Eating More Fish – The Answer To Lupus?
11th March 2003
Press Release from the University of Ulster , Ireland
New research from the University of Ulster today offered hope to millions of lupus sufferers worldwide.
Dr Emeir Duffy, from the School of Biomedical Sciences, and Dr. Gary Meenagh, from Musgrave Park Hospital , have discovered new evidence to suggest that fish oil can greatly reduce the symptoms of the disease.
Systemic Lupus Erythematosus (SLE) or Lupus is a disorder of the Immune System, where the body harms its own healthy cells and tissues. The body tissues become damaged causing painful or swollen joints, unexplained fever, skin rashes, kidney problems, complications to the cardiovascular system and extreme fatigue.
There are approximately 500 diagnosed cases of SLE in Northern Ireland and it is most common in women of child-bearing age.
At present there is no cure but a key to managing lupus is to understand the disease and its impact. Steroids are the main drug used in the treatment of lupus and they should be administered for the shortest period possible to reduce side-effects.
But recently researchers have been looking specifically at its management through diet. Fish oils contain long-chained polyunsaturated fatty acids which are essential for normal growth and development but also have anti-inflammatory and anti-autoimmune properties.
Dr Duffy said: “We have been investigating how fish oil can improve the quality of life for lupus sufferers.
“In lupus, the body's immune system does not work as it should. Antibodies, which help fight viruses, bacteria and other foreign substances, are not produced effectively. The immune system actually produces antibodies against the body's own healthy cells and tissues. These auto-antibodies contribute to inflammation and other symptoms of the disease.
“Participants in the study who were taking fish oil supplements, three times per day for twenty-four weeks, saw a reduction in disease activity, an improvement in quality of life and reported an overall feeling of improved health by the end of the study compared to those taking a placebo supplement. Participants taking the fish oil also showed a reduction in fatigue severity, the most debilitating symptom for lupus sufferers.
“From our study and from other work, there is evidence that increasing dietary intake of the polyunsaturated fats found in fatty fish can have beneficial effects for lupus sufferers. Good examples of fatty fish include mackerel, lake trout, herring, sardines, tuna and salmon”.
=========================================================== 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.
Wednesday, June 06, 2007
New Research Shows Certain Vegetables May Delay the Onset and Reduce the Severity of Lupus
New Research Shows Certain Vegetables May Delay the Onset and Reduce the Severity of Lupus:
Scientists find that a compound abundant in broccoli, cauliflower and cabbage may help fight disease that affects 1.5 million Americans
A team of scientists from the North Shore-Long Island Jewish (LIJ) Research Institute have discovered that a compound found in abundance in the cruciferous family of vegetables delays the onset of systemic lupus erythematosus (SLE) in mice and reduces the severity of the disease once it has developed. This could be good news for individuals at risk for lupus, especially women, as well as those already affected by it.
Published today in the Journal of Nutrition, the study led by Karen Auborn, PhD, evaluated the effect of a supplement of indole-3-carbinol (I3C) - the compound found in broccoli, cauliflower, cabbage and similar vegetables - on the outcome of SLE in mice that are bred to develop the disease genetically. The results showed that mice with lupus lived significantly longer when fed the supplemented diet than did diseased mice fed the normal diet. Some even lived the normal lifespan.
Whether the mice were started on the I3C diet before or after the onset of the disease, the result was the same: there were fewer kidney problems (kidney disease is one of main complications of SLE) and they lived much longer than the control group.
"Mice are not people, of course, but the implication is that a diet rich in cruciferous vegetables could do much to ameliorate the disease," said Dr. Auborn. The findings support the view that I3C may benefit people at risk for SLE as well as those in the early stages of the disease. SLE is often treated with immunosuppressive drugs, which can have serious toxic side effects. By reducing the severity of the disease, I3C may allow a decrease in the dose of immunosuppressive drugs required, thereby reducing toxicity. It may even help prevent the recurrence of the disease.
A person could get the human equivalent dose of I3C tested in the study from about a third to a half of a head of cabbage. So getting the proper amount of I3C from food is realistic, although I3C also is available on the market as a dietary supplement.
According to the Lupus Foundation of America, approximately 1.5 million Americans suffer from some form of lupus, of which SLE is the most common. While it is unknown why women are nine times more likely to develop the disease than men, it is suspected that estrogen plays a role. Research has shown that women with SLE have abnormal estrogen metabolism. In many cancer prevention studies, I3C has been shown to exhibit antiestrogenic activity in the body. The North Shore-LIJ investigators had theorized that because I3C is an antiestrogen, it may prevent, delay, or even represent an adjunct treatment for lupus. They were right, at least for mice that genetically develop lupus.
The North Shore-LIJ Research Institute is planning a human study of the effects of I3C on SLE. Nicholas Chiorazzi, MD, a world-renowned rheumatologist and member of the study team, is working closely with Richard A. Furie, MD, chief of rheumatology at North Shore University Hospital in Manhasset, to make this a reality.
"It will be essential to determine if I3C can have similar effects in patients with SLE. Such studies are more difficult in humans because individual patients with lupus differ in their genetic backgrounds and also because the effects of lupus vary greatly from person to person, at least as far as the organs targeted by the problem," said Dr. Chiorazzi, who is also director and CEO of the North Shore-LIJ Research Institute.
This research was supported by grants from the Ryan Caulfield Foundation, the Willa and Robert Bernhard Fund, and the National Institutes of Health.
About the North Shore-Long Island Jewish Research Institute The North Shore-LIJ Research Institute is among the top seven percent of institutions nationally that receive funding from the National Institutes of Health. Building on its strengths in immunology and inflammation, oncology and cell biology, human genetics, and neurodegenerative and psychiatric disorders, its goal is to understand the biological processes that underlie various diseases and translate this knowledge into new tools for diagnosis and treatment.
===========================================================
This information is for "informational purposes" and is not meant to be used for medical diagnosis. Always consult your physician on matters such as this.
Scientists find that a compound abundant in broccoli, cauliflower and cabbage may help fight disease that affects 1.5 million Americans
A team of scientists from the North Shore-Long Island Jewish (LIJ) Research Institute have discovered that a compound found in abundance in the cruciferous family of vegetables delays the onset of systemic lupus erythematosus (SLE) in mice and reduces the severity of the disease once it has developed. This could be good news for individuals at risk for lupus, especially women, as well as those already affected by it.
Published today in the Journal of Nutrition, the study led by Karen Auborn, PhD, evaluated the effect of a supplement of indole-3-carbinol (I3C) - the compound found in broccoli, cauliflower, cabbage and similar vegetables - on the outcome of SLE in mice that are bred to develop the disease genetically. The results showed that mice with lupus lived significantly longer when fed the supplemented diet than did diseased mice fed the normal diet. Some even lived the normal lifespan.
Whether the mice were started on the I3C diet before or after the onset of the disease, the result was the same: there were fewer kidney problems (kidney disease is one of main complications of SLE) and they lived much longer than the control group.
"Mice are not people, of course, but the implication is that a diet rich in cruciferous vegetables could do much to ameliorate the disease," said Dr. Auborn. The findings support the view that I3C may benefit people at risk for SLE as well as those in the early stages of the disease. SLE is often treated with immunosuppressive drugs, which can have serious toxic side effects. By reducing the severity of the disease, I3C may allow a decrease in the dose of immunosuppressive drugs required, thereby reducing toxicity. It may even help prevent the recurrence of the disease.
A person could get the human equivalent dose of I3C tested in the study from about a third to a half of a head of cabbage. So getting the proper amount of I3C from food is realistic, although I3C also is available on the market as a dietary supplement.
According to the Lupus Foundation of America, approximately 1.5 million Americans suffer from some form of lupus, of which SLE is the most common. While it is unknown why women are nine times more likely to develop the disease than men, it is suspected that estrogen plays a role. Research has shown that women with SLE have abnormal estrogen metabolism. In many cancer prevention studies, I3C has been shown to exhibit antiestrogenic activity in the body. The North Shore-LIJ investigators had theorized that because I3C is an antiestrogen, it may prevent, delay, or even represent an adjunct treatment for lupus. They were right, at least for mice that genetically develop lupus.
The North Shore-LIJ Research Institute is planning a human study of the effects of I3C on SLE. Nicholas Chiorazzi, MD, a world-renowned rheumatologist and member of the study team, is working closely with Richard A. Furie, MD, chief of rheumatology at North Shore University Hospital in Manhasset, to make this a reality.
"It will be essential to determine if I3C can have similar effects in patients with SLE. Such studies are more difficult in humans because individual patients with lupus differ in their genetic backgrounds and also because the effects of lupus vary greatly from person to person, at least as far as the organs targeted by the problem," said Dr. Chiorazzi, who is also director and CEO of the North Shore-LIJ Research Institute.
This research was supported by grants from the Ryan Caulfield Foundation, the Willa and Robert Bernhard Fund, and the National Institutes of Health.
About the North Shore-Long Island Jewish Research Institute The North Shore-LIJ Research Institute is among the top seven percent of institutions nationally that receive funding from the National Institutes of Health. Building on its strengths in immunology and inflammation, oncology and cell biology, human genetics, and neurodegenerative and psychiatric disorders, its goal is to understand the biological processes that underlie various diseases and translate this knowledge into new tools for diagnosis and treatment.
===========================================================
This information is for "informational purposes" and is not meant to be used for medical diagnosis. Always consult your physician on matters such as this.
Monday, April 30, 2007
Lupus: Its Impact on Young People
By Robert H. Phillips, Ph.D.
Introduction
Most of the books and articles written about lupus are targeted for adults with lupus. There is a lot of information about the disease, such as its symptoms, treatment and lifestyle changes necessitated, all of which is very valuable. But there is a unique population that is not addressed often enough in this written material: the young person with lupus.
Lupus is most commonly diagnosed in women of childbearing age, and men are diagnosed as well, although in smaller numbers. But it is important to remember that boys and girls before childbearing years also can be diagnosed with lupus. And although they may experience many of the same problems that adults do (such as pain, other physical symptoms and medication side effects), certain problems are more “exclusively theirs.”
This article will address a sampling of the problems that may affect young people with lupus. (It is not the scope of this article to discuss medical facts. Rather, it focuses on the psychosocial issues that may appear). Rarely will a young person with lupus experience every psychological problem due to lupus. However, it's important for everyone involved – the young person, other family members, friends, educators and healthcare professionals – to be aware of, and sensitive to, these difficulties.
Denial
It is interesting that adolescents, more than virtually every other age group, have a common, non-verbalized (and sometimes loudly verbalized) belief about their medical status: “I'm fine!” They don't want to feel sick; they don't want to be sick; and they don't want to be different. But lupus may throw a monkey wrench into that.
What makes it especially difficult, though, is that unless lupus is affecting the young person so aggressively that they are virtually unable to move, in many cases the young person's attitude will continue to be “I'm fine.” This can be frustrating for other family members, who are trying to be protective and helpful, and for healthcare professionals, who have more difficulty treating a young person if the answers to questions about symptoms are evasive or denying.
School
Adults who work generally have a clear sense of their responsibilities and obligations at their jobs; they know that if they don't work, changes will result – many of which can be difficult and unpleasant. Young people, whose primary job is to go to school, may go because they want to and because they know they are expected to. Yet they may not be aware of the far-reaching implications of inconsistent school attendance.
For young people with lupus, the attitude about school varies. Some are upset and frustrated if lupus interferes with consistent school attendance and schoolwork performance. Others see school as less important and may have few or no qualms about missing excessive time in school.
Young people with lupus may have a more difficult time in school if they have to deal with the cruelty of other children (“What is that ugly rash on your face?”) or the ignorance of teachers (“You've missed too much school work; either get with the program or you're going to fail.”) In addition, because of lupus, young people may find themselves ostracized and even excluded from activities that were once within their physical capabilities.
Peer pressure
Peer pressure may also affect young people with lupus. The need to “fit in” – at its strongest during childhood and adolescence – can be devastating to someone who has a chronic illness with noticeable physical effects (e.g., rashes, bloating, etc.) and behavioral effects (slower, more painful movements, etc.).
