What is Lupus?
Contents
Lupus is a chronic, autoimmune disease which causes inflammation of
various parts of the body, especially the skin, joints, blood and
kidneys. The body's immune system normally makes proteins called
antibodies to protect the body against viruses, bacteria and other
foreign materials. These foreign materials are called antigens. In an
autoimmune disorder such as lupus, the immune system loses its ability
to tell the difference between foreign substances (antigens) and its
own cells and tissues. The immune system then makes antibodies directed
against "self." These antibodies, called "auto-antibodies," react with
the "self" antigens to form immune complexes. The immune complexes
build up in the tissues and can cause inflammation, injury to tissues,
and pain.
More people have lupus than AIDS, cerebral palsy, multiple sclerosis, sickle-cell
anemia and cystic fibroses combined. LFA market research data show that
between 1,400,00 and 2,00,00 people reported to have been diagnosed with
lupus. (Study conducted by Bruskin/Goldring Research, 1994.) For most people,
lupus is a mild disease affecting only a few organs. For others, it may
cause serious and even life-threatening problems. Thousands of Americans
die each year from lupus-related complications.
There are three types of lupus: discoid, systemic, and drug-induced. Discoid
lupus is always limited to the skin. It is identified by a rash that may
appear on the face, neck and scalp. Discoid lupus is diagnosed by examining
a biopsy of the rash. In discoid lupus the biopsy will show abnormalities
that are not found in skin with the rash. Discoid lupus does not generally
involve the body's internal organs. Therefore, the ANA test, a blood test
used to detect systemic lupus, may be negative in patients with discoid lupus.
However, in a large number of patients with discoid lupus, the ANA test is
positive, but at a low level or "titer."
In approximately 10 percent of the people with lupus, discoid lupus can evolve
into the systemic form of the disease, which can affect almost any organ
or system of the body. This cannot be predicted or prevented. Treatment
of discoid lupus will not prevent its progression to the systemic form.
Individuals who progress to the systemic form probably had systemic lupus
at the outset, with the discoid rash as their main symptom.
Systemic lupus is usually more severe than discoid lupus, and can affect
almost any organ or system of the body. For some people, only the skin and
joints will be involved. In others, the joints, lungs, kidneys, blood or
other organs and/or tissues may be affected. Generally, no two people with
systemic lupus will have identical symptoms. Systemic lupus may include
periods in which few, if any symptoms are evident (remission) and other times
when the disease becomes more active (flare). Most often when people mention
"lupus," they are referring to the systemic form of the disease.
Drug-induced lupus occurs after the use of certain prescribed drugs. The
symptoms of drug-induced lupus are similar to those of systemic lupus.
The drugs most commonly connected with drug-induced lupus are hydralazine
(used treat high blood pressure or hypertension) and procainamide (used
to treat irregular heart rhythms). However, not everyone who takes these
drugs will develop drug-induced lupus. Only about 4 percent of the people
who take these drugs will develop the antibodies suggestive of lupus. Of
those 4 percent, only an extremely small number will develop overt drug-induced
lupus. The symptoms usually fade when the medications are
discontinued.
Although drug-induced lupus and discoid lupus share features of systemic
lupus, the rest of this brochure primarily discusses systemic lupus.
The cause(s) of lupus is unknown, but environmental and genetic factors are
involved. While scientists believe there is a genetic predisposition to
the disease, it is known that environmental factors also play a critical
role in triggering lupus. Some of the environmental factors that may trigger
the disease are: infections, antibiotics (especially those in the sulfa and
penicillin groups), ultraviolet light, extreme stress, and certain
drugs.
Although lupus is known to occur within families, there is no known gene
or genes which are thought to cause the illness. Only 10 percent of lupus
patients will have a close relative (parent or sibling) who already has or
may develop lupus. Statistics show that only about 5% of the children born
to individuals with lupus will develop the illness.
Lupus is often called a "woman's disease" despite the fact that many men
are affected. Lupus can occur at any age, and in either sex, although it
occurs 10-15 times more frequently among adult females than among adult males.
The symptoms of the disease are the same in men and women. People of African,
American Indian, and Asian origin are thought to develop the disease more
frequently than Caucasian women, but the studies that led to this result
are small and need corroboration.
Hormonal factors may explain why lupus occurs more frequently in females
than in males. The increase of disease symptoms before menstrual periods
and/or during pregnancy support the belief that hormones, particularly estrogen,
may be involved. However, the exact hormonal reason for the greater prevalence
of lupus in women, and the cyclic increase in symptoms, is unknown.
