What is Lupus

Definition

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 fibrosis combined. LFA market research data show that between 1,400,000 and 2,000,000 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.

Types of Lupus

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 without 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 similas to those of systemic lupus. The drugs most commonly connected with drug-induced lupus are hydralazine (used to treat high blood pressure or hypertension) and procainamide (used to treat irregular heart rhythms). However, not everyone who takes 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.

Cause

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 orgin are thought the 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.

Pregnancy and Lupus

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 should 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 to 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."

Symptoms

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.

Diagnosis

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 diagnis of lupus, the American Rheumatism Association issued a list of 11
symptoms or signs that help distinguish lupus from other diseases (see 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.

Laboratory Tests Used in the Diagnosis of Lupus

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 rheumatic 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 in:

1. individuals with other connective tissue diseases;
2. individuals without symptoms;
3. patients being treated with certain drugs, including procainamide, hydralazine, isoniazid, and chlorpromazine;
4. individuals with conditions other than lupus, such as scleroderma, rheumatoid arthritis, infectious
mononucleosis and other chronic infectious diseases such as lepromatous leprosy, subacute bacterial
endocarditis, malaria, etc., and liver disease.

ANA test reports include a titier. The titer indicates how many times an individual's blood msut be diluted to
get a sample free of anti-nuclear antibodies. Thus, a titier 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 tests 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 remebers 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 expert 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, withgrowing 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 hteir 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 or clinical immunologist specializing in lupus is recommended. Refferals can be made through your family doctor, the local medical society, or the local Lupus Foundation chapter.

Flares (What Triggers Lupus?)

What triggers an attack of lupus in 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 the npossibly 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. Im 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.

Treatment

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. Regualr 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, exceessive 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 disease, 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: Corticosteroids (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 diadetes, increased risk of infection and stomach ulcers.

Anti-malarials: Chloroquine (Aralen) or hydroxy-
chloroquine (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. It may take months before these drugs demonstrate a beneficial effect. Side efects are rare, and consisit of occasional diarrhea or rashes. Some anti-malarial drugs, such as quinine and chloroquine, can effect 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 cortico-steroid 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 [hysician know when a change in therpy 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.

NUTRITION AND DIET

Although much is stil not known about the nutritional factors in many kinds of disease, no one questions the necessity of a well-balanced. 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 bee suggestions about various foods and the treatment of lupus. One example is fish oil. Howwever, these diets have been used only in animals with limited success and should not become the main-stay of a person's diet.

PROGNOSIS

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 lifespan 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 a new manifestation of their lupus. Although some people with lupus have severe recurrent attacks andare 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 physician's 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 there fore a sensible idea to maintain control of a disease that tomorrow may be curable.