Systemic Lupus Erythematosus

This page is provided by Thomas J. A. Lehman MD

Delivering the best care - with great care

 Dr. Lehman is the author of many textbook chapters and articles on the care of children and young adults with SLE.  He practices in New York City.  Click here for more information about Dr. Lehman or the Hospital for Special Surgery.

 Unsure if you are getting the best care?   Click here for information on a book I have written about childhood rheumatic disease which discusses lupus in much more detail

 

What is systemic lupus erythematosus?

Systemic lupus erythematosus (SLE) is a challenging 'autoimmune' whose wide variety of manifestations makes it a necessary part of the differential diagnosis for children and adolescents with many different presenting complaints. Nonspecific complaints of fatigue and malaise are the most common initial symptoms of SLE in children and adolescents. The typical 'butterfly' rash is present in less than one-third of affected children. Many pediatricians dismiss SLE from their differential diagnosis as 'uncommon in childhood', but positive tests for antinuclear antibodies (ANA) and other findings which warrant inclusion of SLE in the differential diagnosis are in fact quite common. Further with increased recognition of 'milder' cases SLE is far more common than previously thought. Despite the popular misconception of ANA and antibodies directed against deoxyribonucleic acid (anti-DNA) do not mean that SLE is a disease in which the body is 'reacting against itself.' SLE is not truly an 'autoimmune' disease. The primary problem in SLE is persistent nonspecific activation of the immune system which results in widespread tissue deposition of immune complexes.  Thus most of the damage that occurs in SLE is ‘bystander’ damage.  As a result of the deposition of immune complexes there is inflammation that damages the tissues where ever the immune complexes have landed.  See my book for a much more extensive discussion than I can provide here.  The chapter on SLE is 40 pages long and there are additional chapters on medications, laboratory tests, and dealing with chronic disease.

The immune system is normally turned on in the setting of overwhelming infection and is 'shut off' when the infection resolves. In SLE the normal regulatory process fails, and immune system activation continues unchecked. The nature of the immune complexes which result and where they are deposited are determined by a combination of genetics and previous environmental history (i.e. everything the individual's immune system has previously been exposed to). Thus, no two patients will have an identical pattern of immune complex formation or clinical disease expression.

ANA and anti-DNA are important in diagnosing SLE because their presence in significant titer documents abnormal immune system activation. Most of the immune complexes which cause organ damage do not contain antibodies against 'self.' The damage results from organ specific reactions to the excessive deposition of immune complexes.

The most important thing to understand if you or someone you love has been diagnosed with SLE is that the course of the disease is highly varied.  You want to be in the hands of an excellent physician with extensive experience in dealing with SLE.  The approach to dealing with this disease has changed dramatically in the last decade.  Children with mild SLE which is easily controlled with a low dose of corticosteroids (e.g. less than 20 mg of prednisone per day) often do well on only this regimen.  However significant doses of prednisone continued for long periods of time are often associated with side affects which are always unpleasant and sometimes severe.  Prolonged use of even moderate doses of prednisone will cause changes in appearance, weakening of bones, increased risk of heart disease and many other problems.  It is important to avoid this if possible (not always possible).

The key to avoiding unacceptable long term side effects of prednisone is the early recognition of children who have SLE that is not going to be controlled with a low dose of corticosteroids.  These children should be begun on stronger immunosuppressive agents as soon as the severity of their disease is recognized.  If you wait until the bad effects of the corticosteroids are well established to decide to do something different you are already way behind.  It is far better to see the problem coming and prevent it from happening.  Repairing the damage done by steroids is not always possible.  The most striking observation of the past 20 years is that the children who had severe disease when they first came to me were doing much better after ten years than the children who came to me with moderate disease.  Why??  Because I immediately gave the children with severe disease aggressive therapy.  In the end they got much less prednisone than the child who had moderate disease.  Those children were constantly going up and down on their steroids because their disease was never fully controlled.  Once this was recognized and we gave these children more aggressive therapy so that this stopped happening, the children did very well.