A question of concern to many families is whether or not a young woman with
lupus should risk becoming pregnant. The current general view is that there
is no absolute reason why a woman with lupus would not get pregnant, unless
she has moderate to severe organ involvement (i.e., central nervous system,
kidney, or heart and lungs) which would place the mother at risk. However,
there is some increased risk of disease activity during or immediately (3
or 4 weeks) after pregnancy. If a person is monitored carefully, the danger
can be minimized. A pregnant woman with lupus should be closely followed
by both her obstetrician and her "lupus doctor."
Although lupus can affect any part of the body, most people experience symptoms
in only a few organs. Table 1 lists the most common symptoms of people with
lupus. (Look here if you have a browser capable
of displaying tables, otherwise here.)
Because many lupus symptoms mimic other illnesses, are sometimes vague and
may come and go, lupus can be difficult to diagnose. Diagnosis is usually
made by a careful review of a person's entire medical history coupled with
an analysis of the results obtained in routine laboratory tests and some
specialized tests related to immune status. Currently, there is no single
laboratory test that can determine whether a person has lupus or not. To
assist the physician in the diagnosis of lupus, the American Rheumatism Association
issued a list of 11 symptoms or signs that help distinguish lupus from other
diseases (Table 2). A person should have four or more of these symptoms
to suspect lupus. The symptoms do not all have to occur at the same
time.
Table 2
The Eleven Criteria Used For The Diagnosis of Lupus Erythematosus
- Malar Rash
- Rash over the cheeks
- Discoid Rash
- Red raised patches
- Photosensitivity
- Reaction to sunlight, resulting in the development or increase in skin rash
- Oral Ulcers
- Ulcers in the nose or mouth, usually painless
- Arthritis
- Nonerosive arthritis involving two or more peripheral joints (arthritis
in which the bones around the joints do not become destroyed)
- Serositis
- Pleuritis or pericarditis
- Renal Disorder
- Excessive protein in the urine (greater than 0.5 gm/day or 3+ on test
sticks) and/or cellular casts (abnormal elements the urine, derived from
red and/or white cells and/or kidney tubule cells)
- Neurologic Disorder
- Seizures (convulsions) and/or psychosis in the absence of drugs or metabolic
disturbances which are known to cause such effects
- Hematologic Disorder
- Hemolytic anemia or leukopenia (white blood count below 4,000 cells per
cubic millimeter) or lymphopenia (less than 1,500 lymphocytes per cubic millimeter)
or thrombocytopenia (less than 100,000 platelets per cubic millimeter).
The leukopenia and lymphopenia must be detected on two or more occasions.
The thrombocytopenia must be detected in the absence of drugs known to induce
it
- Immunologic Disorder
- Positive LE prep test, positive anti-DNA test, positive anti-Sm test
or false positive syphilis test (VDRL).
- Antinuclear Antibody
- Positive test for antinuclear antibodies (ANA) in the absence of drugs
known to induce it.
The first laboratory test ever devised was the LE (lupus erythematosus) cell
test. When the test is repeated many times, it is eventually positive in
about 90 percent of the people with systemic lupus. Unfortunately, the LE
cell test is not specific for systemic lupus (despite the official-sounding
name). The test can also be positive in up to 20 percent of the people with
rheumatoid arthritis, in some patients with other rheumatic conditions like
Sjogren's syndrome or scleroderma, in patients with liver disease, and in
persons taking certain drugs (such as procainamide, hydralazine, and
others).
The immunofluorescent antinuclear antibody (ANA or FANA) test is more specific
for lupus than the LE cell prep test. The ANA test is positive in virtually
all people with systemic lupus, and is the best diagnostic test for systemic
lupus currently available. If the test is negative, the patient will likely
not have systemic lupus On the other hand, a positive ANA, by itself, is
not diagnostic of lupus since the test may also be positive:
- individuals with other connective tissue diseases
- individuals without symptoms
- patients being treated with certain drugs, including procainamide, hydralazine,
isoniazid, and chloropromazine
- individuals with conditions other than lupus, such as scleroderma, rheumatoid
arthritis, infectious mononucleosis and other chronic infectious diseases such
as lepromatous leprosy, suacute bacterial endocarditis, malaria, etc., and
liver disease.
ANA test reports include a titer. The titer indicates how many times an individual's
blood must be diluted to get a sample free of anti-nuclear antibodies. Thus,
a titer of 1:640 shows a greater concentration of anti-nuclear antibodies
than a titer of 1:320 or 1:160. The titer is always highest in people with
lupus. Patients with active lupus have ANA test that are very high in
titer.
Laboratory tests which measure complement levels in the blood are also
of some value. Complement is a blood protein that, with antibodies,
destroys bacteria. It is an "amplifier" of immune function. If the
total blood complement level is low, or the C3 or C4 complement values
are low, and the person also has a positive ANA, some weight is added
to the diagnosis of lupus. Low C3 and C4 complement levels in
individuals with positive ANA test results may also be indicative of
lupus kidney disease.