It is heartwarming to hear stories of young people with lupus whose friendships continue despite their illness. Yet it is sad to hear of other stories in which the person with lupus is ridiculed and even abandoned by former friends.
Parents
Young people with lupus may have difficulties with parents being overprotective (“Stay inside, the sun is out”), not protective enough (“You want to go to the beach? Do whatever you want”), or insensitive to their needs (“Stop complaining about your pain, already. Get up and finish your school work”).
Parents may be concerned about the effects their child's lupus will have on the family, such as financial issues, problems with or neglect of other children, or even feeling like their independence is being inhibited. Any of these concerns can likewise affect the young person with lupus. Already unhappy because of having lupus, but feeling responsible for problems within the family or with the parents, a young person may feel guilty to the extent that it interferes with their physical and emotional health.
For example, the young person may not tell parents about a serious lupus symptom, knowing it might mean another trip to the doctor or even the possibility of hospitalization.
Siblings
Brothers and sisters of a young person with lupus may be very resentful. Being less able to understand the physical impact of the disease, they may dislike the added “attention” being directed at their sick sibling. They also may not like getting less attention, and may act out in an attempt to regain their “share” of parental interactions. Their resentment toward their sick sibling may be manifested in many hurtful ways, such as anger, ignoring instructions, spiteful behavior, concealing important information from their parents, etc.
A final note
It is difficult enough for anyone to live with lupus, but the young person with lupus has added, age-related problems. Being aware of the potential impact of lupus in young people does not eliminate these problems. But increased awareness can pave the way to a better understanding of the unique needs of young people, and can lead to methods for better alleviating the problems that may occur.
Some Helpful Suggestions for Parents and Other Adults Dealing with Young People
Be Sensitive to the young Person's unique needs. Lupus can be a difficult disease to live with, especially for a child who has fewer “coping strategies” in place. Don't assume that the young person has the emotional strength or the social support network to handle lupus-related problems successfully.
Communicate appropriately. Try to look at any lupus-related issues through the eyes of the young person. See what they see. Feel what they feel. Using anger and aggressiveness in “forcing issues” is rarely productive. Calm, constructive discussion is a much more positive way to address lupus-related issues.
As much as possible, treat the young person like an adult. Plan together the appropriate ways to treat, and live with, lupus. Demonstrating adult-like behavior in interactions with young people is more likely to generate adult-like behavior in return.
Educate significant others. Any individuals who are not familiar with lupus, including family members, friends and teachers, can be obstacles to successful living with lupus. This is especially important in school, since the young person is going to spend a good number of hours there each day. Provide pamphlets and other information to teachers, guidance counselors and even classmates, so that school can truly be a “home away from home.”
Reprinted with permission of the Lupus Foundation of America, ©2001. Dr. Robert H. Phillips is founder and director of the Center for Coping in Long Island, NY ( www.coping.com) . He has been in private practice as a licensed psychologist since 1975 and has published and spoken widely on coping with physical ailments and other psychological topics. He has appeared on dozens of television and radio programs and currently is the host of “Coping Conversations, “ a weekly radio talk show on WKJY-FM (98.3) on Long Island .
Dr. Phillips is the published author of more than 20 books, including the highly popular Coping With Lupus (now completely revised and updated for its third edition); Lupus: Everything You Need to Know (with co-author Dr. Robert Lahita); and his new Successful Living With Lupus: An Action Workbook, published last year. These books, as well as several others, are available for sale from the Lupus Foundation of Minnesota (952-746-5151).
Dr. Phillips has served on the National Board of Directors of the Lupus Foundation of America and currently is a member of the Lupus News Advisory Board.
=========================================================== 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.
By Robert H. Phillips, Ph.D.
Introduction
Most of the books and articles written about lupus are targeted for adults with lupus. There is a lot of information about the disease, such as its symptoms, treatment and lifestyle changes necessitated, all of which is very valuable. But there is a unique population that is not addressed often enough in this written material: the young person with lupus.
Lupus is most commonly diagnosed in women of childbearing age, and men are diagnosed as well, although in smaller numbers. But it is important to remember that boys and girls before childbearing years also can be diagnosed with lupus. And although they may experience many of the same problems that adults do (such as pain, other physical symptoms and medication side effects), certain problems are more “exclusively theirs.”
This article will address a sampling of the problems that may affect young people with lupus. (It is not the scope of this article to discuss medical facts. Rather, it focuses on the psychosocial issues that may appear). Rarely will a young person with lupus experience every psychological problem due to lupus. However, it's important for everyone involved – the young person, other family members, friends, educators and healthcare professionals – to be aware of, and sensitive to, these difficulties.
Denial
It is interesting that adolescents, more than virtually every other age group, have a common, non-verbalized (and sometimes loudly verbalized) belief about their medical status: “I'm fine!” They don't want to feel sick; they don't want to be sick; and they don't want to be different. But lupus may throw a monkey wrench into that.
What makes it especially difficult, though, is that unless lupus is affecting the young person so aggressively that they are virtually unable to move, in many cases the young person's attitude will continue to be “I'm fine.” This can be frustrating for other family members, who are trying to be protective and helpful, and for healthcare professionals, who have more difficulty treating a young person if the answers to questions about symptoms are evasive or denying.
School
Adults who work generally have a clear sense of their responsibilities and obligations at their jobs; they know that if they don't work, changes will result – many of which can be difficult and unpleasant. Young people, whose primary job is to go to school, may go because they want to and because they know they are expected to. Yet they may not be aware of the far-reaching implications of inconsistent school attendance.
For young people with lupus, the attitude about school varies. Some are upset and frustrated if lupus interferes with consistent school attendance and schoolwork performance. Others see school as less important and may have few or no qualms about missing excessive time in school.
Young people with lupus may have a more difficult time in school if they have to deal with the cruelty of other children (“What is that ugly rash on your face?”) or the ignorance of teachers (“You've missed too much school work; either get with the program or you're going to fail.”) In addition, because of lupus, young people may find themselves ostracized and even excluded from activities that were once within their physical capabilities.
Peer pressure
Peer pressure may also affect young people with lupus. The need to “fit in” – at its strongest during childhood and adolescence – can be devastating to someone who has a chronic illness with noticeable physical effects (e.g., rashes, bloating, etc.) and behavioral effects (slower, more painful movements, etc.).
It is heartwarming to hear stories of young people with lupus whose friendships continue despite their illness. Yet it is sad to hear of other stories in which the person with lupus is ridiculed and even abandoned by former friends.
Parents
Young people with lupus may have difficulties with parents being overprotective (“Stay inside, the sun is out”), not protective enough (“You want to go to the beach? Do whatever you want”), or insensitive to their needs (“Stop complaining about your pain, already. Get up and finish your school work”).
Parents may be concerned about the effects their child's lupus will have on the family, such as financial issues, problems with or neglect of other children, or even feeling like their independence is being inhibited. Any of these concerns can likewise affect the young person with lupus. Already unhappy because of having lupus, but feeling responsible for problems within the family or with the parents, a young person may feel guilty to the extent that it interferes with their physical and emotional health.
For example, the young person may not tell parents about a serious lupus symptom, knowing it might mean another trip to the doctor or even the possibility of hospitalization.
Siblings
Brothers and sisters of a young person with lupus may be very resentful. Being less able to understand the physical impact of the disease, they may dislike the added “attention” being directed at their sick sibling. They also may not like getting less attention, and may act out in an attempt to regain their “share” of parental interactions. Their resentment toward their sick sibling may be manifested in many hurtful ways, such as anger, ignoring instructions, spiteful behavior, concealing important information from their parents, etc.
A final note
It is difficult enough for anyone to live with lupus, but the young person with lupus has added, age-related problems. Being aware of the potential impact of lupus in young people does not eliminate these problems. But increased awareness can pave the way to a better understanding of the unique needs of young people, and can lead to methods for better alleviating the problems that may occur.
Some Helpful Suggestions for Parents and Other Adults Dealing with Young People
Be Sensitive to the young Person's unique needs. Lupus can be a difficult disease to live with, especially for a child who has fewer “coping strategies” in place. Don't assume that the young person has the emotional strength or the social support network to handle lupus-related problems successfully.
Communicate appropriately. Try to look at any lupus-related issues through the eyes of the young person. See what they see. Feel what they feel. Using anger and aggressiveness in “forcing issues” is rarely productive. Calm, constructive discussion is a much more positive way to address lupus-related issues.
As much as possible, treat the young person like an adult. Plan together the appropriate ways to treat, and live with, lupus. Demonstrating adult-like behavior in interactions with young people is more likely to generate adult-like behavior in return.
Educate significant others. Any individuals who are not familiar with lupus, including family members, friends and teachers, can be obstacles to successful living with lupus. This is especially important in school, since the young person is going to spend a good number of hours there each day. Provide pamphlets and other information to teachers, guidance counselors and even classmates, so that school can truly be a “home away from home.”
Reprinted with permission of the Lupus Foundation of America, ©2001. Dr. Robert H. Phillips is founder and director of the Center for Coping in Long Island, NY ( www.coping.com) . He has been in private practice as a licensed psychologist since 1975 and has published and spoken widely on coping with physical ailments and other psychological topics. He has appeared on dozens of television and radio programs and currently is the host of “Coping Conversations, “ a weekly radio talk show on WKJY-FM (98.3) on Long Island .
Dr. Phillips is the published author of more than 20 books, including the highly popular Coping With Lupus (now completely revised and updated for its third edition); Lupus: Everything You Need to Know (with co-author Dr. Robert Lahita); and his new Successful Living With Lupus: An Action Workbook, published last year. These books, as well as several others, are available for sale from the Lupus Foundation of Minnesota (952-746-5151).
Dr. Phillips has served on the National Board of Directors of the Lupus Foundation of America and currently is a member of the Lupus News Advisory Board.
=========================================================== 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.
Kids Adapting to Lupus
Kids Adapting to Lupus
About 25 percent of lupus begins in childhood and adolescence, posing special problems. Pain, fatigue, the interruption of school and sports activities, limitations of mobility, change in appearance, and the feeling of being different are all particularly difficult for kids.
Lupus in childhood threatens the normal development process of gaining social and academic skills, the formation of a solid identity, and the development of independence and separation from parents. Very young children have rather concrete notions of disease, essentially that it is a result of accidents or catching germs from someone else. The more abstract reasoning required to understand lupus as an autoimmune disease does not usually develop fully before adolescence. In addition, children are focused on the immediate aspects and consequences of their disease, such as whether or not they can go to school or spend time with their friends or engage in any particular activity that day or that week. It's not until adolescence that children begin to understand the way in which their disease might interfere with their future goals. Depression stemming from this perception of future loss may be more of an issue for adolescents.
Lupus in children is essentially the same disease as that which occurs in adults. Some newborn infants of mothers with SLE experience abnormally slow heart rhythms and temporary skin rashes, the so-called "neonatal lupus syndrome", this is caused by antibodies that originate in the mother, but affect the newborn. This is not true lupus. In childhood, as in adulthood, true lupus can cause joint pain, fever, fatigue and butterfly rash as well as serious organ dysfunction, such as kidney or central nervous system involvement. Test results are similar, and treatment recommendations for childhood lupus are essentially the same as that for adults.
In most parts of this country, children with lupus are treated by pediatricians and often by a rheumatologist with special training in pediatric lupus. Generally, by the time patients reach adolescence, their care is transferred to an adult rheumatologist. However, the timing of this transfer may vary considerably and should be managed sensitively. Another significant difference with the treatment of lupus in children has to do with the effects of prednisone and other corticosteroids; calcium and vitamin D supplements are used to avoid or delay future problems with osteoporosis.
Over time, the adolescent patient should assume an increasingly autonomous role in her or his own care. They should be expected to do the talking in an appointment and have answers directed to them rather than to their parents. They should gradually gain responsibility for handling medications and scheduling their appointments while parents monitor the situation. They also have a right to confidentiality that should be respected.
However, as much as kids want to become independent from their parents, they are anxious about taking these steps, whether it be going away to college, or communication directly with their doctors, making appointments, or generally assuming responsibility for this aspect of their lives. They may forget their medications, appointments, and their need for rest and sleep. These are opportunities to provide additional guidance, reminders, and encouragement and not to usurp control of their healthcare. These are also good opportunities for open dialogue about the issues of independence and expectations of support. Studies of other chronic childhood diseases, such as asthma and diabetes, suggest that kids often fail to take their medications in the prescribed dosage. It is very important to involve children as active participants in their own health care.
