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SYSTEMIC LUPUS ERYTHEMATOSUS |
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Some call it the “great imitator,” because its symptoms vary so widely
that it is often mistaken for other disorders. Systemic lupus erythematosus,
often referred to simply as lupus, is a serious disease affecting the
joints, kidneys, and skin. Although lupus can be a fatal disease, there
is much reason for hope. Improvements in therapy have significantly increased
life expectancy and quality of life.
Fast Facts
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Lupus affects 10 times as many women as men.
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Treatment depends on the type and severity of symptoms you experience.
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Given its complex nature, lupus requires treatment by a rheumatologist and
your own active participation in maintaining your health.
What lupus is
Systemic lupus erythematosus (also called SLE or lupus) is a chronic
inflammatory disease that can affect the skin, joints, kidneys, lungs,
nervous system, and/or other organs of the body. The most common symptoms
include skin rashes and arthritis, often accompanied by fatigue and fever.
The clinical course of SLE varies from mild to severe, and typically involves
alternating periods of remission and relapse.
What causes lupus
SLE is an autoimmune disorder which develops when the body's own immune
system, which normally protects against cancers and invading infections,
begins to attack the patient's own tissues (known medically as a “loss
of self-tolerance”). This occurs first through the production of “auto-antibodies” (antibodies
are immune system cells that attack foreign microbes; auto-antibodies
attack a person's own cells). As the attack continues, other immune system
cells join the fight. This leads to inflammation, blood vessel abnormalities
(vasculitis) and deposition of immune system cells in organs which causes tissue
damage.
It is not known why this inflammatory reaction begins, but it probably
occurs because of some combination of inborn or hereditary predispositions
and environmental factors (such as viruses, the ultraviolet rays in sunlight,
Silica dust, and allergies to medications). Recent research suggests that
people affected by lupus may have a defect in the normal biological process
of clearing old and damaged cells from the body, which then causes an
abnormal stimulation of the immune system.
Who gets lupus
SLE occurs 10 times more often in women than in men. The disorder typically
develops in people in their twenties and thirties. SLE is more common
in certain ethnic groups, particularly in blacks and Asians, who also
tend to be more severely affected.
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| This picture illustrates a malar rash which is a red rash
on the
cheeks and nose that is often brought out by exposure to the
sun. |
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How lupus is diagnosed
Diagnosis of SLE may be suspected on the basis of symptoms, but is confirmed
by a series of blood tests. Of particular interest is the antinuclear
antibody (ANA), which is present in virtually all the patients with lupus.
Other tests such as the anti-double strand DNA (dsDNA) and anti-smith
antibodies (Sm) are more specific and are used to confirm the diagnosis
of lupus. The levels of certain complement proteins (a part of the immune
system) are also used to help diagnose and monitor the disease.
If anti-phospholipid antibodies are present, this not only helps to establish
a diagnosis of lupus, but also indicate there is an increased risk of
specific complications. These include an increased risk of miscarriage
and an increased risk for developing blood clots that may lead to stroke
or lung injury.
Typical clinical features include:
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Fever, fatigue, and weight loss
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Arthritis involving multiple joints for several weeks
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Butterfly-shaped rash over the cheeks or other rashes
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Skin rash appearing in areas exposed to the sun
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Sores in the mouth or nose for more than a month
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Loss of hair, sometimes in spots or around the hairline
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Seizures, strokes and mental disorders
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Blood clots in different locations
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Miscarriages in some patients
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Blood or protein in the urine or tests that suggest poor kidney function
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Low blood counts (anemia, low white blood cells or low platelets)
Other symptoms include chest pain when the patient breathes deeply, heartburn,
abdominal pain and poor circulation to the fingers and toes.
All of these symptoms can develop gradually, making lupus hard to diagnose.
(See section below on the role of a rheumatologist.)
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| Lupus is a disease that can lead to inflammation in multiple
organs
including the kidneys, the lining tissue of the heart and lungs
(pleuritis and pericarditis), and the brain as well as the joints
and
skin. |
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How lupus is treated
Management of SLE can be a challenge. Treatment depends on symptoms and
their severity. Careful and frequent medical evaluation is therefore important
for monitoring symptoms and adjusting treatment as necessary.
Conservative treatment is appropriate for patients with muscle or joint
pain, fatigue, skin manifestations (such as rashes), and other features
that are not life-threatening. Conservative options include nonsteroidal
anti-inflammatory drugs (NSAIDs) such as ibuprofen (Motrin, Advil) and
naproxen (Naprosyn) and anti-malarial medications such as hydroxychloroquine (Plaquenil).
More aggressive therapy is required for life-threatening and more serious
manifestations such as kidney inflammation, lung or heart involvement,
and central nervous system symptoms. Treatment in these circumstances
might involve high dose corticosteroids such as prednisone (Deltasone)
and other immunosuppressive drugs such as azathioprine (Imuran),
cyclophosphamide (Cytoxan), and cyclosporine (Neoral,
Sandimmune).
