Diagnosis Can Be Difficult

A selection from the Lupus Foundation of America Newsletter Article Library
ApprovedLFA Patient Education Committee
92-035

Research focusing on the nature of lupus (SLE) has accelerated sharply over the past twenty years. It is believed that the symptoms of lupus are the result of an abnormally functioning immune system. What causes the malfunction is not yet known. The normal immune system functions to protect the body against damage by viruses, bacteria and other foreign substances.

In lupus, this same immune system appears to react against the body's own healthy cells forming antibodies against them. This causes inflammation and the subsequent symptoms of the disease. Since the immune system functions throughout the body, the symptoms of lupus can vary widely in type and intensity, depending on the parts of the body being affected.

Why is it difficult?
Most people involved in lupus research and treatment would probably agree that lupus still remains a difficult disease to diagnose. Two reasons account for this difficulty:
There is no single set of symptoms that are uniformly specific to lupus.
There are no laboratory tests yet available that can prove conclusively that a person has or does not have lupus. Almost every symptom of lupus can also be easily attributed to other illnesses or disorders. In addition, the symptoms are sometimes vague or they may come and go spontaneously.

For instance, fever, weight loss, marked fatigue and weakness which are often experienced by someone with lupus, may also be symptoms of many others disorders, some more threatening, some less so.

Likewise if transient (temporary) joint or muscle pain is the initial problem, here again there are so many causes of such symptoms that it may be very difficult to link these to lupus. If pleurisy is a symptom and it spontaneously clears up rather quickly, the physician may assume that a virus was the cause and not necessarily lupus.

Approach to diagnosis
The diagnosis of lupus is usually made after a careful review of the patient's medical history, coupled with analysis of blood study results from both routine laboratory testing and some specialized tests related to immune system status. Since symptoms may present themselves slowly and may evolve over months or years, it is important that a physician follow the patient to see what happens.

Often it can take years for the diagnosis to be made. This can be a very difficult time for the person seeking relief from numerous symptoms. Only by a comprehensive examination can the probability of lupus be assessed and even then it is sometimes very difficult to be sure.

Evaluation of symptoms
The first principle in making a diagnosis of SLE is that the individual has clinical evidence of a multi-system disease (i.e. has shown abnormalities in several different organ systems). The following are typical manifestations (symptoms) which might lead to suspicion of SLE.

• Skin: butterfly rash; ulcers in the roof of the mouth; hair loss.
  
• Joints: pain; redness and swelling.
• Kidney: abnormal urinalysis suggesting kidney disease.
• Lining membranes: pleurisy; pericarditis and/or peritonitis (taken together this type of inflammation is known as polyserositis).
• Blood: hemolytic anemia (the red cells are destroyed by autoantibodies); leukopenia (low white blood cell count);thrombocytopenia (low platelets).
• Lungs: infiltrates that may be transient.
• Nervous system: convulsions (seizures); psychosis; nerve abnormalities that cause strange sensations or alter muscular ability.

Evaluation of immune status
The second diagnostic principle is to examine the status of the immune system in individuals having a suspicious clinical history. In general, physicians now look for evidence of autoantibodies.

At this time some commonly used tests of immune status in the diagnosis of SLE are:
The anti-nuclear antibody test (ANA): a test to determine if autoantibodies to cell nuclei are present in the blood.

The anti-DNA antibody test: to determine if the patient has antibodies to the genetic material in the cell. The anti-Sm antibody test: to determine if there are antibodies to this substance, a nuclear protein. A variety of tests for the presence of immune complexes in the blood.
Tests to examine the total level of serum complement - a group of proteins involved in the inflammation which can occur in immune reactions - and tests to assess the specific level of C3 and C4, two proteins of this group.

LE cell prep: An examination of the blood looking for a certain kind of cell which has ingested the swollen antibody-coated nucleus of another cell. A positive ANA may occur sometime during the course of the illness in about 90 percent of patients with SLE, but it also occurs in a variety of other illnesses and in as much as 5 percent of the normal population. It is a very sensitive test and is now more frequently performed than the LE prep.