Within the family, it is important to not treat the child with lupus differently than other children. For example, kids should not be over-sheltered or protected from the usual expectations or developmental challenges. Parents commonly feel guilty about their children with lupus because of the influence of genetic factors in its onset. However, they need to manage that guilt in such a way as not to become over-involved in the life of their ill child to the point of suspending their lives or neglecting the need of their other children.
Kids benefit from having some contact with adults who have had lupus and yet managed to live full and gratifying lives. One patient, Vicki Croke, now a journalist for the Boston Globe, has written about the importance of discovering the example of Henrietta Aladjem. When Vicki was quite ill she met "Hennie", whose disease by then had been in remission for many years, Vicki's hope was restored. Kids above all need a vision for themselves in the future and adult role models to help provide it. They also benefit from having contact with other kids with illness, either in support groups or in chat groups on the Internet. One nine year-old with lupus, Jessica, has a web site in which she described her illness, the disruption in her activities, the teasing about prednisone-related weight gain and her recovery. The teasing improved when her mom, a nurse, came to school and explained the illness to her class.
Fortunately, children and adolescents are extremely resilient. With the support of their family, friends, and doctors, most manage to negotiate the extra challenges imposed by illness. Sometimes counseling or psychotherapy can be helpful as well. At times parents need counseling even more than their children. If more difficulties or anxiety persist, psychiatric intervention and / or referral are recommended.
By Malcom P. Rogers, MD.
=========================================================== This information is for"informational purposes" and is not meant to be used for medical diagnosis. Alwaysconsult your physician on matters such as this.
About 25 percent of lupus begins in childhood and adolescence, posing special problems. Pain, fatigue, the interruption of school and sports activities, limitations of mobility, change in appearance, and the feeling of being different are all particularly difficult for kids.
Lupus in childhood threatens the normal development process of gaining social and academic skills, the formation of a solid identity, and the development of independence and separation from parents. Very young children have rather concrete notions of disease, essentially that it is a result of accidents or catching germs from someone else. The more abstract reasoning required to understand lupus as an autoimmune disease does not usually develop fully before adolescence. In addition, children are focused on the immediate aspects and consequences of their disease, such as whether or not they can go to school or spend time with their friends or engage in any particular activity that day or that week. It's not until adolescence that children begin to understand the way in which their disease might interfere with their future goals. Depression stemming from this perception of future loss may be more of an issue for adolescents.
Lupus in children is essentially the same disease as that which occurs in adults. Some newborn infants of mothers with SLE experience abnormally slow heart rhythms and temporary skin rashes, the so-called "neonatal lupus syndrome", this is caused by antibodies that originate in the mother, but affect the newborn. This is not true lupus. In childhood, as in adulthood, true lupus can cause joint pain, fever, fatigue and butterfly rash as well as serious organ dysfunction, such as kidney or central nervous system involvement. Test results are similar, and treatment recommendations for childhood lupus are essentially the same as that for adults.
In most parts of this country, children with lupus are treated by pediatricians and often by a rheumatologist with special training in pediatric lupus. Generally, by the time patients reach adolescence, their care is transferred to an adult rheumatologist. However, the timing of this transfer may vary considerably and should be managed sensitively. Another significant difference with the treatment of lupus in children has to do with the effects of prednisone and other corticosteroids; calcium and vitamin D supplements are used to avoid or delay future problems with osteoporosis.
Over time, the adolescent patient should assume an increasingly autonomous role in her or his own care. They should be expected to do the talking in an appointment and have answers directed to them rather than to their parents. They should gradually gain responsibility for handling medications and scheduling their appointments while parents monitor the situation. They also have a right to confidentiality that should be respected.
However, as much as kids want to become independent from their parents, they are anxious about taking these steps, whether it be going away to college, or communication directly with their doctors, making appointments, or generally assuming responsibility for this aspect of their lives. They may forget their medications, appointments, and their need for rest and sleep. These are opportunities to provide additional guidance, reminders, and encouragement and not to usurp control of their healthcare. These are also good opportunities for open dialogue about the issues of independence and expectations of support. Studies of other chronic childhood diseases, such as asthma and diabetes, suggest that kids often fail to take their medications in the prescribed dosage. It is very important to involve children as active participants in their own health care.
Within the family, it is important to not treat the child with lupus differently than other children. For example, kids should not be over-sheltered or protected from the usual expectations or developmental challenges. Parents commonly feel guilty about their children with lupus because of the influence of genetic factors in its onset. However, they need to manage that guilt in such a way as not to become over-involved in the life of their ill child to the point of suspending their lives or neglecting the need of their other children.
Kids benefit from having some contact with adults who have had lupus and yet managed to live full and gratifying lives. One patient, Vicki Croke, now a journalist for the Boston Globe, has written about the importance of discovering the example of Henrietta Aladjem. When Vicki was quite ill she met "Hennie", whose disease by then had been in remission for many years, Vicki's hope was restored. Kids above all need a vision for themselves in the future and adult role models to help provide it. They also benefit from having contact with other kids with illness, either in support groups or in chat groups on the Internet. One nine year-old with lupus, Jessica, has a web site in which she described her illness, the disruption in her activities, the teasing about prednisone-related weight gain and her recovery. The teasing improved when her mom, a nurse, came to school and explained the illness to her class.
Fortunately, children and adolescents are extremely resilient. With the support of their family, friends, and doctors, most manage to negotiate the extra challenges imposed by illness. Sometimes counseling or psychotherapy can be helpful as well. At times parents need counseling even more than their children. If more difficulties or anxiety persist, psychiatric intervention and / or referral are recommended.
By Malcom P. Rogers, MD.
=========================================================== This information is for"informational purposes" and is not meant to be used for medical diagnosis. Alwaysconsult your physician on matters such as this.
Tuesday, March 13, 2007
The Genetic Basis of Lupus
The Genetic Basis of Lupus
Lupus is an illness in which the immune system appears to have gotten confused. Instead of attacking viruses, bacteria or cancer cells – which is what it's there to do – the immune system attacks the person's own body.
The evidence that lupus is a genetic disease is quite clear. Lupus runs in families. If you have lupus, there's a five percent chance that one of your siblings will get the disease.
If you are a non-identical twin, the chances are about the same as for a sister, suggesting that nothing major happens in the womb to cause lupus. But if you are an identical twin – and your twin has lupus – the chances go up to 57 percent that you will develop the disease. Thus, there is strong evidence for a genetic basis, but it is clearly not a simple genetic basis.
If just one gene you could inherit always caused lupus, at least one out of four siblings should get lupus, considering the classic laws of genetics. Some people who are related to lupus patients have various lupus-like symptoms, suggesting that they have acquired “incomplete lupus” without developing the full-blown disease.
So how do we explain that? And, since identical twins are genetically identical, why does lupus affect only 57 percent of identical twins? We're just beginning to understand that there are several possible explanations.
Multiple Genes Involved
The first important concept is that more than one gene may be involved. One-fourth of your siblings should inherit any one gene, but if three, four or more different genes need to be inherited together, this doesn't explain why both identical twins don't get the disease, since they inherit the same genes.
A concept that could explain why some identical twins of lupus patients are spared is called gene penetrance. This means that you can have a gene – and that gene may cause you to be susceptible to a disease – but the illness still doesn't show up. This is possible because something in the environment is needed to get the disease started. This environment can be either outside your body or inside your body, which is greatly influenced by infections or toxins, or even your other genes. The idea that a certain gene could be there – but not expressing itself completely – is called “incomplete penetrance.”
A third concept, developed in recent years, is that there are cases where genes actually change or rearrange themselves in the body after the first cell divides at the moment of creation. So even identical twins can end up with somewhat different genes that develop later, after they have separated from each other in the womb, or after birth. This can be true for certain genes that have to do with the immune system.
The Role of Genes
What do we know about lupus that helps us to understand the roles of genes? First, there is the predominance of women to men who get the disease. It seems likely that this has to do with a direct effect of hormones on the immune system. Female hormones may help create the environment that allows a lupus gene to penetrate.
Much research is being done to identify the genes that lupus patients share. The ones that are best understood are a series of genes which regulate how the immune system works and where and when it might attack.
These genes are part of the network that is involved in tissue typing. They allow the immune system to recognize and attack foreign invasions from viruses, bacteria, or cancer cells, and to distinguish them from things that should not be attacked, such as parts of the body or tissue-typed organ transplants.
If you were looking for a defective gene that causes lupus, major histocompatibility complex (MHC) genes, whose normal function is to regulate immune responsiveness, would be good candidates since the immune system in lupus gets confused into attacking a person's own body. And, in fact, MHC genes are shared by many lupus patients.
How Genes Work
How do these genes work? They make proteins that act very much like the Lord Chamberlain to a very paranoid queen. Imagine a world in your bloodstream that is rather like medieval times with lots of small castles, each inhabited by a different queen, each queen waited on by a special court made up of one Lord Chamberlain and a lot of little soldiers.
If an infection enters the bloodstream, an invading particle will be picked up by a special Lord Chamberlain, who is genetically programmed to recognize it. He takes the particle back to his own castle and formally presents it to the queen as if it were the ambassador from a foreign country. The queen takes one look at the particle, shouts, “Off with its head!” – and all sorts of things start to happen.
First, immune-fighting cells get made. Then little proteins called antibodies get made. These are like little soldiers who specifically know how to recognize that original invading particle. So they leave cells and run around the blood stream attacking anything that looks to them like the particle.
If they get confused and think your kidney or joints look like an invading particle, then you might develop lupus. But the antibodies would never have started attacking if the Queen hadn't started shouting. And the Queen would have kept quiet if the Lord Chamberlain hadn't brought the particle into her castle.
The Lord Chamberlain (who is an MHC molecule) may be shared by many lupus patients and may be one of primary genes that put people at risk. The Queen (who is called the I Cell Receptor) and the antibodies are examples of immune genes that can rearrange themselves after the beginning of life, so they may or may not be shared exactly in families, even by identical twins. This could explain why an identical twin of a lupus patient might have more risk of developing lupus than another sister, but not a 100 percent risk.
Self-Attacking Genes
But why do lupus patients carry these genes that can start an attack on their own bodies – and what prevents other people from doing the same thing? In order to have a diverse ability to recognize and protect the body from diverse infections over a lifetime, everybody has some genes capable of making proteins that attack their own organs.
However, immune cells go through a complex educational process early in life in the thymus gland, where they are taught to recognize the difference between “us” and “them” – and what sorts of invaders it is appropriate to attack. Those that misbehave and threaten to attack parts of their own bodies are usually simply killed off by a process called apoptosis. But the well-behaved immune cells “graduate” from the thymus and are allowed to enter the bloodstream.
A gene which regulates the system that eliminates self-attacking cells in the thymus gland is defective in some mice with a lupus-like illness. The jury is still out on whether this is the fact in human lupus – but many researchers are now considering the possibility.
I cannot go into all the other genes that seem to put people at increased risk for lupus. But one important set of genes gives rise to special inflammatory proteins called complement proteins. These proteins act something like the artillery used by the antibody soldiers when they attack, and defects to some of the complement genes have been described in lupus and lupus-like illnesses.
Continued Research Needed
In summary, lupus is a complicated disease with a complicated genetic basis. It involves several genes we know of, and probably more not yet identified, that are important to regulating appropriate immune activity.
There are two reasons why continued research into the causes and genetic basis of lupus is very important – first and foremost, to improve the care of lupus patients. Although treatments are better than 20 years ago, and we encourage lupus patients to have optimism for a fairly normal life, people still die from lupus or from side effects of the medications. And others become very ill and suffer major organ damage. Patient care could be better if we knew more.
Second, what we learn by studying lupus has profound implications for better understanding and treatment for many other diseases. This point should not be forgotten when writing to Congresspersons or fundraising for the Lupus Foundation.
Lupus research provides a large picture window into the mysteries of the immune system and will very likely contribute to the understanding and treatment of AIDS, heart disease, cancer, diabetes and many other diseases. This research includes the one disease that costs more than any other – and affects everyone on earth – the process of aging itself.
