What is Lupus?

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 anaemia 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 similar 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 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.

Known Causes

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.

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

Diagnosing lupus can be difficult because symptoms often mimic other diseases and vary from patient to patient. Your doctor will first attempt to rule out other connective tissue diseases. Some of the tests your doctor may perform include a complete blood count, platelet count and serum electrophoresis to indicate the levels of white blood cells and plasma proteins.

Blood testing for anti-DNA antibodies — which shows whether you have antibodies to the normal genetic material in certain cells — is the most definitive way to identify lupus.

Conventional treatments

For milder cases, nonsteroidal anti-inflammatory drugs (NSAIDs), such as aspirin, can be used to relieve joint pain. Stubborn rashes and more severe joint pain may respond to hydroxychloroquine, which is also used to treat malaria. A short course of corticosteroids reduces inflammation and fever and is recommended for flareups. Cyclophosphamides, which subdue the immune system, may be used for severe cases of lupus involving renal damage. Antidepressants and mild antianxiety drugs can help with the sleeping problems that frequently accompany the disease.

Mild skin rashes can be treated topically with over-the-counter corticosteroid creams; thicker lesions may require prescription fluorinated steroid creams or injections of triamcinolone.

Prevention

Because no one knows what causes lupus, there is no way to prevent it. Flareups can be managed, however, by avoiding known triggers such as sunlight, stress and lack of sleep. Pay careful attention to your diet and exercise. In addition, keep a record of your symptoms — when they occur, what precedes them and how long they last — and adjust your routine with them in mind.

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Contacts

Hampshire Lupus Group

Brian Hanner
Director
Lupus UK
St James House
Eastern Road
Romford
Essex
RM1 3NH

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