Recently mycophenolate
mofetil (CellCept) has been used to
treat severe lupus kidney disease. Sometimes several medications must
be combined to control the disease and prevent tissue damage.
Treatment depends upon an individual assessment of risks and benefits.
Most immunosuppressive medications, for instance, may cause significant
side effects such as increased risk of infections, nausea, vomiting, hair
loss, diarrhea, high blood pressure, and osteoporosis. Rheumatologists
may also reduce or discontinue a medication after the disease goes into
remission for a period of time.
Clinical trials to evaluate new treatments are always being undertaken,
with the hope that more promising drugs will be identified and made more
widely available.
Broader health impact of lupus
Even when it is not active, SLE may cause complications later on. One of
these problems is accelerated atherosclerosis (clogging of the arteries),
which increases the risk of heart attacks and other cardiovascular events
like heart failure and strokes. This makes it crucial to try to prevent
such complications by reducing other risk factors for heart disease such
as smoking, high blood pressure, and high cholesterol. SLE may also cause
kidney disease, which can progress to renal failure and require dialysis.
This can be prevented by the early and aggressive treatment of the first
signs of kidney disease.
Living with lupus
While the treatment for SLE has improved and long-term survival has increased,
it remains a chronic disease that can limit activities. Although severe
sickness can occur, this usually results from the most serious forms of
the disease. More often quality of life is compromised by symptoms like
fatigue and joint pains, which are not life threatening. The best way
to control lupus is to be very careful to take all your medications as
prescribed, visit your physician regularly, and learn as much as you can
about lupus, your medications, and your progress. Get involved in your
care. Don't take a back seat.
The unpredictability of lupus and the occasional lack of response to
treatment may cause some patients to be depressed, lose hope or the will
to keep fighting, or other emotional distress such as irritability or
anger. A good doctor-patient relationship and solid support from family
and friends are key to learning to cope with this chronic and often unpredictable
illness.
Maintaining an active lifestyle will usually help keep joints flexible
and may prevent cardiovascular complications. This does not mean overdoing
it; the best approach is to alternate light to moderate exercise with
periods of rest or relaxation.
Patients with lupus should avoid excessive sun exposure because the ultraviolet
rays in sunlight can cause a skin rash to flare, and may even trigger
a more serious flare in the disease itself. Wearing protective clothing
(long sleeves, a big-brimmed hat) and using sunscreen liberally when outdoors
on a sunny day should protect against such complications.
Young women with lupus who wish to have a baby should carefully plan
their pregnancies. With the guidance of their doctor, they should try
to time their pregnancies for a period when the disease is least active.
It is crucial to carefully monitor the pregnancy and avoid certain medications
(such as cyclophosphamide,
cyclosporine, and mycophenolate
mofetil) that would harm the baby. Women whose disease is very active,
or who are taking some of these harmful medications should use birth control.
The possibility that the use of estrogen may induce or worsen lupus has
always been a concern for rheumatologists. Recent research has shown that
estrogen can trigger some mild or moderate flares of lupus, but does not
cause very severe exacerbation of symptoms. However, because estrogen can
increase the risk of blood clots, it should be avoided in patients with
lupus whose blood tests have revealed the presence of antiphospholipid antibodies.
Points to Remember
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The best way to control lupus is to be very careful
to take all your medications as prescribed, visit your physician
regularly, and learn as much as you can about lupus, your medications,
and your progress. Get involved in your care. Don't take a back
seat. |
Most people with SLE can live normal lives, but this disorder must be
carefully monitored and treatment adjusted as necessary to prevent serious
complications.
The role of a rheumatologist in the treatment
of lupus
Lupus is a complex disease. Rheumatologists specialize in the diagnosis
and treatment of autoimmune disorders such as lupus, and will therefore
be able to provide patients with the best advice about treatment options.
To find a rheumatologist
For more information about rheumatologists, click
here.
For a listing of rheumatologists in your area, click
here.
For more information
The American College of Rheumatology has compiled this list to give you
a starting point for your own additional research. The ACR does not endorse
or maintain these Web sites, and is not responsible for any information
or claims provided on them. It is always best to talk with your rheumatologist
for more information and before making any decisions about your care.
The Arthritis Foundation
www.arthritis.org
The Lupus Foundation of America
www.lupus.org
Lupus Research Institute
www.LupusResearchInstitute.org
National Institute of Arthritis and Musculoskeletal and Skin Diseases
Information Clearinghouse
www.niams.nih.gov
Updated May 2004
Written by Ellen Ginzler, MD, and Jean Tayar, MD, and reviewed by the American
College of Rheumatology Communications and Marketing Committee.