Many parents are afraid of the ‘risks’ of more aggressive therapy, but in truth it is the consequences of long term prednisone in too high a dosage that do the most damage to children with SLE.  Small doses are necessary and should be accepted.  But the rare risks of more aggressive therapy are rare.  The long term consequences of prednisone in moderate to high doses affect everyone who takes these doses of the drug for an extended period.  Early aggressive knowledgeable care by an experienced physician is the key to getting the best results for your child.  You need a physician who is comfortable dealing with SLE and one who is comfortable dealing with adolescents.  Never forget that if you have a child who is refusing to cooperate because the prednisone side effects are altering their appearance you are at great risk.  Many of these children stop making the medication and often even their parents don’t immediately know.  When that happens the disease may flare uncontrollably or they may develop an infection with disastrous results.

Isn't SLE a very rare disease?

Systemic lupus erythematosus remains uncommon. Old data estimate the prevalence of SLE in childhood to range from 0/100,000 for white females less than fifteen years of age to 31/100,000 for Oriental females ten to twenty years of age. Over the age of ten years, SLE becomes much more common among girls because of the 'synergistic' effect of female sex hormones. However a significant number of boys are included in every series. Race is also a significant risk factor. The incidence of SLE in the 10 - 20 year old age group varies from 4.4/100,000 white females to 31/100,000 Oriental females with blacks 19.86/100,000 and Hispanics 13/100,000 in between.

When do I need to think of SLE?

The most common manifestations of SLE in childhood are nonspecific and consistent with diverse diagnoses. The differential diagnosis of the child with progressive fatigue and malaise includes multiple chronic illness ranging from infections (e.g. tuberculosis) to malignancies. A variety of findings in the initial work-up of a child with chronic illness should alert the physician to the possibility of SLE. ANA testing is a useful screen for SLE. However, this test has a very high sensitivity (the vast majority of children with SLE are ANA positive), but it has a very poor specificity (the vast majority of ANA positive children do not have SLE)  Click here for more information about ANA testing. Widespread arthritis of the small joints of the hands and feet on careful examination is a useful finding that suggests SLE. Small effusions of the knee joints are also commonly present with active disease. A positive test for ANA and arthritis of the small joints may also be present in children with other illness including Lyme disease and juvenile rheumatoid arthritis.

Routine laboratory evaluation including a complete blood count, erythrocyte sedimentation rate, and chemistry panel will often suggest SLE in affected children. There are elevated levels of most acute phase reactants. This results in a reversed albumin globulin ratio, elevated white blood cell count, elevated platelet count, and elevated erythrocyte sedimentation rate. A decreased white blood cell count or platelet count suggests either decreased production or increased peripheral destruction. Decreased production may occur with marrow infiltrative processes such as leukemia, while increased destruction may occur with SLE.

Once the diagnosis of SLE has been suspected, it may be confirmed using established criteria. The American College of Rheumatology has published eleven criteria for the diagnosis of definite SLE. Tests designed to increase the specificity of ANA testing such as anti-DNA testing and tests for antibodies to the extractable nuclear antigens (SSA/Ro, SSB/La, Sm, and RNP) are also available. Antibodies to SSA/Ro and RNP are found in a wide variety of 'normal' individuals in isolation they are of uncertain significance. They do not indicate the individual will develop SLE. Antibodies to RNP are similarly nonspecific. Antibodies to Sm are more closely linked to SLE. They are found in roughly two-thirds of patients with active SLE, but relatively few 'normals.'

What's next?

Once the diagnosis of SLE has been established the most important step is to determine the appropriate therapy. Children with active SLE may need to immediately begin corticosteroid therapy. However, many patients with mild SLE can be successfully without steroids. In contrast, some children present with severe life threatening disease manifestations. These children may require immediate therapy with high dose corticosteroids and/or immunosuppressive agents such as cyclophosphamide.  The most important thing in treating children and adults with SLE is to find a doctor with excellent experience in the area and make sure you are getting the best possible care.  Treating SLE is like dealing with fires.  As soon as it is recognized it must be dealt with quickly and effectively.  Delays in beginning appropriate treatment are like delays in calling the fire department.  The longer the delay the more damage has been allowed to accumulate. See my book for a much more extensive discussion than I can provide here.  The chapter on SLE is 40 pages long and there are additional chapters on medications, laboratory tests, and dealing with chronic disease.

Why is renal involvement so important?

SLE is an unpredictable disease and may be fatal with or without renal involvement. Renal involvement is the most prevalent life threatening complication of SLE. For most children with significant renal involvement this takes the form of hematuria or proteinuria.  However, in the hands of experienced specialists children with SLE most often do very well.  It is important to find the best possible care for your family.

