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Systemic Lupus Erythematosus

Lupus is one of many disorders of the immune system known
as autoimmune diseases. In autoimmune diseases, the
immune system turns against parts of the body it is designed
to protect. This leads to inflammation and damage to various
body tissues. Lupus can affect many parts of the body,
including the joints, skin, kidneys, heart, lungs, blood vessels,
and brain. Although people with the disease may have many
different symptoms, some of the most common ones include
extreme fatigue, painful or swollen joints (arthritis),
unexplained fever, skin rashes, and kidney problems.

At present, there is no cure for lupus. However, lupus can be
effectively treated with drugs, and most people with the
disease can lead active, healthy lives.

We know that many more women than men have lupus.
Lupus is three times more common in African American
women than in Caucasian women and is also more common in
women of Hispanic, Asian, and Native American descent. In
addition, lupus can run in families, but the risk that a child or
a brother or sister of a patient will also have lupus is still quite
low.

There are several kinds of lupus:
Systemic lupus erythematosus (SLE) is the form of the
disease that most people are referring to when they say
“lupus.” The word “systemic” means the disease can affect
many parts of the body. The symptoms of SLE may be mild
or serious. Although SLE usually first affects people between
the ages of 15 and 45 years, it can occur in childhood or later
in life as well. This booklet focuses on SLE.

Discoid lupus erythematosus is a chronic skin disorder in
which a red, raised rash appears on the face, scalp, or
elsewhere. The raised areas may become thick and scaly and
may cause scarring. The rash may last for days or years and
may recur.

Subacute cutaneous lupus erythematosus refers to skin
lesions that appear on parts of the body exposed to sun. The
lesions do not cause scarring.

Drug-induced lupus is a form of lupus caused by
medications. Many different drugs can cause drug-induced
lupus. Symptoms are similar to those of SLE (arthritis, rash,
fever, and chest pain) and they typically go away completely
when the drug is stopped.

Neonatal lupus is a rare disease that can occur in newborn
babies of women with SLE, Sjögren’s syndrome, or no
disease at all. Scientists suspect that neonatal lupus is caused
by autoantibodies in the mother’s blood called anti-Ro (SSA)
and anti-La (SSB). At birth, the babies have a skin rash, liver
problems, and low blood counts. These symptoms gradually
go away over several months. In rare instances, babies with
neonatal lupus may have a serious heart problem that slows
down the natural rhythm of the heart.

Understanding What Causes Lupus
Lupus is a complex disease, and its cause is unknown. It is
likely that a combination of genetic, environmental, and
possibly hormonal factors work together to cause the disease.

In lupus, the body’s immune system does not work as it
should. A healthy immune system produces proteins called
antibodies and specific cells called lymphocytes that help fight
and destroy viruses, bacteria, and other foreign substances
that invade the body. In lupus, the immune system produces
antibodies against the body’s healthy cells and tissues. These
antibodies, called autoantibodies, contribute to the
inflammation of various parts of the body and can cause
damage to organs and tissues. The most common type of
autoantibody that develops in people with lupus is called an
antinuclear antibody (ANA) because it reacts with parts of the
cell’s nucleus.

Symptoms of Lupus
Each person with lupus has slightly different symptoms that
can range from mild to severe and may come and go over
time. However, some of the most common symptoms of
lupus include painful or swollen joints (arthritis), unexplained
fever, and extreme fatigue. A characteristic red skin rash—the
so-called butterfly or malar rash—may appear across the nose
and cheeks. Rashes may also occur on the face and ears,
upper arms, shoulders, chest, and hands. Because many
people with lupus are sensitive to sunlight (called
photosensitivity), skin rashes often first develop or worsen
after sun exposure.

Common Symptoms of Lupus
* Painful or swollen joints and muscle pain
* Unexplained fever
* Red rashes, most commonly on the face
* Chest pain upon deep breathing
* Unusual loss of hair
* Pale or purple fingers or toes from cold or stress
(Raynaud's phenomenon)
* Sensitivity to the sun
* Swelling (edema) in legs or around eyes
* Mouth ulcers
* Swollen glands
* Extreme fatigue

Other symptoms of lupus include chest pain, hair loss, anemia
(a decrease in red blood cells), mouth ulcers, and pale or
purple fingers and toes from cold and stress. Some people
also experience headaches, dizziness, depression, confusion,
or seizures. New symptoms may continue to appear years
after the initial diagnosis, and different symptoms can occur
at different times. In some people with lupus, only one
system of the body, such as the skin or joints, is affected.
Other people experience symptoms in many parts of their
body. Just how seriously a body system is affected varies
from person to person.