Tissue biopsy
Sometimes examination of a tissue sample can be helpful in making the diagnosis. A kidney biopsy, for example, can show certain changes characteristic of SLE if the kidney disease is severe. Even in early kidney involvement, examination of biopsy tissue can show deposits of antibodies and immune complexes in the kidney's filtration unit.

A skin biopsy can be helpful in identifying deposits of antibodies and complement proteins found at the junction of the outer skin layer, called the epidermis, and the underlying part of the skin, the dermis. A "positive band test" is significant only when the tissue sample is taken from an area which is not involved by the rash. The results, like those of a kidney biopsy, should be interpreted in combination with the clinical history, as well as all the other tests performed.

Criteria
In 1982, the American Rheumatism Association published a revised set of criteria to aid physicians in making the diagnosis of Lupus. The criteria (see note) are:

• Malar Rash
• Discoid Rash
• Photosensitivity
• Oral Ulcers
• Arthritis
• Serositis
• Renal disorder
• Neurologic disorder
• Hematological disorder
• Immunologic disorder

Positive fluorescent antinuclear antibody (FANA) or ANA test result A physician observing a person to have at least 4 out of the 11 criteria, either serially or collectively, should be suspicious to the possibility of lupus being the underlying disorder. However, physicians must also be careful in utilizing criteria for an individual case, as other diseases could also conform to the criteria.

Presently, the diagnosis of lupus is usually based on these findings:
evidence of a multi-system disease (more than one organ involved):
the presence of autoantibodies; the exclusion of other diseases and disorders which can mimic the features of lupus. Despite advances in medical education and technology it is still not uncommon for lupus to be incorrectly diagnosed or require a lengthy period of time to be diagnosed mainly because the symptoms vary so widely, come and go frequently, and because the disease mimics so many other disorders.

An important fact to remember concerning the treatment for lupus is that the diagnosis does not indicate the particular therapy to be used. In the absence of a cure, present-day treatment of lupus is still primarily tailored to symptomatic relief and not to the diagnosis.

=========================================================== This information is for"informational purposes" and is not meant to be used for medical diagnosis. Alwaysconsult your physician on matters such as this.

Late Onset Lupus Fact Sheet

• Lupus can occur at any age, in either sex, in any race.
  
• 15% of people with Systemic Lupus Erythematosus (SLE) develop it later in life after age 55.
  
• Late onset lupus affects women 8 times more often than men. Compared with younger SLE patients, late onset lupus affects a higher percentage of men.
  
• Late onset lupus is found primarily in Caucasians, but occurs in all races.
  
• Symptoms in most cases are relatively mild and commonly include: arthritis, pleurisy (chest pain with deep breathing), pericarditis (inflammation of the sac around the heart), muscle aches, dry eyes and dry mouth (Overlap syndrome).
  
• Uncommon symptoms include: fever, swollen lymph glands, seizure, psychoses, and Raynaud's Phenomenon (fingers turn blue or white in the cold).
  
• Because symptoms of lupus in older people mimic other diseases, eg., rheumatoid arthritis, Sjogren's syndrome, polymyalgia rheumatica, distinguishing among them is difficult and may result in a delayed or missed diagnosis.
  
• Severe kidney involvement is less common in late onset lupus.
  
• The average age of onset is 59 years; average age at diagnosis is 62 years.
  
• As a rule, older people with lupus do better and their lupus can be managed with conservative therapy. When corticosteroids are required, symptoms are controlled with lower doses (i.e., less than 25 mg/day for one month).
  
• Drug-induced lupus occurs more often in older people because they are more likely to have conditions (high blood pressure, heart disease) that require treatment that may cause the symptoms of lupus. Symptoms generally fade when the medication is discontinued.
  

Is Your Child at Risk?Atherosclerotic Heart Disease in Young People with Lupus

Is Your Child at Risk?Atherosclerotic Heart Disease in Young People with Lupus
Susan Manzi, M.D., M.P.H., Janice M. Sabatine, Ph.D., and Laura E. Schanberg, M.D.
From Lupus News Fall 2002, Vol. 22, N. 3

It has been well established that accelerated atherosclerosis, or hardening of the arteries, occurs in adults with lupus, along with its eventual clinical outcomes-myocardial infarction (heart attack) and stroke.