By Joan T. Merrill, MD, St. Luke's-Roosevelt Hospital Center, New York City. Reprinted with permission from the newsletter of the SLE Foundation, New York.
=========================================================== 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 is an illness in which the immune system appears to have gotten confused. Instead of attacking viruses, bacteria or cancer cells – which is what it's there to do – the immune system attacks the person's own body.
The evidence that lupus is a genetic disease is quite clear. Lupus runs in families. If you have lupus, there's a five percent chance that one of your siblings will get the disease.
If you are a non-identical twin, the chances are about the same as for a sister, suggesting that nothing major happens in the womb to cause lupus. But if you are an identical twin – and your twin has lupus – the chances go up to 57 percent that you will develop the disease. Thus, there is strong evidence for a genetic basis, but it is clearly not a simple genetic basis.
If just one gene you could inherit always caused lupus, at least one out of four siblings should get lupus, considering the classic laws of genetics. Some people who are related to lupus patients have various lupus-like symptoms, suggesting that they have acquired “incomplete lupus” without developing the full-blown disease.
So how do we explain that? And, since identical twins are genetically identical, why does lupus affect only 57 percent of identical twins? We're just beginning to understand that there are several possible explanations.
Multiple Genes Involved
The first important concept is that more than one gene may be involved. One-fourth of your siblings should inherit any one gene, but if three, four or more different genes need to be inherited together, this doesn't explain why both identical twins don't get the disease, since they inherit the same genes.
A concept that could explain why some identical twins of lupus patients are spared is called gene penetrance. This means that you can have a gene – and that gene may cause you to be susceptible to a disease – but the illness still doesn't show up. This is possible because something in the environment is needed to get the disease started. This environment can be either outside your body or inside your body, which is greatly influenced by infections or toxins, or even your other genes. The idea that a certain gene could be there – but not expressing itself completely – is called “incomplete penetrance.”
A third concept, developed in recent years, is that there are cases where genes actually change or rearrange themselves in the body after the first cell divides at the moment of creation. So even identical twins can end up with somewhat different genes that develop later, after they have separated from each other in the womb, or after birth. This can be true for certain genes that have to do with the immune system.
The Role of Genes
What do we know about lupus that helps us to understand the roles of genes? First, there is the predominance of women to men who get the disease. It seems likely that this has to do with a direct effect of hormones on the immune system. Female hormones may help create the environment that allows a lupus gene to penetrate.
Much research is being done to identify the genes that lupus patients share. The ones that are best understood are a series of genes which regulate how the immune system works and where and when it might attack.
These genes are part of the network that is involved in tissue typing. They allow the immune system to recognize and attack foreign invasions from viruses, bacteria, or cancer cells, and to distinguish them from things that should not be attacked, such as parts of the body or tissue-typed organ transplants.
If you were looking for a defective gene that causes lupus, major histocompatibility complex (MHC) genes, whose normal function is to regulate immune responsiveness, would be good candidates since the immune system in lupus gets confused into attacking a person's own body. And, in fact, MHC genes are shared by many lupus patients.
How Genes Work
How do these genes work? They make proteins that act very much like the Lord Chamberlain to a very paranoid queen. Imagine a world in your bloodstream that is rather like medieval times with lots of small castles, each inhabited by a different queen, each queen waited on by a special court made up of one Lord Chamberlain and a lot of little soldiers.
If an infection enters the bloodstream, an invading particle will be picked up by a special Lord Chamberlain, who is genetically programmed to recognize it. He takes the particle back to his own castle and formally presents it to the queen as if it were the ambassador from a foreign country. The queen takes one look at the particle, shouts, “Off with its head!” – and all sorts of things start to happen.
First, immune-fighting cells get made. Then little proteins called antibodies get made. These are like little soldiers who specifically know how to recognize that original invading particle. So they leave cells and run around the blood stream attacking anything that looks to them like the particle.
If they get confused and think your kidney or joints look like an invading particle, then you might develop lupus. But the antibodies would never have started attacking if the Queen hadn't started shouting. And the Queen would have kept quiet if the Lord Chamberlain hadn't brought the particle into her castle.
The Lord Chamberlain (who is an MHC molecule) may be shared by many lupus patients and may be one of primary genes that put people at risk. The Queen (who is called the I Cell Receptor) and the antibodies are examples of immune genes that can rearrange themselves after the beginning of life, so they may or may not be shared exactly in families, even by identical twins. This could explain why an identical twin of a lupus patient might have more risk of developing lupus than another sister, but not a 100 percent risk.
Self-Attacking Genes
But why do lupus patients carry these genes that can start an attack on their own bodies – and what prevents other people from doing the same thing? In order to have a diverse ability to recognize and protect the body from diverse infections over a lifetime, everybody has some genes capable of making proteins that attack their own organs.
However, immune cells go through a complex educational process early in life in the thymus gland, where they are taught to recognize the difference between “us” and “them” – and what sorts of invaders it is appropriate to attack. Those that misbehave and threaten to attack parts of their own bodies are usually simply killed off by a process called apoptosis. But the well-behaved immune cells “graduate” from the thymus and are allowed to enter the bloodstream.
A gene which regulates the system that eliminates self-attacking cells in the thymus gland is defective in some mice with a lupus-like illness. The jury is still out on whether this is the fact in human lupus – but many researchers are now considering the possibility.
I cannot go into all the other genes that seem to put people at increased risk for lupus. But one important set of genes gives rise to special inflammatory proteins called complement proteins. These proteins act something like the artillery used by the antibody soldiers when they attack, and defects to some of the complement genes have been described in lupus and lupus-like illnesses.
Continued Research Needed
In summary, lupus is a complicated disease with a complicated genetic basis. It involves several genes we know of, and probably more not yet identified, that are important to regulating appropriate immune activity.
There are two reasons why continued research into the causes and genetic basis of lupus is very important – first and foremost, to improve the care of lupus patients. Although treatments are better than 20 years ago, and we encourage lupus patients to have optimism for a fairly normal life, people still die from lupus or from side effects of the medications. And others become very ill and suffer major organ damage. Patient care could be better if we knew more.
Second, what we learn by studying lupus has profound implications for better understanding and treatment for many other diseases. This point should not be forgotten when writing to Congresspersons or fundraising for the Lupus Foundation.
Lupus research provides a large picture window into the mysteries of the immune system and will very likely contribute to the understanding and treatment of AIDS, heart disease, cancer, diabetes and many other diseases. This research includes the one disease that costs more than any other – and affects everyone on earth – the process of aging itself.
By Joan T. Merrill, MD, St. Luke's-Roosevelt Hospital Center, New York City. Reprinted with permission from the newsletter of the SLE Foundation, New York.
=========================================================== This information is for"informational purposes" and is not meant to be used for medical diagnosis. Alwaysconsult your physician on matters such as this.
Difficult-to-Manage Lupus
Difficult-to-Manage Lupus
Having treated over 2,000 lupus patients over the last 20 years, I was intrigued when the editor of Lupus Update asked me to write about “Difficult to Manage Lupus.” After all, isn't all lupus difficult to manage? However, there are certain situations that are more difficult to manage than others. Ten patient cases are presented here:
When not to overtreat? Resistant non-organ-threatening disease?
A patient with Systemic Lupus Erythematosus (SLE) has persistently active rashes, fevers, fatigue and pain on taking a deep breath despite nonsteroidals and Plaquenil. There is no evidence for heart, lung, liver, central nervous system, hematologic or renal involvement. There are circumstances when certain treatments often do more harm than good if oral steroids or methotrexate are added in this situation, so I might try a few other things first. Consider increasing Plaquenil, consider DHEA, use very high dose nonsteroids or a local one-time only steroid boost.
Cyclophosphamide (Cytoxan) resistant lupus nephritis
Fifteen to twenty percent of all individuals with SLE have lupus in their kidneys and biopsies showing proliferative disease. With no treatment, they will be on dialysis within 2-3 years. Although Cytoxan is the treatment of choice for them, this toxic therapy does not work or wears off 30-40 percent of the time. What do I do? My options include: consider adding azathioprine (Imuran) to the Cytoxan and continue treatments; add plasmapheresis or pulse-dose steroids; or substitute nitrogen mustard for Cytoxan. Consider rebiopsying the patient and make sure that the renal lesion is reversible. Sometimes, it's better to let a patient go on to dialysis and transplant them than treat them further. Newer treatments may be utilized: cyclosporin, mycophenolate mofetil (CellCept) or tacrillimus (Prograf, FK 506). Drug trials with LJP394 and Biogen's antiCD40 ligand are available at selected medical centers.
Refractory chronic cutaneous lupus with no systemic disease
A patient has lupus rashes covering 70 percent of the body, but their ANA is negative and all blood work is normal. Plaquenil has not helped. I have had some success with: switching from Plaquenil to Chloroquine and adding quinacrine, retinoids such as Accutante or Soriatene; antileprosy drugs including thalidomide, clofazimine or dapsone; topical nitrogen mustard or BCNU.
Lifestyle-altering central nervous system (CNS) symptoms with a normal MRI scan of the brain and blood tests showing slight activity
When my patients complain about not thinking clearly, severe headaches and profound fatigue, the issue is raised as to whether it could be vasculitis of the CNS. True CNS vasculitis in SLE is usually obvious (e.g., fevers, meningitis-like picture, psychosis, seizures), and responds to high dose steroids. But could the patient have “subclinical vasculitis?” In fact, this is rare and most often the symptoms are due to abnormal blood flow to the brain due to a dysfunction of the autonomic nervous system and / or the dysfunction of chemicals known as cytokines (interleukins, interferons, etc.). I frequently order a SPECT scan (which might include antineural antibodies) and antiribosomal P antibodies and a spinal tap. Make sure that your doctor obtains LE cells, oligoclonal bands, antineuronal antibodies and IgG synthesis rate in addition to the usual determinations.
Cognitive impairment in patients without CNS vasculitis
How do we treat the patient in number 4 (above) who does not have vasculitis? Interventions are useful that improve the blood flow to the brain regulated by the autonomic nervous system (which controls the dilation or constriction of blood vessels, thus regulating our pulse and blood pressure), such as: biofeedback, relaxation techniques, cognitive therapy and counseling. Additionally, serotonin boosters (Prozac, Zoloft, Paxil) may help give a patient more energy and clarity. Antimalarials (Plaquenil, quinacrine) and DHEA can be useful. Steroids may seem to help at first but make things worse in the long run and should be avoided unless there is evidence for inflammation.
More than one miscarriage in a patient without anticardiolipin antibodies
Antiphospholipid antibodies and the circulating lupus anticoagulant can cause miscarriages. Most primary care doctors stop the workup after obtaining a negative anticardiolipin antibody and circulating anticoagulant test and don't treat the patient. On the other hand, aggressive reproductive immunologists unnecessarily treat patients with expensive and toxic approaches such as heparin, prednisone and intravenous gamma globulin for subsequent pregnancies which would be successful in any case. Active lupus by itself can cause miscarriages. I'm in the middle. I check for three to four different phospholipid antibodies, Protein C, Protein S, antithrombin III, Factor V Leiden mutation, BDRL and kaolin PTTs.
Is the muscle and joint aching a lupus flare or fibromyalgia?
Fibromyalgia can be as discomforting as lupus-associated inflammation but is made worse by corticosteroids. It is important not to inappropriately treat. I generally only treat symptoms of lupus with higher doses of anti-inflammatory medicine when there is objective evidence of joint swelling (synovitis), a high CPK (muscle enzyme), a high sed rate or CRP (blood tests for inflammation), low C3 complement or high anti-DNA. Lacking this, sometimes I have had to resort to obtaining a bone scan to assess if somebody with profound musculoskeletal discomfort and SLE is inflamed or experiencing a flare or fibromyalgia. The latter is treated with tricyclics, serotonin boosters and muscle relaxants and is seen in 25 percent of lupus patients.