New tests of individual antigen antibody reactions have been developed
which are very helpful in the diagnosis of SLE. These include the
anti-DNA antibody test, the anti-Sm antibody test, the anti-RNP
antibody test, the anti-Ro antibody test, and tests which measure serum
complement levels. These tests can also be further explained by your
physician.
Laboratory tests are most useful when one remembers the following
information. If an individual has signs and symptoms supporting the
diagnosis of lupus (e.g., at least four of the American Rheumatism
Association criteria), a positive ANA confirms
the diagnosis and no further testing is necessary. If a person has
only two or three of the American Rheumatism Association criteria, then
a positive ANA supports the diagnosis. In these cases, unless more
specific tests are positive (e.g., anti-DNA, anti-Sm, anti-Ro) the
diagnosis of lupus is uncertain until more clinical findings develop or
other more specific blood tests, as cited above become positive.
Physicians will sometimes also perform skin biopsies of both the
individual's rashes and his or her normal skin. These biopsies can
help diagnose systemic lupus in about 75 percent of patients.
The interpretation of all these positive or negative tests, and their
relationship to symptoms, is frequently difficult. A test may be
positive one time and negative another time, reflecting the relative
activity of the disease or other variables. When questions cannot be
resolved, consult an export in lupus.
When someone has many symptoms and signs of lupus and has positive
tests for lupus, physicians have little problem making a correct
diagnosis and initiating treatment. However, a more common problem
occurs when an individual has vague, seemingly unrelated symptoms of
achy joints, fever, fatigue, or pains. Some doctors may think the
person is neurotic. Others may try different drugs in the hope of
suppressing the symptoms. Fortunately, with growing awareness of
lupus, an increasing number of physicians will consider the possibility
of lupus in the diagnosis.
A patient can help the doctor by being open and honest. A healthy
dialogue between patient and doctor results in better medical care, not
only for people with lupus, but for anyone seeking medical treatment.
To whom should a person go for a diagnosis of lupus? Most individuals
usually seek the help of their family doctor first, and this is often
sufficient. However, when unresolved questions arise or complications
develop, another opinion from a specialist may be advisable. The
choice of specialist depends on the problem. For example, you would
see a nephrologist for a kidney problem or a dermatologist for a skin
problem. Most often, a rheumatologist or clinical immunologist
specializing in lupus is recommended. Referrals can be made through
your family doctor, the local medical society, or the local Lupus
Foundation chapter.
What triggers an attack of lupus n a susceptible person? Scientists
have noted common features in many lupus patients. In some, exposure
to the sun causes sudden development of a rash and then possibly other
symptoms. In others, an infection, perhaps a cold or a more serious
infection, does not get better, and then complications arise. These
complications may be the first signs of lupus. In still other cases, a
drug taken for some illness produces the signaling symptoms. In some
women, the first symptoms and signs develop during pregnancy. In
others, they appear soon after delivery. Many people cannot remember
or identify any specific factor. Obviously, many seemingly unrelated
factors can trigger the onset of the disease.
For the vast majority of people with lupus, effective treatment can
minimize symptoms, reduce inflammation, and maintain normal bodily
functions.
Preventive measures can reduce the risk of flares. For photosensitive
patients, avoidance of (excessive) sun exposure and/or the regular
application of sun screens will usually prevent rashes. Regular
exercise helps prevent muscle weakness and fatigue. Immunization
protects against specific infections. Support groups, counseling,
talking to family members, friends, and physicians can help alleviate
the effects of stress. Needless to say, negative habits are hazardous
to people with lupus. These include smoking, excessive consumption of
alcohol, too much or too little of prescribed medication, or postponing
regular medical checkups.
Treatment approaches are based on the specific needs and symptoms of
each person. Because the characteristics and course of lupus may vary
significantly among people, it is important to emphasize that a
thorough medical evaluation and ongoing medical supervision are
essential to ensure proper diagnosis and treatment.
Medications are often prescribed for people with lupus, depending on
which organ(s) are involved, and the severity of involvement.
Effective patient-physician discussions regarding the selection of
medication, its possible side effects, and any changes in doses are
vital. Commonly prescribed medications include:
- Nonsteroidal Anti-Inflammatory Drugs (NSAIDs)
- These medications are prescribed for a variety of rheumatic
diseases, including lupus. The compounds include acetylsalicylic
acid (e.g., aspirin), ibuprofen (Motrin), naproxen (Naprosyn),
indomethacin (Indocin), sulindac (Clinoril), tolmetin (Tolectin),
and a large number of others. These drugs are usually
recommended for muscle and joint pain, and arthritis. Aspirin
may cause stomach upsets for some people. This effect can be
minimized by taking them with meals, milk, or antacids. The
other NSAIDs work in the same way as aspirin, but tend to be less
irritating to the stomach than aspirin, and often require fewer
pills per day to have the same effect as aspirin.