However, atherosclerotic heart disease is emerging as one of the most serious concerns in children and adolescents with lupus. Young, premenopausal women with lupus are up to 50 times more likely to have a heart attack than women of the same age who do not have lupus (1). Our research and that of other investigators indicate that these clinical outcomes are generally related to accelerated atherosclerosis (2,3).

Evidence of heart problems in children with lupusChildren with lupus are known to experience myocardial infarction and stroke. Although the frequency of atherosclerosis in these young patients is not fully known, emerging evidence suggests the incidence may be similar to that in premenopausal women with lupus.

It is not unreasonable to suspect atherosclerotic development at young ages, as evidence of fatty streak formation-an early step in plaque formation-has been noted in healthy children as young as two to three years of age.

Atherosclerosis-promoting patterns of cholesterol, triglycerides, and other lipoproteins in children and adolescents with lupus have been documented (4). Coronary blood flow abnormalities were found in 16 percent of children and adolescents with lupus who had no cardiac symptoms. This suggests that there may be a significant percentage of young lupus patients with undiagnosed heart disease.

In addition, evidence of carotid atherosclerotic plaque and abnormal coronary blood flow have been detected two to five years after the onset of lupus, indicating that atherosclerosis may develop very early in the course of disease (5,6).

What causes heart disease in lupus?We do not completely understand the underlying biological cause for accelerated atherosclerosis in young patients with lupus, probably because we do not yet completely understand the underlying biological cause of lupus. One thing we do know is that two key factors are the disease of lupus, itself, and its treatment.

It was initially thought that the increased risk of heart disease in patients with lupus might be due to traditional risk factors, such as:
hypertension
diabetes
obesity
elevated cholesterol levels.

But recent evidence indicates that the presence of lupus itself, or the treatment for lupus, contribute more than those risk factors alone (2,7). The task now is to identify the biological processes occurring in lupus or resulting from its treatment that promote atherosclerosis.
It was once believed that excess cholesterol built up as plaque inside the blood vessels and obstructed blood flow. Investigators now know that fewer than 20 percent of heart attacks are due to restricted blood flow in progressively narrowed coronary vessels. More commonly, heart attacks occur when an atherosclerotic plaque ruptures and a blood clot forms around the plaque (8).

There are several potential parallels between lupus and the formation and rupture of atherosclerotic plaques (9).

A. For instance, damage to the lining of the blood vessels provokes an inflammatory response, which leads to deposits of immune cells containing fat droplets into the arterial wall. This in turn leads to plaque formation.

B. Inflammation is also responsible for thinning the fibrous cap that covers a plaque, making it more vulnerable to rupture. The inflammatory substances known as cytokines that drive the formation of atherosclerotic plaques and increase the vulnerability of the plaques to rupture are the same substances that play a major role in the inflammatory processes seen in lupus. This may explain why cardiovascular disease is accelerated in lupus.

C. Another factor related to lupus as a prime suspect as sources of damage to the lining of the arterial wall is high levels of circulating immune complexes. Although immune responses are important in the body's normal response to damage and microbial infections, in systemic lupus-and perhaps in atherosclerosis in general-these normally protective immune responses become dysregulated, leading to a high degree of inflammation and tissue damage.

D. A wide variety of autoantibodies may also hold responsibility. Elevated levels of antiphospholipid antibodies, which are often found in people with lupus, have traditionally been linked to an increased risk of blood clotting and may increase the risk of clot formation at the plaque site. More recent evidence suggests that these antibodies may also facilitate the uptake of oxidized low density lipoprotein, the "bad cholesterol," into inflammatory cells in the vessel wall. This is a key step in the formation of atherosclerotic plaque (10).

E. The amino acid homocysteine is another agent that is often elevated in lupus patients and is a likely source of arterial injury. Elevated levels of homocysteine have been linked to thrombosis in lupus patients (11) and to coronary heart disease and stroke in non-lupus patients. The reasons for elevated homocysteine in lupus are not entirely known, but may be related to kidney disease, diet, or treatment.

