle weakness also occurs. Overlapping symptoms of lupus, derrnatomyositis, and scleroderma is not uncommon. Some of these overlap cases (including those referred to as mixed connective tissue disease) respond well to treatment and do not develop significant kidney or neurologic disease.Some patients with lupus require very little treatment.
They may need only steroid skin creams or ointments and sunscreens. Other patients may require the use of simple pain medications and mild anti-inflammatory medicines such as aspirin and NSAIDS (nonsteroidal anti-inflammatory drugs) for muscle pain or arthritis. Drugs such as hydroxychloroquine (an anti-malarial medication) are used for patients with arthritis and skin disease. Adrenal corticosteroids (steroids) given by mouth are usually reserved for the more serious cases such as those with kidney or central nervous system disorders, certain blood cell problems, or serositis. To supplement steroids or to replace them when they don't work, immunosuppressive drugs, which were originally developed for use in cancer chemotherapy but have also been found to be helpful in treating lupus, are used. (These and other treatments are dealt with further in Chapter 3.)
In addition to medical treatment, there are other important considerations in the treatment of lupus. Clubs and support groups are available to offer education and psychological help. (See Chapter 19.) Physical and occupational therapy and other methods used by rehabilitation medicine can provide pain relief and can help to maintain functioning (discussed in Chapter 16). Psychotherapy and counselling can be an important addition to other forms of treatment and are available from different sources including psychiatrists, psychologists, and social workers. (These are mentioned in connection with depression in Chapter 10 and with sexual dysfunction in Chapter 17.)
Finally, one of the most helpful aspects of the treatment program is a good doctor-patient relationship. This will be the subject of an entire chapter later on (Chapter 20).Over the years, we have had more and more success in the treatment of lupus. Studies done at Johns Hopkins University in 1954 showed a survival rate less than 50% after four years. Several large studies done in the 1980's, however, revealed an approximate 87% five year survival and an approximate 78% ten year survival. A smaller study from Holland published in 1989 found an even better ten year survival of 87%! There is good reason to believe that this upward trend will continue in the future as we learn more about the disease and how to control it.
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This information is for "informational purposes" and is not meant to be used for medical diagnosis. Always consult your physician on matters such as this.
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