- Acetaminophen
- Acetaminophen (e.g., Tylenol) is a mild analgesic that can often
be used for pain. It has the advantage of less stomach
irritation than aspirin, but it is not nearly as effective at
suppressing inflammation as aspirin.
- Corticosteroids
- Coricosteroids (steroids) are hormones that have
anti-inflammatory and immunoregulatory properties. They are
normally produced in small quantities by the adrenal gland. This
hormone controls a variety of metabolic functions in the body.
Synthetically produced corticosteroids are used to reduce
inflammation and suppress activity of the immune system. The
most commonly prescribed drug of this type is Prednisone.
Because steroids have a variety of side effects, the dose has to
be regulated to maximize the beneficial
anti-immune/anti-inflammatory effects and minimize the negative
side effects. Side effects occur more frequently when steroids
are taken over long periods of time at high doses (for example,
60 milligrams of Prednisone taken daily for periods of more than
one month). Such side effects include weight gain, a round face,
acne, easy bruising, "thinning" of the bones (osteoporosis), high
blood pressure, cataracts, onset of diabetes, increased risk of
infection and stomach ulcers.
- Anti-malarials
- Chloroquine (Aralen) or hydroxychloroquine (Plaquenil), commonly
used in the treatment of malaria, may also be very useful in some
individuals with lupus. they are most often prescribed for skin
and joint symptoms of lupus. They are most often prescribed for
skin and joint symptoms of lupus. It may take months before
these drugs demonstrate a beneficial effect. Side effects are
rare, and consist of occasional diarrhea or rashes. Some
anti-malarial drugs, such as quinine and chloroquine, can affect
the eyes. Therefore, it is important to see an eye doctor
(ophthalmologist) regularly. The manufacturer suggests an eye exam
before starting the drug and one exam every six months
thereafter. However your physician might suggest a yearly exam
as sufficient.
- Cytotoxic Drugs
- Azathioprine (Imuran) and cyclophosphamide (Cytoxan) are in a
group of agents known as cytotoxic or immunosuppressive drugs.
these drugs act in a similar manner to the corticosteroid drugs
in that they suppress inflammation and tend to suppress the
immune system. The side effects of these drugs include anemia,
low white blood cell count, and increased risk of infection.
Their use may also predispose an individual to developing cancer.
People with lupus should learn to recognize early symptoms of
disease activity. In that way they can help the physician know
when a change in therapy is needed. Regular monitoring of the
disease by laboratory tests can be valuable because noticeable
symptoms may occur only after the disease has significantly
flared. Changes in blood test results may indicate the disease
is becoming active even before the patient develops symptoms of a
flare. Generally, it seems that the earlier such flares are
detected, the more easily they can be controlled. Also, early
treatment may decrease the chance of permanent tissue or organ
damage and reduce the time one must remain on high doses of drugs
Although much is still not known about the nutritional factors in many
kinds of disease, no one questions the necessity of a well-balanced
diet. Fad diets, advocating an excess or an exclusion of certain types
of foods, are much more likely to be detrimental than beneficial in any
disease, including lupus. Scientists have shown that both antibodies
and other cells of the immune system may be adversely affected by
nutritional deficiencies or imbalances. Thus, significant deviations
from a balanced diet may have profound effects on a network as complex
as the immune system.
There have been suggestions about various foods and the treatment of
lupus. One example is fish oil. However, these diets have been used
only in animals with limited success and should not become the mainstay
of a person's diet.
The idea that lupus is generally a fatal disease is one of the gravest
misconceptions about this illness. In fact, the prognosis of lupus is
much better than ever before. Today, with early diagnosis and current
methods of therapy, 80-90 percent of people with lupus can look forward
to a normal life span if they follow the instructions of their
physician, take their medication(s) as prescribed, and know when to
seek help for unexpected side-effects of a medication or an new
manifestation of their lupus. Although some people with lupus have
severe recurrent attacks and are frequently hospitalized, most people
with lupus rarely require hospitalization. There are many lupus
patients who never have to be hospitalized, especially if they are
careful and follow their physicians instructions.
New research brings unexpected findings each year. The progress made
in treatment and diagnosis during the last decade has been greater than
that made over the past 100 years. It is therefore a sensible idea to
maintain control of a disease that tomorrow may be curable
- Author
- Robert G Lahita, M.D., Ph.D.
Chief, Division of Rheumatology and Connective Tissue Diseases
St. Luke's/Roosevelt Hospital Center
Associate Professor, College of Physicians Surgeons
Columbia University, New York, NY
- Acknowledgment
- The above text was taken from one of the many brochures
available from the Lupus Foundation of America
Lupus Home Page
lupus@piper.hamline.edu
Last modified: 1996-10-09