Is there a link between corticosteroids and atherosclerosis?With the advent of glucocorticoids (prednisone) in the 1950s, there has been a significant improvement in the lifespan of young people with lupus. Yet there is concern that these agents may actually contribute to the development of atherosclerosis, either directly by promoting plaque formation or indirectly by intensifying risk factors such as:

weight gain
hypertension
elevated serum glucose and lipid levels.

In contrast, some evidence indicates that the anti-inflammatory effects of glucocorticoids may actually provide protection against atherosclerosis, suggesting that poorly controlled lupus activity may contribute to cardiac disease, with corticosteroid treatment providing a degree of protection.

Perhaps with the use of newer biologic therapies with similar anti-inflammatory and immunomodulatory effects as corticosteroids, but fewer adverse side effects, we will begin to see a reduction in heart disease.

How can atherosclerosis be managed in young people? It is of critical importance that physicians and patients be aware of the increased risk of cardiovascular complications in lupus. Young people generally view their risk of heart disease as negligible, yet cardiovascular disease intervention and prevention has the potential to significantly lengthen and improve the quality of their lives over many years.

Chest pain. Any physician treating a young person with lupus, regardless of the patient's age or sex, should be suspicious of chest pain. Because the patients are young and because chest pain in lupus may be attributable to other causes, physicians may overlook conditions, such as angina (chest pain due to myocardial ischemia). Yet often there are no warning signs for an impending heart attack. For these reasons, a major focus on management strategies should rest on preventing the development of atherosclerosis.

Diet. There have been reports on the potential benefits of diet modification in controlling abnormal lipid levels in children with lupus, but diet alone is not always sufficient, and pharmacologic therapy may be necessary. However, the type, timing, and dosage of such therapy in children have not been well established by large studies.

Blood clots. Measures to reduce potential blood clots, such as anticoagulation or antiplatelet therapy, should be considered in patients at increased risk, such as those with kidney disease, antiphospholipid antibodies, and other coronary disorders.

There are few clinical data on the effects of non-steroidal anti-inflammatory drugs (NSAIDs) on atherosclerosis. However, some evidence suggests that the selective inhibitors of the prostaglandin-producing enzyme COX-2 might actually enhance blood clot formation in some non-lupus populations. Further investigation in this area is currently underway.

Aspirin. There is strong evidence from clinical trials to support the use of low-dose aspirin therapy in preventing heart attacks in the general population. At low doses, aspirin is probably
reducing the clotting risk but not reducing inflammation.

Dietary supplements. There is some evidence that the antioxidant vitamins E and C may improve arterial dilatation in children with familial hypercholesterolemia or combined hyperlipoproteinemia. However, the long-term benefit of antioxidant therapies in reducing cardiovascular risks in lupus is unknown. Measures to reduce homocysteine levels with folate supplementation may be beneficial; again, however, the effects on prevention of coronary events are unproven.

Steroids. Based on the possible opposing effects of corticosteroids-increasing traditional risk factors and controlling inflammation-there are no established recommendations about the use of corticosteroids concerning cardiovascular risk in lupus. In general, judicious use of these agents to control the underlying disease and to minimize the proven long-term side effects is recommended.

Table 1 (below) illustrates suggested strategies to manage and/or prevent atherosclerosis in young patients with lupus. These strategies are targeted at both traditional cardiovascular risk factors and at potential lupus-specific factors. Physicians should communicate these potential risks to patients and their parents, and provide relevant information and resources for patient education.

Table 1. Strategies For Managing Cardiovascular Disease Risk In Children With Lupus

Step 1: Physician awareness
Recognize increased risk in young population.
Conduct a thorough cardiac evaluation if there is any suspicion of heart disease.

Step 2: Patient education
Make patients and parents aware of increased risk.

Step 3: Minimize traditional cardiovascular risk factors
Encourage a regular aerobic exercise program.
Establish guidelines for a heart-healthy diet.
Assist with weight loss program, if necessary.
Start a smoking cessation program.
Control hypertension and diabetes, if present.
Treat hyperlipidemia.

Step 4: Address potential lupus-specific risk factors
Use corticosteroids judiciously.
Reduce homocysteine levels (folate supplementation).
Consider aspirin or anticoagulant therapy for patients at high risk for blood clotting.