Diagnosing Lupus
Diagnosing lupus can be difficult. It may take months or even
years for doctors to piece together the symptoms to
diagnose this complex disease accurately.

The most useful tests identify certain autoantibodies often
present in the blood of people with lupus. For example, the
antinuclear antibody (ANA) test is commonly used to look for
autoantibodies that react against components of the nucleus,
of the body’s cells. Most people with lupus test positive for
ANA; however, there are a number of other causes of a
positive ANA besides lupus, including infections, other
autoimmune diseases, and occasionally as a finding in healthy
people.

In addition, there are blood tests for individual types of
autoantibodies that are more specific to people with lupus,
although not all people with lupus test positive for these and
not all people with these antibodies have lupus. These
antibodies include anti-DNA, anti-Sm, anti-RNP, anti-Ro
(SSA), and anti-La (SSB). The doctor may use these antibody
tests to help make a diagnosis of lupus.

Some tests are used less frequently but may be helpful if the
cause of a person's symptoms remains unclear. The doctor
may order a biopsy of the skin or kidneys if those body
systems are affected. Some doctors may order a test for
anticardiolipin (or antiphospholipid) antibody. The presence of
this antibody may indicate increased risk for blood clotting
and increased risk for miscarriage in pregnant women with
lupus. Again, all these tests merely serve as tools to give the
doctor clues and information in making a diagnosis. The
doctor will look at the entire picture—medical history,
symptoms, and test results—to determine if a person has
lupus.

People with lupus often have increased erythrocyte
sedimentation rate and low complement levels, especially
during flares of the disease. X rays and other imaging tests
can help doctors see the organs affected by SLE.

Diagnostic Tools for Lupus
Medical history
Complete physical examination
Laboratory tests:
o Complete blood count (CBC)
o Erythrocyte sedimentation rate (ESR)
o Urinalysis
o Blood chemistries
o Complement levels
o Antinuclear antibody test (ANA)
o Other autoantibody tests (anti-DNA, anti-Sm, anti-RNP,
anti-Ro [SSA], anti-La [SSB])
o Anticardiolipin antibody test
Skin biopsy
Kidney biopsy

Treating Lupus
Immunosuppressives: For some patients whose kidneys or
central nervous systems are affected by lupus, a type of drug
called an immunosuppressive may be used.
Immunosuppressives, such as cyclophosphamide (Cytoxan)
and mycophenolate mofetil (CellCept), restrain the overactive
immune system by blocking the production of immune cells.
These drugs may be given by mouth or by infusion (dripping
the drug into the vein through a small tube). Side effects may
include nausea, vomiting, hair loss, bladder problems,
decreased fertility, and increased risk of cancer and infection.
The risk for side effects increases with the length of
treatment. As with other treatments for lupus, there is a risk
of relapse after the immunosuppressives have been stopped.

Other Therapies: In some patients, methotrexate (Folex,
Mexate, Rheumatrex), a disease-modifying antirheumatic
drug, may be used to help control the disease. Working
closely with the doctor helps ensure that treatments for lupus
are as successful as possible. Because some treatments may
cause harmful side effects, it is important to report any new
symptoms to the doctor promptly. It is also important not to
stop or change treatments without talking to the doctor first.

Alternative and Complementary Therapies: Because of the
nature and cost of the medications used to treat lupus and
the potential for serious side effects, many patients seek
other ways of treating the disease. Some alternative
approaches people have tried include special diets, nutritional
supplements, fish oils, ointments and creams, chiropractic
treatment, and homeopathy. Although these methods may
not be harmful in and of themselves, and may be associated
with symptomatic or psychosocial benefit, no research to date
shows that they affect the disease process or prevent organ
damage. Some alternative or complementary approaches may
help the patient cope or reduce some of the stress associated
with living with a chronic illness. If the doctor feels the
approach has value and will not be harmful, it can be
incorporated into the patient's treatment plan. However, it is
important not to neglect regular health care or treatment of
serious symptoms. An open dialogue between the patient and
physician about the relative values of complementary and
alternative therapies allows the patient to make an informed
choice about treatment options.

Because some treatments may cause harmful side effects...
report any new symptoms to the doctor promptly.

Content Credit NIAMS NIH GOV 2006

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