The patients with non-organ threatening disease who want to treat their disease “naturally”
Twenty percent of patients with non-organ threatening disease will evolve organ threatening disease over five years, but this percentage decreases to five percent with two years of Plaquenil. No herb or spice has been shown to be specifically effective for SLE, and studies are sorely needed. None of these preparations, marketed as nutritional supplements to avoid FDA regulation, are standardized. Avoid believing testimonials and only rely on peer-reviewed published controlled studies. Let the buyer beware.
My eight-year-old daughter has aches and a positive ANA. Should I be worried?
Ten percent of women with SLE will have a daughter with the disease, and two percent a son with it. Twenty percent with SLE will have an offspring with an autoimmune disorder (most commonly autoimmune thyroid disease). Fifty percent of children of lupus patients have a positive ANA. Girls who are prepubertal develop lupus very rarely. Their positive ANA is inherited and their aches are usually due to growing pains. We usually advise against ANA or antibody testing unless there is objective evidence for a problem such as a fever, swollen joints or rash.
The 70-year-old woman with a diagnosis of new-onset lupus
Ten percent of the population develops ANAs as they age. When senior citizens are found to have a positive ANA and have a high sedimentation rate and joint aches, they often come to rheumatologists with a diagnosis of lupus. In reality, the overwhelming majority do not have the disease. Polymyalgia rheumatica, fibromyalgia, rheumatoid arthritis and particularly Sjogren's syndrome need to be ruled out.
By Daniel J. Wallace, MD, Clinical Professor of Medicine, UCLA School of Medicine. Reprinted with permission from the Maryland Lupus Foundation. Dr. Wallace is the author of “The Lupus Book: A Guide for Patients and their Families”.
=========================================================== This information is for"informational purposes" and is not meant to be used for medical diagnosis. Alwaysconsult your physician on matters such as this.
Having treated over 2,000 lupus patients over the last 20 years, I was intrigued when the editor of Lupus Update asked me to write about “Difficult to Manage Lupus.” After all, isn't all lupus difficult to manage? However, there are certain situations that are more difficult to manage than others. Ten patient cases are presented here:
When not to overtreat? Resistant non-organ-threatening disease?
A patient with Systemic Lupus Erythematosus (SLE) has persistently active rashes, fevers, fatigue and pain on taking a deep breath despite nonsteroidals and Plaquenil. There is no evidence for heart, lung, liver, central nervous system, hematologic or renal involvement. There are circumstances when certain treatments often do more harm than good if oral steroids or methotrexate are added in this situation, so I might try a few other things first. Consider increasing Plaquenil, consider DHEA, use very high dose nonsteroids or a local one-time only steroid boost.
Cyclophosphamide (Cytoxan) resistant lupus nephritis
Fifteen to twenty percent of all individuals with SLE have lupus in their kidneys and biopsies showing proliferative disease. With no treatment, they will be on dialysis within 2-3 years. Although Cytoxan is the treatment of choice for them, this toxic therapy does not work or wears off 30-40 percent of the time. What do I do? My options include: consider adding azathioprine (Imuran) to the Cytoxan and continue treatments; add plasmapheresis or pulse-dose steroids; or substitute nitrogen mustard for Cytoxan. Consider rebiopsying the patient and make sure that the renal lesion is reversible. Sometimes, it's better to let a patient go on to dialysis and transplant them than treat them further. Newer treatments may be utilized: cyclosporin, mycophenolate mofetil (CellCept) or tacrillimus (Prograf, FK 506). Drug trials with LJP394 and Biogen's antiCD40 ligand are available at selected medical centers.
Refractory chronic cutaneous lupus with no systemic disease
A patient has lupus rashes covering 70 percent of the body, but their ANA is negative and all blood work is normal. Plaquenil has not helped. I have had some success with: switching from Plaquenil to Chloroquine and adding quinacrine, retinoids such as Accutante or Soriatene; antileprosy drugs including thalidomide, clofazimine or dapsone; topical nitrogen mustard or BCNU.
Lifestyle-altering central nervous system (CNS) symptoms with a normal MRI scan of the brain and blood tests showing slight activity
When my patients complain about not thinking clearly, severe headaches and profound fatigue, the issue is raised as to whether it could be vasculitis of the CNS. True CNS vasculitis in SLE is usually obvious (e.g., fevers, meningitis-like picture, psychosis, seizures), and responds to high dose steroids. But could the patient have “subclinical vasculitis?” In fact, this is rare and most often the symptoms are due to abnormal blood flow to the brain due to a dysfunction of the autonomic nervous system and / or the dysfunction of chemicals known as cytokines (interleukins, interferons, etc.). I frequently order a SPECT scan (which might include antineural antibodies) and antiribosomal P antibodies and a spinal tap. Make sure that your doctor obtains LE cells, oligoclonal bands, antineuronal antibodies and IgG synthesis rate in addition to the usual determinations.
Cognitive impairment in patients without CNS vasculitis
How do we treat the patient in number 4 (above) who does not have vasculitis? Interventions are useful that improve the blood flow to the brain regulated by the autonomic nervous system (which controls the dilation or constriction of blood vessels, thus regulating our pulse and blood pressure), such as: biofeedback, relaxation techniques, cognitive therapy and counseling. Additionally, serotonin boosters (Prozac, Zoloft, Paxil) may help give a patient more energy and clarity. Antimalarials (Plaquenil, quinacrine) and DHEA can be useful. Steroids may seem to help at first but make things worse in the long run and should be avoided unless there is evidence for inflammation.
More than one miscarriage in a patient without anticardiolipin antibodies
Antiphospholipid antibodies and the circulating lupus anticoagulant can cause miscarriages. Most primary care doctors stop the workup after obtaining a negative anticardiolipin antibody and circulating anticoagulant test and don't treat the patient. On the other hand, aggressive reproductive immunologists unnecessarily treat patients with expensive and toxic approaches such as heparin, prednisone and intravenous gamma globulin for subsequent pregnancies which would be successful in any case. Active lupus by itself can cause miscarriages. I'm in the middle. I check for three to four different phospholipid antibodies, Protein C, Protein S, antithrombin III, Factor V Leiden mutation, BDRL and kaolin PTTs.
Is the muscle and joint aching a lupus flare or fibromyalgia?
Fibromyalgia can be as discomforting as lupus-associated inflammation but is made worse by corticosteroids. It is important not to inappropriately treat. I generally only treat symptoms of lupus with higher doses of anti-inflammatory medicine when there is objective evidence of joint swelling (synovitis), a high CPK (muscle enzyme), a high sed rate or CRP (blood tests for inflammation), low C3 complement or high anti-DNA. Lacking this, sometimes I have had to resort to obtaining a bone scan to assess if somebody with profound musculoskeletal discomfort and SLE is inflamed or experiencing a flare or fibromyalgia. The latter is treated with tricyclics, serotonin boosters and muscle relaxants and is seen in 25 percent of lupus patients.
The patients with non-organ threatening disease who want to treat their disease “naturally”
Twenty percent of patients with non-organ threatening disease will evolve organ threatening disease over five years, but this percentage decreases to five percent with two years of Plaquenil. No herb or spice has been shown to be specifically effective for SLE, and studies are sorely needed. None of these preparations, marketed as nutritional supplements to avoid FDA regulation, are standardized. Avoid believing testimonials and only rely on peer-reviewed published controlled studies. Let the buyer beware.
My eight-year-old daughter has aches and a positive ANA. Should I be worried?
Ten percent of women with SLE will have a daughter with the disease, and two percent a son with it. Twenty percent with SLE will have an offspring with an autoimmune disorder (most commonly autoimmune thyroid disease). Fifty percent of children of lupus patients have a positive ANA. Girls who are prepubertal develop lupus very rarely. Their positive ANA is inherited and their aches are usually due to growing pains. We usually advise against ANA or antibody testing unless there is objective evidence for a problem such as a fever, swollen joints or rash.
The 70-year-old woman with a diagnosis of new-onset lupus
Ten percent of the population develops ANAs as they age. When senior citizens are found to have a positive ANA and have a high sedimentation rate and joint aches, they often come to rheumatologists with a diagnosis of lupus. In reality, the overwhelming majority do not have the disease. Polymyalgia rheumatica, fibromyalgia, rheumatoid arthritis and particularly Sjogren's syndrome need to be ruled out.
By Daniel J. Wallace, MD, Clinical Professor of Medicine, UCLA School of Medicine. Reprinted with permission from the Maryland Lupus Foundation. Dr. Wallace is the author of “The Lupus Book: A Guide for Patients and their Families”.
=========================================================== This information is for"informational purposes" and is not meant to be used for medical diagnosis. Alwaysconsult your physician on matters such as this.
Wednesday, December 20, 2006
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.
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.
Thursday, October 19, 2006
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.
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.
Tuesday, August 29, 2006
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.
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.
Mary P. Brassell, MA, CRRN
Many couples experience some type of sexual difficulty when one partner has a chronic disease such as lupus. Sexuality may be affected by disease symptoms, medications and/or psychological concerns. Disease symptoms of lupus which may influence sexual activity include: oral ulcers, vaginal ulcers, arthritis, Sjogren's syndrome, and Raynaud's phenomenon. Each of these problems will be discussed in detail. Oral ulcers occur in about 10-15% of people with lupus.
They can interfere with pleasant oral sensations. A prescription mouthwash with antibiotics and/or steroids may be necessary and can help heal the ulcers. Remember, they do not last forever! Vaginal ulcers are present in less than 5% of patients with lupus. They are rarely painful but when they are they can interfere with intercourse. A prescribed steroid cream or other medication can be used to treat them. Alternate forms of sexual expression can also be used until healing has taken place. Joint pains or arthritis that often accompany lupus may respond to warm baths, a few warming up exercises, and taking anti-inflammatory medication an hour before sexual activity. The vaginal dryness associated with Sjogren's syndrome responds well to the use of a water soluble lubricant which is absorbed and does not have to be removed. (Vaseline-like ointments should be avoided since they can encourage infection.) Water soluble lubricants (e.g. K-Y jelly) are available over the counter at drugstores or supermarkets.
People who have Raynaud's phenomenon have a condition in which blood vessels constrict (become narrower) when they are exposed to cold. It can be very painful. Raynaud's can cause fingers and toes to change color (first white, then blue, then red). Here are some solutions: Avoid having sex in an air conditioned room. Wear socks if your feet are sensitive to cold. Try a warm bath (not hot) prior to sexual activity as it will help open the blood vessels. During sex, blood pools in the genital area and less blood goes to the fingers and toes. Take the bottom position - it helps avoid the pressure on hands and feet that can further reduce blood flow. Medications can certainly influence sex life. Tranquilizers (antianxiety agents), anti-hypertensives (drugs that lower high blood pressure), and corticosteroids (prednisone) can affect both sexual desire (libido) and performance.
Some anti-hypertensives decrease libido in men and women as well as produce temporary impotence. It is best to discuss these drugs with your doctor, who may be able to prescribe a different blood pressure drug that won't interfere with sexual performance. A small percentage of men and women have experienced libido changes while on steroids. If you experience any of these changes, talk with your doctor. Psychological factors can also have a powerful influence on a satisfying sex life. If the person with lupus has developed low self esteem, unhappiness about the way their body looks, feelings of worthlessness, depression, fatigue, and feelings of inadequacy, the sexual relationship will certainly be affected. The patient may withdraw from his/her partner.
The healthy partner may feel that this withdrawal means rejection and the loss of affection and love. If communication does not occur, the relationship may be in serious jeopardy. Sexual pleasure is an important aspect of any relationship. Some studies have reported that arthritis patients are free from joint pain for up to six hours after intercourse. Sex, therefore, seems to be therapeutic. Even if intercourse is not possible, other forms of sexual expression such as cuddling, holding, stroking, kissing, and closeness are all manifestations of sexual affection that can help reinforce your sense of self-worth and desirability.
Many publications contain various suggestions for different methods of sexual expression. The Arthritis Foundation has a publication titled "Living and Loving with Arthritis". It costs less than a dollar and can be obtained from the local branch of the Arthritis Foundation. This booklet contains information about sexual expression, illustrations of comfortable positions to assume during sexual activity, and some sound advice about coping with sexual challenges. Suggestions for improving a sexual relationship are offered. Sexuality is a part of life. It is a normal and usual activity of daily living. Whether sexual problems are physical or emotional or both, willingness by botb partners to discuss and search for solutions to sexual problems is essential.
