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
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"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 Lehman’s experience and compassion are evident on every page of this
book, and they help guide the reader—child, 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