Physicians also should work together with the patients and parents to encourage a heart-healthy diet, a regular exercise program that involves aerobic activity, and weight loss, if necessary. Patients should be advised not to start smoking and to quit if they have already started. Hypertension and diabetes should be managed aggressively.

Bottom line
It is clear that premature atherosclerosis in children, adolescents, and young, premenopausal women with lupus is a substantial medical concern. The reasons that atherosclerosis is accelerated in lupus patients likely involve the inflammatory and immune-mediated mechanisms shared by these two disease processes.

Until new biologic therapies are available that can halt the immune dysregulation and resulting inflammation and vascular damage in lupus, we must promote aggressive approaches to reducing traditional cardiovascular risk factors.

Noninvasive methods for specifically identifying vulnerable plaques might also pinpoint those lupus patients at greatest risk for heart attack and those most likely to benefit from intervention. Investigations into the pathways that lead to premature heart disease in lupus may provide an ideal model for examining the role of inflammation in all populations with cardiovascular disease.

About the AuthorsSusan Manzi, MD, M.P.H., is an Associate Professor of Medicine and Epidemiology at the University of Pittsburgh School of Medicine in Pennsylvania.
Janice M. Sabatine, Ph.D. is a medical writer and editor.
Laura E. Schanberg, MD is an Assistant Professor of Pediatric Rheumatology at Duke University Medical Center in Durham, NC.

References
1. Manzi S, Meilahn EN, Rairie JE, Conte CG, Medsger TA, Jr., Jansen-McWilliams L et al. Age-specific incidence rates of myocardial infarction and angina in women with systemic lupus erythematosus: comparison with the Framingham Study. Am J Epidemiol 1997;145:408-415.2. Manzi S, Selzer F, Sutton-Tyrrell K, Fitzgerald SG, Rairie JE, Tracy RP et al. Prevalence and risk factors of carotid plaque in women with systemic lupus erythematosus. Arthritis Rheum 1999;42:51-60.3. Selzer F, Sutton-Tyrrell K, Fitzgerald S, Tracy R, Kuller L, Manzi S. Vascular stiffness in women with systemic lupus erythematosus. Hypertension 2001;37:1075-1082.4. Ilowite NT. Premature atherosclerosis in systemic lupus erythematosus. J Rheumatol 2000;27 Suppl 58:15-19.5. Gazarian M, Feldman BM, Benson LN, Gilday DL, Laxer RM, Silverman ED. Assessment of myocardial perfusion and function in childhood systemic lupus erythematosus. J Pediatr 1998;132:109-116.6. Falaschi F, Ravelli A, Martignoni A, Migliavacca D, Sartori M, Pistorio A et al. Nephrotic-range proteinuria, the major risk factor for early atherosclerosis in juvenile-onset systemic lupus erythematosus. Arthritis Rheum 2000;43:1405-1409.7. Esdaile JM, Abrahamowicz M, Grodzicky T, Li Y, Panaritis C, du BR et al. Traditional Framingham risk factors fail to fully account for accelerated atherosclerosis in systemic lupus erythematosus. Arthritis Rheum 2001;44:2331-2337.8. Libby P. What have we learned about the biology of atherosclerosis? The role of inflammation. Am J Cardiol 2001;88:3J-6J.9. Manzi S. Systemic lupus erythematosus: a model for atherogenesis? Rheumatology (Oxford) 2000;39:353-359.10. Vaarala O. Autoantibodies to modified LDLs and other phospholipid-protein complexes as markers of cardiovascular diseases. J Intern Med 2000;247:381-384.11. Petri M, Roubenoff R, Dallal GE, Nadeau MR, Selhub J, Rosenberg IH. Plasma homocysteine as a risk factor for atherothrombotic events in systemic lupus erythematosus. Lancet 1996;348:1120-1124.

July 30, 2003

=========================================================== 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 and Pregnancy

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.

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.

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.

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.

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.

1. Bleeding gums: Gingiva which bleeds on eating or brushing teeth, or bleeds without any obvious cause.
2. Swollen gums: Enlarged gingiva which is not bound tightly around each tooth.
3. 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.
4. 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.

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.

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.

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.

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.