There are many extra-renal complications of childhood SLE. Neurologic involvement is the most difficult. When a child or adolescent with SLE becomes noncompliant and difficult, the family and physicians often ascribe the behavior to the psychological problems associated with adolescence and chronic illness or chronic corticosteroid therapy. Some times it is caused by the lupus itself.

CONCLUSIONS

SLE is a challenging disease with varied manifestations resulting from widespread immune complex deposition. It may present as an acute illness with fever, rash, and hematuria, or as chronic fatigue and malaise which might be mistaken for 'school phobia.' Although SLE remains an infrequent disease in general pediatric practice, mild cases are more frequent than previously recognized. Our understanding of the role of genetics and environmental agents in the pathogenesis of SLE has improved over the past ten years. In addition, the past ten years have seen refinements in the use of immunosuppressive regimens such as cyclophosphamide which have lead to both improved quality of life and improved survival for children with active SLE unresponsive to corticosteroids. While long term concerns regarding the safety and efficacy of immunosuppressive drug regimens persist, the future is increasingly bright for children with SLE who receive appropriate care in a timely fashion.

We have extensive experience in caring for children and young adults with SLE at the Hospital for Special Surgery.  Dr. Lehman has published numerous papers on the best treatments for children with SLE and authored the chapters on SLE in children and adolescents in many of the leading textbooks.  For more information about Dr. Lehman or the Hospital for Special Surgery click on these links.   Thomas J. A. Lehman or Hospital for Special Surgery

 

For a more detailed discussion of SLE in childhood, the diagnosis, the lab tests, the medicine and how to cope with it see….

My book –click here to order at a discount from Amazon.com!!

 30% off today at Amazon.com

 

"This comprehensive guidebook is a must-read for pediatricians and health care professionals who treat children and adolescents. For parents of children who have already been diagnosed with rheumatic disease, as well as children who have baffling, undiagnosed symptoms, this book will be a valuable resource."Enid Engelhard, CSW, Director of Social Services, S.L.E. Foundation, Inc.

    Dr. Tom Lehmans experience and compassion are evident on every page of this book, and they help guide the readerchild, parent, and healthcare professional alike through the world of childhood arthritis.  This book is an absolute gem written with a single goal in mind:  improve the lives of kids with arthritis. -- Jack Klippel, M.D. President and CEO of the Arthritis Foundation

 

     Dr. Lehman has given parents and families of children with arthritis the first book that speaks to the parent and child as equals.  His book explains the illnesses, the medications, the lab tests, and the disease course in simple, understandable lay language and givens them valuable insight into how a pediatric rheumatologist thinks.  Bravo!-- Charles Spencer, M.D., Professor of Clinical Pediatrics, University of Chicago, La Rabida

 

It’s not just growing pains.
A guide to childhood muscle, bone, and joint pain,
rheumatic diseases and the latest treatments

 

Click here to see the table of contents

 

It has always been a frustration trying to answer the many questions I have received from people over the web.  I can’t take the time and give them the detail I would like to.  I have to take care of my patients.  This book is a distillation of my experience answering questions for parents and health professionals over 25 years of practice.  If you want to know about the diseases, the tests, the medications, or how to be sure you are getting the best care– If you are the family member of a child with joint pains, this book will give you the answers.  If you are a general physician, a pediatrician, or a nurse who cares for children with these diseases it will answer many of the questions families ask you, and you can recommend it to them.  It will also answer many of your questions about what shots to give, what precautions to take, and the other questions families, pediatricians, and other health care providers have asked me over the years.

Dr. Lehman is the author of many textbook chapters and articles on the care of children and young adults with SLE.  He practices in New York City.  Click here for more information about Dr. Lehman or the Hospital for Special Surgery.

Click here for The Lupus Foundation web page

The Arthritis Foundation also works with children with lupus.

Last Updated 8/15/2004

Click for BOOKS dealing with SLE

This site provided by Thomas J. A. Lehman MD
Chief, Division of Pediatric Rheumatology
The Hospital for Special Surgery
535 E 70 St,
New York, NY 10021
212-606-1151, fax 212-606-1938, e-mail goldscout@aol.com