Help in reaching solutions can be sought from a physician, from nurses in the field of arthritis and lupus, from psychologists, and/or from sex therapists. infon-nation is readily available at low cost. All that is required is that the patient and partner find the necessary person or publication. Sexuality is best thought of as another form of communication that helps couples enjoy each other and deepen their intimacy in a loving relationship. Sometimes if this communication does not happen, other parts of the relationship will suffer. Solutions can be achieved with a little effort by both patient and partner, but there must be an effort!
=========================================================== This information is for"informational purposes" and is not meant to be used for medical diagnosis. Alwaysconsult your physician on matters such as this.
Tuesday, August 01, 2006
Rehabilitation in Lupus
Rehabilitation in Lupus
Bertram Greenspun, DO
Rehabilitation medicine is the field of medicine that concerns itself not only with how the disease process has affected the individual but also with how the disease has changed the person's ability to maintain the lifestyle and roles in life which they had before the disease started.
The rehabilitation team concentrates on how to improve the person's functioning and independence to the highest possible level. Systemic Lupus Erythematosus (SLE) at times presents problems that can be helped by an individualized rehabilitation program. Among the body systems that can be affected by lupus (and may be helped by rehabilitation) are the central nervous system (CNS), the musculoskeletal system (muscles, bones and joints), and the vascular system (blood vessels).
The CNS can be involved in a variety of ways. Patients who develop stroke-like syndromes or problems of the spinal cord usually require an in-patient program in a comprehensive rehabilitation center. These problems often result in weakness of one or both arms or legs. Weakness can be so profound that it may result in paralysis. Under such circumstances, passive range of motion (ROM) exercises should be started by the physical therapist (PT). Family and friends may also be instructed by the physical therapist to perform ROM exercises for the patient. These exercises are done without the patient having to do any of the work. Since some degree of strength almost always returns when the stroke or the spinal cord damage resolves, it is vital not to allow contractures to develop.
Contractures happen when the joints are allowed to stiffen to the point where they will be unable to bend. Should this occur, even if muscle power returns it will do no good unless the stiffness is reduced or eliminated. This can take a very long time and even require surgery. It is much wiser and easier to prevent contractures in the first place than it is to correct them after the fact. As the patient improves, he/she can move the joint along with the physical therapist. Then the exercise is known as active-assistive ROM. When the patient can move the joint through the entire ROM, he/she no longer needs the assistance of another person and is instructed on how to do regular, active ROM exercises independently.
In addition to ROM exercises, strengthening exercises can be started under proper supervision as soon as is medically possible. The strengthening program should begin at a low intensity that is well within the capability of the patient. It is increased only gradually and slowly. The patient must learn to "listen" to his/her body. This means that if muscle pain or fatigue lasts for more than an hour to an hour and a half after a session, the level of exercise intensity should be temporarily reduced. if the discomfort or fatigue continues after each session the physician who prescribed the program should be notified.
Too much exercise, done too quickly, can easily make the situation worse. If the weakness is associated with acute inflammatory joint pain, isometric exercises can be helpful. Isometric exercises involve contracting the muscles without moving the involved joint. In this way strength can be maintained without triggering the intense pain associated with moving an acutely inflamed joint. Since isometric exercises can cause the blood pressure to rise they should be performed cautiously and without holding the breath, especially in the individual with hypertension. Almost all patients with lupus will, at some point in the disease process, have joint pain. There is often associated morning stiffness and joint swelling. Tenderness and painful movement are common. Deformities may result. Most of these problems can be dealt with on an out-patient basis.
An early morning shower will frequently shorten the duration of morning stiffness. Moist heat is quite helpful for most joint problems and is used prior to ROM exercises. This can be provided by using an inexpensive hydrocollator pack (a wan-n, moistened, sand-filled canvas bag). The moist heat helps to prevent or reduce joint pain caused by motion. For chronic pain, deep heat, such as is provided by ultrasound, can be applied by a PT.
For acute, severe joint pain, ice is most helpful. Bed rest is occasionally prescribed for widespread severe pain. In such cases, it is especially important to properly position the patient in bed to avoid the development of contractures of the joints. One of the most common mistakes is to put pillows under the knees. This forces the knees into a bent position and, along with them, the hips. To imagine the difficulties this can cause, try walking with your hips and knees bent. Both musculoskeletal and CNS manifestations can lead to problems in performing the activities of daily living (ADL) such as dressing, bathing, toileting, etc. An occupational therapist (OT) can be most helpful in suggesting ways to solve these problems.
The OT can provide tools such as button hooks and long-handled sponges to make these activities easier, and can fit splints to allow painful hands and arms to rest while keeping them positioned properly. The OT can educate the patient in joint conservation techniques (ways to protect the joints yet still get the task done). The OT can also aid in the selection of devices to make living at home less difficult (tub benches, raised toilet seats, stair glides, etc.) and can help evaluate the home environment from the point of view of the patient's safety. If mobility has become a problem as a result of joint or CNS involvement, the PT teaches transfer techniques (bed to chair, sit to stand,etc.) and ambulation training, including going up and down stairs. The physical therapist also teaches patients to make proper use of assistive devices such as canes and crutches and helps the patient to adapt to leg braces and splints.
As a result of lupus vasculitis (inflammation of the blood vessels), patients may develop ulcerations of the skin. Special exercises can then be used to help improve circulation and whirlpool treatments can be given to clean the ulcers by removing dead tissue. Rarely, as a result of ulcerations of the feet or ankles, or as a result of blood clots in the major leg arteries, amputation of part of a leg may become necessary.
There has been dramatic improvement in prosthetic (artificial) feet in the last three to four years, allowing many more normal activities such as running and jumping, and materials are now available that result in a much lighter limb. In each state, there is a government office concerned with helping individuals with disabilities obtain training or education so that they can return to gainful employment if their disability prevents them from resuming their previous vocation. This office is usually known as the Office of Vocational Rehabilitation. Social workers can be most helpful in directing people with lupus-related disabilities to the appropriate office or case worker.
The social worker is familiar with the many resources that can help the person with lupus obtain a wide variety of services. Social workers are available in medical clinics, hospitals and social agencies and are members of all rehabilitation teams. If lupus patients have to be hospitalized in a rehabilitation center they will encounter physicians responsible for directing their rehabilitative care. Such physicians are specialists in Physical Medicine and Rehabilitation and are called "physiatrists". In addition to the PT, the OT and the social worker, the rehabilitation team usually includes a number of other professionals. These are a rehabilitation nurse (who has had special training in working with patients disabled by different diseases) a psychiatrist and/or psychologist (to help with the emotional problems that may be present), a recreational therapist (to get patients involved in activities that they may have enjoyed previously or to introduce new interesting activities that make them aware that life can still be enjoyed) and a speech therapist (to help in problems with language or with swallowing difficulties). As well as working with the patient, all members of the rehabilitation team work with the patient's family and friends so that they know what to do and what not to do, when the patient goes home. In summary, the primary purpose of any rehabilitation program is to improve the functional independence of the individual.
Most of the patients requiring a rehabilitation program can be treated in an out-patient setting or at home. Only a small minority will require an intensive in-patient program. While no patients are cured, almost all can be helped.
=========================================================== This information is for"informational purposes" and is not meant to be used for medical diagnosis. Alwaysconsult your physician on matters such as this.
Bertram Greenspun, DO
Rehabilitation medicine is the field of medicine that concerns itself not only with how the disease process has affected the individual but also with how the disease has changed the person's ability to maintain the lifestyle and roles in life which they had before the disease started.
The rehabilitation team concentrates on how to improve the person's functioning and independence to the highest possible level. Systemic Lupus Erythematosus (SLE) at times presents problems that can be helped by an individualized rehabilitation program. Among the body systems that can be affected by lupus (and may be helped by rehabilitation) are the central nervous system (CNS), the musculoskeletal system (muscles, bones and joints), and the vascular system (blood vessels).
The CNS can be involved in a variety of ways. Patients who develop stroke-like syndromes or problems of the spinal cord usually require an in-patient program in a comprehensive rehabilitation center. These problems often result in weakness of one or both arms or legs. Weakness can be so profound that it may result in paralysis. Under such circumstances, passive range of motion (ROM) exercises should be started by the physical therapist (PT). Family and friends may also be instructed by the physical therapist to perform ROM exercises for the patient. These exercises are done without the patient having to do any of the work. Since some degree of strength almost always returns when the stroke or the spinal cord damage resolves, it is vital not to allow contractures to develop.
Contractures happen when the joints are allowed to stiffen to the point where they will be unable to bend. Should this occur, even if muscle power returns it will do no good unless the stiffness is reduced or eliminated. This can take a very long time and even require surgery. It is much wiser and easier to prevent contractures in the first place than it is to correct them after the fact. As the patient improves, he/she can move the joint along with the physical therapist. Then the exercise is known as active-assistive ROM. When the patient can move the joint through the entire ROM, he/she no longer needs the assistance of another person and is instructed on how to do regular, active ROM exercises independently.
In addition to ROM exercises, strengthening exercises can be started under proper supervision as soon as is medically possible. The strengthening program should begin at a low intensity that is well within the capability of the patient. It is increased only gradually and slowly. The patient must learn to "listen" to his/her body. This means that if muscle pain or fatigue lasts for more than an hour to an hour and a half after a session, the level of exercise intensity should be temporarily reduced. if the discomfort or fatigue continues after each session the physician who prescribed the program should be notified.
Too much exercise, done too quickly, can easily make the situation worse. If the weakness is associated with acute inflammatory joint pain, isometric exercises can be helpful. Isometric exercises involve contracting the muscles without moving the involved joint. In this way strength can be maintained without triggering the intense pain associated with moving an acutely inflamed joint. Since isometric exercises can cause the blood pressure to rise they should be performed cautiously and without holding the breath, especially in the individual with hypertension. Almost all patients with lupus will, at some point in the disease process, have joint pain. There is often associated morning stiffness and joint swelling. Tenderness and painful movement are common. Deformities may result. Most of these problems can be dealt with on an out-patient basis.
An early morning shower will frequently shorten the duration of morning stiffness. Moist heat is quite helpful for most joint problems and is used prior to ROM exercises. This can be provided by using an inexpensive hydrocollator pack (a wan-n, moistened, sand-filled canvas bag). The moist heat helps to prevent or reduce joint pain caused by motion. For chronic pain, deep heat, such as is provided by ultrasound, can be applied by a PT.
For acute, severe joint pain, ice is most helpful. Bed rest is occasionally prescribed for widespread severe pain. In such cases, it is especially important to properly position the patient in bed to avoid the development of contractures of the joints. One of the most common mistakes is to put pillows under the knees. This forces the knees into a bent position and, along with them, the hips. To imagine the difficulties this can cause, try walking with your hips and knees bent. Both musculoskeletal and CNS manifestations can lead to problems in performing the activities of daily living (ADL) such as dressing, bathing, toileting, etc. An occupational therapist (OT) can be most helpful in suggesting ways to solve these problems.
The OT can provide tools such as button hooks and long-handled sponges to make these activities easier, and can fit splints to allow painful hands and arms to rest while keeping them positioned properly. The OT can educate the patient in joint conservation techniques (ways to protect the joints yet still get the task done). The OT can also aid in the selection of devices to make living at home less difficult (tub benches, raised toilet seats, stair glides, etc.) and can help evaluate the home environment from the point of view of the patient's safety. If mobility has become a problem as a result of joint or CNS involvement, the PT teaches transfer techniques (bed to chair, sit to stand,etc.) and ambulation training, including going up and down stairs. The physical therapist also teaches patients to make proper use of assistive devices such as canes and crutches and helps the patient to adapt to leg braces and splints.
As a result of lupus vasculitis (inflammation of the blood vessels), patients may develop ulcerations of the skin. Special exercises can then be used to help improve circulation and whirlpool treatments can be given to clean the ulcers by removing dead tissue. Rarely, as a result of ulcerations of the feet or ankles, or as a result of blood clots in the major leg arteries, amputation of part of a leg may become necessary.
There has been dramatic improvement in prosthetic (artificial) feet in the last three to four years, allowing many more normal activities such as running and jumping, and materials are now available that result in a much lighter limb. In each state, there is a government office concerned with helping individuals with disabilities obtain training or education so that they can return to gainful employment if their disability prevents them from resuming their previous vocation. This office is usually known as the Office of Vocational Rehabilitation. Social workers can be most helpful in directing people with lupus-related disabilities to the appropriate office or case worker.
The social worker is familiar with the many resources that can help the person with lupus obtain a wide variety of services. Social workers are available in medical clinics, hospitals and social agencies and are members of all rehabilitation teams. If lupus patients have to be hospitalized in a rehabilitation center they will encounter physicians responsible for directing their rehabilitative care. Such physicians are specialists in Physical Medicine and Rehabilitation and are called "physiatrists". In addition to the PT, the OT and the social worker, the rehabilitation team usually includes a number of other professionals. These are a rehabilitation nurse (who has had special training in working with patients disabled by different diseases) a psychiatrist and/or psychologist (to help with the emotional problems that may be present), a recreational therapist (to get patients involved in activities that they may have enjoyed previously or to introduce new interesting activities that make them aware that life can still be enjoyed) and a speech therapist (to help in problems with language or with swallowing difficulties). As well as working with the patient, all members of the rehabilitation team work with the patient's family and friends so that they know what to do and what not to do, when the patient goes home. In summary, the primary purpose of any rehabilitation program is to improve the functional independence of the individual.
Most of the patients requiring a rehabilitation program can be treated in an out-patient setting or at home. Only a small minority will require an intensive in-patient program. While no patients are cured, almost all can be helped.
=========================================================== This information is for"informational purposes" and is not meant to be used for medical diagnosis. Alwaysconsult your physician on matters such as this.
Lupus in Childhood
Lupus in Childhood
Phyllis Slutsky, MEd, RNBarbara E. Ostrov, MD
About 20-25% of all lupus occurs in children. While the exact number of cases is not known, it is not a rare disorder. Girls have lupus about three to seven times more often than boys. Research is being done to study many possible causes of lupus.
Several studies have shown that there is a tendency for lupus to run in families. It is known that there are certain inherited factors called HLA types which make a person more likely to develop lupus. (See Chapter 4.) There is also an inherited lack of immune system proteins (serum complements or immunoglobulins) which increase the risk for developing lupus in some people.
When a susceptible person is exposed to a "trigger" such as a virus or other environmental factor, lupus can develop. A particular kind of lupus may develop in infants of mothers who have SLE. This is called "neonatal lupus" and is associated with an anti-Ro antibody in the mother. An infant with neonatal lupus may have a rash on the face, scalp, or chest. Very rarely, these infants may also be born with an abnormally slow heart rhythm (congenital heart block).
During the first six months to one year, the rash disappears but the slow heart rhythm can be serious and may require the placement of a pacemaker. Systemic lupus erythematosus in childhood can range from mild to severe and affects each individual differently. The usual course of lupus includes periods of "flare-ups" (when symptoms are active) and remissions (when symptoms go away).
Stressors such as infections (which may be more frequent in childhood) and hormonal changes may lead to a flare of symptoms. This may explain why lupus is more common in girls after puberty and may flare up during menstruation and pregnancy. In children, as in adults, sun exposure can bring on symptoms of lupus. Serious complications of lupus are most common when organs such as kidneys, heart or the central nervous system are involved.
The most common time to find out what body systems are involved is at the time that lupus is first diagnosed. As time goes on, the likelihood decreases that new body systems will become involved. Diagnosing lupus involves a thorough physical examination and often many laboratory tests.
There are guidelines which have been developed by the American College of Rheumatology which are used to diagnose lupus. (See Table 1) Four of their eleven selected symptoms and/or laboratory tests (criteria) must be present to make a definite diagnosis. Symptoms of lupus may start in a variety of ways.
A child may be very ill or have only mild symptoms. The most common symptoms in children are rash, fever, fatigue, and joint pain and swelling. Decreased appetite and weight loss are particularly common in children during the active phase of the disease. Inflammation of the kidneys (nephritis), inflammation of the heart covering (pericarditis), enlargement of the liver and spleen, and a low blood count (cytopenia) may be more frequent in children than in adults. Many children have rashes that can appear on the face or anywhere on the body. Ulcers of the mouth and nose are common in children with lupus. Hair loss (alopecia) may range from gradual thinning and change in texture, to loss of large amounts of hair when combing.
Central nervous system symptoms (affecting the brain and spinal column) can occur and may cause headaches, seizures, or a change in memory or thinking ability. Visual changes can also occur, making it important to have frequent checks by an eye doctor. Changes in mood or behavior can occur in children with lupus. These can be an understandable reaction to having an illness. Feelings of sadness or irritability do not necessarily mean there is central nervous system involvement. However, it is important to keep a careful watch on a child's behavior to decide if these things are caused by the lupus or are due to the child's reaction to the illness.
Diagnostic tests such as spinal taps, EEGS, and brain x-rays and scans can be helpful in deciding what is caused by lupus and what is due to psychosocial causes. The kidneys can be affected by lupus. A urinalysis may reveal problems which can then be further investigated with other tests. It is common to collect urine for 24 hours to do further studies. A kidney biopsy is sometimes necessary to determine how seriously the kidney is affected.
This test is used as a guideline for treatment and for monitoring changes over time. The treatment of lupus depends on what part of the body is affected. Being careful about sun exposure is important for all children. Planning the child's schedule to avoid exposure during the peak time of 12:00-3:00 P.M. each day is recommended. Children should be included in working out the schedule so that they understand the importance of any restrictions and will be more willing to cooperate. Another important aspect of treatment is to be alert for any signs of a developing infection.
Families should be aware that fever and increased tiredness or shortness of breath could be a sign of either a "flare-up" of lupus or possibly an infection. The doctor may need to evaluate the child when fever develops. There are a variety of medications used to treat lupus. Many children are treated with a non-steroidal anti-inflammatory drug (NSAID) which helps reduce inflammation and joint pain. Antimalarial drugs such as Plaquenil are sometimes used to treat the skin rashes of lupus. Steroids are sometimes necessary to treat lupus. The goal when using steroids is to use the lowest possible amount needed.
As symptoms improve, smaller and smaller doses are gradually given until the medication can be stopped altogether. When high doses of steroids are used over a long period of time, side effects can occur such as eye problems, and a decrease in the growth of the child. Giving steroids every other day helps to lower the chances of harmful side effects. Some other drugs used mainly when kidneys are affected by lupus are Cytoxan and Imuran. These work to control the reaction of a child's immune system so that it causes less harmful effects on the body. Recent studies have shown that these drugs can be used safely in children and can control the more serious effects of lupus on internal organs. A chronic illness like lupus will of course have a large effect on a child's life.
Often a child misses school during "flares". It is important to make contact with the school as soon as possible and stay in contact during the absent period. A school counselor or nurse can make arrangements to help the child. Some children may need home or hospital tutoring while others may be able to do make-up work on their own. This depends on how sick they are and how long they will be out of school. Because lupus symptoms come and go, arrangements may need to be made before a child is sick so that tutoring can start right away if needed. Many rheumatology centers that treat lupus have a health care team that includes a social worker and a nurse who can help the family with getting services needed for their child. Often children with lupus (like those with other chronic conditions) should have an Individualized Education Plan (IEP) .
This is an evaluation of all of the child's educational, psychosocial and physical needs which is done by a "study team" in each school district. The IEP identifies what each child needs in order to function well at school. Every child in the USA is entitled to this plan due to a government law (PL#94 -142). Children with lupus are also entitled to vocational planning services in order to prepare for school or job training for their future employment.
Each state has an Office of Vocational Rehabilitation (OVR) which offers career counseling that takes into account a child's physical abilities. Any illness in a family member is likely to cause reactions in other members of the family. These can help or hurt successful coping. There is often an increased burden on the family when caring for a sick child. This can also mean lost work time for parents and added financial problems due to medical expenses. Studies have been done to measure the effects on a family of a child with chronic illness. The families that coped best were those that did not place their child in a sick role, and so did not limit the child's activities in daily life.
Families based around a good quality marriage, and with good support from family, friends and/or religious groups cope better with a chronic illness. Support groups for parents of children with lupus exist through the AJAO (American juvenile Arthritis Organization) which is a part of the Arthritis Foundation. The large number of teenage and young adult women with lupus raises some special concerns. A major problem for teenagers is the change in appearance, often on the face, caused by lupus.
The characteristic rash and puffiness of lupus, or the acne caused by steroids cause the child to wonder, "How do I look to the world?" It is important to discuss this as openly as possible with the teenager, as these issues are a major source of stress and worry. Concerns about sexuality and pregnancy are also often uppermost in teenagers' minds when they become ill. Standard oral contraceptives which contain the hormone estrogen may cause an increase in symptoms of lupus and therefore are not recommended.
Sometimes the mini-pill, which contains only the hormone progesterone, is safe in lupus patients. Diaphragms and condoms are fine, and all of these options should be reviewed and discussed. It is recommended that a woman not become pregnant during an active period of lupus. Lupus has not been found to affect the ability to get pregnant, but miscarriages are more common when the disease is active. It is very possible to become pregnant and have a healthy baby, but careful planning with the doctor for the best time to get pregnant is advised. There are many obstetricians available who specialize in "high risk" pregnancies. Because of the many issues and adjustments necessary for a teenager with lupus, individual counseling may be necessary and can be helpful to some adolescents.
Support groups are an ideal way for teens to get the peer support and acceptance they need. Teens who attend these groups report a decrease in feelings of isolation and are better able to accept their illness. There has been a remarkable improvement in the prognosis of children with lupus during the past thirty years. This has happened due to improvements in diagnosis and treatment. Most importantly, children and their families need to seek careful and competent medical treatment and to cultivate the support of their health care team, their school and their community.
=========================================================== This information is for"informational purposes" and is not meant to be used for medical diagnosis. Alwaysconsult your physician on matters such as this.
Phyllis Slutsky, MEd, RNBarbara E. Ostrov, MD
About 20-25% of all lupus occurs in children. While the exact number of cases is not known, it is not a rare disorder. Girls have lupus about three to seven times more often than boys. Research is being done to study many possible causes of lupus.
Several studies have shown that there is a tendency for lupus to run in families. It is known that there are certain inherited factors called HLA types which make a person more likely to develop lupus. (See Chapter 4.) There is also an inherited lack of immune system proteins (serum complements or immunoglobulins) which increase the risk for developing lupus in some people.
When a susceptible person is exposed to a "trigger" such as a virus or other environmental factor, lupus can develop. A particular kind of lupus may develop in infants of mothers who have SLE. This is called "neonatal lupus" and is associated with an anti-Ro antibody in the mother. An infant with neonatal lupus may have a rash on the face, scalp, or chest. Very rarely, these infants may also be born with an abnormally slow heart rhythm (congenital heart block).
During the first six months to one year, the rash disappears but the slow heart rhythm can be serious and may require the placement of a pacemaker. Systemic lupus erythematosus in childhood can range from mild to severe and affects each individual differently. The usual course of lupus includes periods of "flare-ups" (when symptoms are active) and remissions (when symptoms go away).
Stressors such as infections (which may be more frequent in childhood) and hormonal changes may lead to a flare of symptoms. This may explain why lupus is more common in girls after puberty and may flare up during menstruation and pregnancy. In children, as in adults, sun exposure can bring on symptoms of lupus. Serious complications of lupus are most common when organs such as kidneys, heart or the central nervous system are involved.
The most common time to find out what body systems are involved is at the time that lupus is first diagnosed. As time goes on, the likelihood decreases that new body systems will become involved. Diagnosing lupus involves a thorough physical examination and often many laboratory tests.
There are guidelines which have been developed by the American College of Rheumatology which are used to diagnose lupus. (See Table 1) Four of their eleven selected symptoms and/or laboratory tests (criteria) must be present to make a definite diagnosis. Symptoms of lupus may start in a variety of ways.
A child may be very ill or have only mild symptoms. The most common symptoms in children are rash, fever, fatigue, and joint pain and swelling. Decreased appetite and weight loss are particularly common in children during the active phase of the disease. Inflammation of the kidneys (nephritis), inflammation of the heart covering (pericarditis), enlargement of the liver and spleen, and a low blood count (cytopenia) may be more frequent in children than in adults. Many children have rashes that can appear on the face or anywhere on the body. Ulcers of the mouth and nose are common in children with lupus. Hair loss (alopecia) may range from gradual thinning and change in texture, to loss of large amounts of hair when combing.
Central nervous system symptoms (affecting the brain and spinal column) can occur and may cause headaches, seizures, or a change in memory or thinking ability. Visual changes can also occur, making it important to have frequent checks by an eye doctor. Changes in mood or behavior can occur in children with lupus. These can be an understandable reaction to having an illness. Feelings of sadness or irritability do not necessarily mean there is central nervous system involvement. However, it is important to keep a careful watch on a child's behavior to decide if these things are caused by the lupus or are due to the child's reaction to the illness.
Diagnostic tests such as spinal taps, EEGS, and brain x-rays and scans can be helpful in deciding what is caused by lupus and what is due to psychosocial causes. The kidneys can be affected by lupus. A urinalysis may reveal problems which can then be further investigated with other tests. It is common to collect urine for 24 hours to do further studies. A kidney biopsy is sometimes necessary to determine how seriously the kidney is affected.
This test is used as a guideline for treatment and for monitoring changes over time. The treatment of lupus depends on what part of the body is affected. Being careful about sun exposure is important for all children. Planning the child's schedule to avoid exposure during the peak time of 12:00-3:00 P.M. each day is recommended. Children should be included in working out the schedule so that they understand the importance of any restrictions and will be more willing to cooperate. Another important aspect of treatment is to be alert for any signs of a developing infection.
Families should be aware that fever and increased tiredness or shortness of breath could be a sign of either a "flare-up" of lupus or possibly an infection. The doctor may need to evaluate the child when fever develops. There are a variety of medications used to treat lupus. Many children are treated with a non-steroidal anti-inflammatory drug (NSAID) which helps reduce inflammation and joint pain. Antimalarial drugs such as Plaquenil are sometimes used to treat the skin rashes of lupus. Steroids are sometimes necessary to treat lupus. The goal when using steroids is to use the lowest possible amount needed.
As symptoms improve, smaller and smaller doses are gradually given until the medication can be stopped altogether. When high doses of steroids are used over a long period of time, side effects can occur such as eye problems, and a decrease in the growth of the child. Giving steroids every other day helps to lower the chances of harmful side effects. Some other drugs used mainly when kidneys are affected by lupus are Cytoxan and Imuran. These work to control the reaction of a child's immune system so that it causes less harmful effects on the body. Recent studies have shown that these drugs can be used safely in children and can control the more serious effects of lupus on internal organs. A chronic illness like lupus will of course have a large effect on a child's life.
Often a child misses school during "flares". It is important to make contact with the school as soon as possible and stay in contact during the absent period. A school counselor or nurse can make arrangements to help the child. Some children may need home or hospital tutoring while others may be able to do make-up work on their own. This depends on how sick they are and how long they will be out of school. Because lupus symptoms come and go, arrangements may need to be made before a child is sick so that tutoring can start right away if needed. Many rheumatology centers that treat lupus have a health care team that includes a social worker and a nurse who can help the family with getting services needed for their child. Often children with lupus (like those with other chronic conditions) should have an Individualized Education Plan (IEP) .
This is an evaluation of all of the child's educational, psychosocial and physical needs which is done by a "study team" in each school district. The IEP identifies what each child needs in order to function well at school. Every child in the USA is entitled to this plan due to a government law (PL#94 -142). Children with lupus are also entitled to vocational planning services in order to prepare for school or job training for their future employment.
Each state has an Office of Vocational Rehabilitation (OVR) which offers career counseling that takes into account a child's physical abilities. Any illness in a family member is likely to cause reactions in other members of the family. These can help or hurt successful coping. There is often an increased burden on the family when caring for a sick child. This can also mean lost work time for parents and added financial problems due to medical expenses. Studies have been done to measure the effects on a family of a child with chronic illness. The families that coped best were those that did not place their child in a sick role, and so did not limit the child's activities in daily life.
Families based around a good quality marriage, and with good support from family, friends and/or religious groups cope better with a chronic illness. Support groups for parents of children with lupus exist through the AJAO (American juvenile Arthritis Organization) which is a part of the Arthritis Foundation. The large number of teenage and young adult women with lupus raises some special concerns. A major problem for teenagers is the change in appearance, often on the face, caused by lupus.
The characteristic rash and puffiness of lupus, or the acne caused by steroids cause the child to wonder, "How do I look to the world?" It is important to discuss this as openly as possible with the teenager, as these issues are a major source of stress and worry. Concerns about sexuality and pregnancy are also often uppermost in teenagers' minds when they become ill. Standard oral contraceptives which contain the hormone estrogen may cause an increase in symptoms of lupus and therefore are not recommended.
Sometimes the mini-pill, which contains only the hormone progesterone, is safe in lupus patients. Diaphragms and condoms are fine, and all of these options should be reviewed and discussed. It is recommended that a woman not become pregnant during an active period of lupus. Lupus has not been found to affect the ability to get pregnant, but miscarriages are more common when the disease is active. It is very possible to become pregnant and have a healthy baby, but careful planning with the doctor for the best time to get pregnant is advised. There are many obstetricians available who specialize in "high risk" pregnancies. Because of the many issues and adjustments necessary for a teenager with lupus, individual counseling may be necessary and can be helpful to some adolescents.
Support groups are an ideal way for teens to get the peer support and acceptance they need. Teens who attend these groups report a decrease in feelings of isolation and are better able to accept their illness. There has been a remarkable improvement in the prognosis of children with lupus during the past thirty years. This has happened due to improvements in diagnosis and treatment. Most importantly, children and their families need to seek careful and competent medical treatment and to cultivate the support of their health care team, their school and their community.
=========================================================== This information is for"informational purposes" and is not meant to be used for medical diagnosis. Alwaysconsult your physician on matters such as this.
Friday, June 09, 2006
Dental Problems and the Lupus Patient
Dental Problems and the Lupus Patient
Jay A. Denbo, DDS
COMMUNICATION, SELF-EXAMINATION, and PREVENTION are the keys to controlling dental problems for the lupus patient. Lupus patients have specific dental problems because of the disease process and the medications needed to control lupus symptoms.
Communication
Communication between patient, physician, and dentist is essential. Lupus patients should visit the dentist at regular intervals, usually every two to six months. The patients must tell the dentist about their current and past medical history. The dentist should also be supplied with a complete list of medications the patient is taking. The dentist also needs to know the dosage and any side-effects of all the medications. The patient's physician should in turn be made aware of any dental problems and the proposed treatment.
If the lupus patient needs dental surgery, open communication between physician and dentist is especially important. The surgical procedure may well require a change in the dosage of any steroid medications and may require prophylactic (preventive) antibiotics if the patient has certain kinds of lupus heart disease (the kind that affects the valves of the heart). In such cases it may be necessary for the dentist to consult the patient's physician to decide on the best management. Post-operative examinations for the lupus patient must be more frequent and extend over a longer period of time than would be the case for the non-lupus dental patient.
Self-examination
Self-examination of the mouth should regularly be performed by lupus patients. Periodontal disease, which causes the greatest amount of tooth loss in adults and affects almost everyone, usually does not cause pain and may not give warning until significant tissue breakdown has occurred. However, there are signs and symptoms of periodontal disease which the patient can detect with self-examination. There are also certain procedures the patient can perform to prevent and help control periodontal disease. Periodontal disease is a group of inflammatory conditions which affect the gums (gingiva) and supporting bone around each tooth. The most common periodontal disorders are gingivitis (inflammation of the gingiva or gums) and periodontitis (inflammation affecting the bone under the gingiva). The presence of any of the following signs and symptoms may indicate the presence of gingivitis.
Jay A. Denbo, DDS
COMMUNICATION, SELF-EXAMINATION, and PREVENTION are the keys to controlling dental problems for the lupus patient. Lupus patients have specific dental problems because of the disease process and the medications needed to control lupus symptoms.
Communication
Communication between patient, physician, and dentist is essential. Lupus patients should visit the dentist at regular intervals, usually every two to six months. The patients must tell the dentist about their current and past medical history. The dentist should also be supplied with a complete list of medications the patient is taking. The dentist also needs to know the dosage and any side-effects of all the medications. The patient's physician should in turn be made aware of any dental problems and the proposed treatment.
If the lupus patient needs dental surgery, open communication between physician and dentist is especially important. The surgical procedure may well require a change in the dosage of any steroid medications and may require prophylactic (preventive) antibiotics if the patient has certain kinds of lupus heart disease (the kind that affects the valves of the heart). In such cases it may be necessary for the dentist to consult the patient's physician to decide on the best management. Post-operative examinations for the lupus patient must be more frequent and extend over a longer period of time than would be the case for the non-lupus dental patient.
Self-examination
Self-examination of the mouth should regularly be performed by lupus patients. Periodontal disease, which causes the greatest amount of tooth loss in adults and affects almost everyone, usually does not cause pain and may not give warning until significant tissue breakdown has occurred. However, there are signs and symptoms of periodontal disease which the patient can detect with self-examination. There are also certain procedures the patient can perform to prevent and help control periodontal disease. Periodontal disease is a group of inflammatory conditions which affect the gums (gingiva) and supporting bone around each tooth. The most common periodontal disorders are gingivitis (inflammation of the gingiva or gums) and periodontitis (inflammation affecting the bone under the gingiva). The presence of any of the following signs and symptoms may indicate the presence of gingivitis.
- Bleeding gums: Gingiva which bleeds on eating or brushing teeth, or bleeds without any obvious cause.
- Swollen gums: Enlarged gingiva which is not bound tightly around each tooth.
- Red Gums: Gingiva which is red in color. Dark colored gingiva due to naturally occurring melanin (the substance which colors the gums) is not an indication of inflammation.
- Sensitive gums: Gingiva which is painful to brushing. Untreated gingivitis can progress into the tissues under the gingiva and cause the bone which supports the teeth to become weakened and resorb.
Periodontitis is a form of dental disease which is marked by bone loss and will eventually lead to loosening or loss of teeth. It is usually painless and may have the same signs and symptoms as gingivitis. If any signs or symptoms of gingivitis or periodontitis are noted, the tupus patient should tell his/her dentist. A dentist can accurately diagnose and treat periodontat disease.
Prevention
Prevention is the best treatment for dental disease. The only way to prevent periodontal disease is by effective tooth brushing techniques. Putting toothpaste on a toothbrush and swishing the brush around your mouth for 15 to 30 seconds once or twice a day is not effective tooth brushing. Tooth brushing must clean all five surfaces of each tooth. The most critical area of the tooth to clean is immediately adjacent or next to the gingiva or gums. Therefore, the junction between the gum and the tooth should be very carefully brushed. The area between the teeth cannot be cleaned by a toothbrush. Dental floss, dental tape, rubber tips, special (interproximal) brushes and Stimulators are used to remove the dental plaque which is deposited between adjacent teeth. It should take approximately three minutes to brush your teeth thoroughly. Plaque which causes dental caries (cavities) and periodontal disease accumulates on all tooth surfaces and must be removed daily. Any areas of gingival sensitivity, tooth sensitivity, or gingival bleeding require effective brushing or the sensitivity and bleeding will get worse. If it hurts or if it bleeds, it should be brushed harder.
Lupus patients should check the inside of their mouths regularly for any red or irritated areas. If such areas are noticed, these should be brought to the attention of the physician and dentist. Approximately 25% of lupus patients have these kinds of oral problems, which are usually accompanied by a skin irritation or facial rash. Oral problems in lupus are also found on the lips and on the tissue inside the cheeks. Patients with active oral problems should not use denture powder or denture paste to hold in removable dentures. if toothpaste irritates the mouth, baking soda and water should be substituted for it.
=========================================================== This information is for "informational purposes" and is not meant to be used for medical diagnosis. Always consult your physician on matters such as this.
With COMMUNICATION, SELF-EXAMINATION, and PREVENTION, the lupus patient can keep dental problems under control.
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