Lupus

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Achy, swollen joints; fever; skin rashes; fatigue—these are some of the more typical symptoms of systemic lupus erythematosus (SLE), a chronic, inflammatory disorder of the immune system that affects several body systems simultaneously. Approximately 1.5 million to 2 million people in the United States have some form of lupus, and, according to the Lupus Foundation of America, the number may be even higher.

SLE is classified as an autoimmune disorder because the body's immune system, which normally fights harmful bacteria and viruses, also targets healthy tissue, sometimes including skin, joints, kidneys, brain, heart and lungs. The condition can result in immediately serious or life-threatening problems or in chronic low-grade symptoms, such as fatigue and muscle aches, which affect the quality of life.

In Latin, lupus means wolf, and erythematosus means redness. Physicians who first described the disease thought that one of its characteristic rashes looked liked a wolf bite. The terms "SLE" and "lupus" are often used interchangeably, but there are several kinds of lupus, including:

• Systemic lupus erythematosus (SLE) can affect almost any organ or system in the body. In some people with systemic lupus, only the skin and joints are involved; in others, the joints, lungs, kidneys, blood, or other organs and/or tissues are all affected. Any two people with systemic lupus will be unlikely to have all the same symptoms. Systemic lupus may include remission periods during which few or no symptoms are evident and "flares," when the disease becomes active.

• Discoid lupus erythematosus (DLE), also called cutaneous lupus, involves the development of lesions on the face or other sun-exposed areas. The lesions are abnormally red, raised, hard bumps or plaques. They may include an overgrowth of scaly tissue, plugged hair follicles and abnormally widened small blood vessels. Thinning of the healing skin, called atrophic scarring, as well as loss of color in the skin, called dyspigmentation, may occur in older lesions. If the condition involves the scalp, there may be permanent scarring and loss of hair. Lesions are usually on the face or other sun-exposed areas. Many people have DLE without SLE. In approximately 10 percent of these cases, DLE later progresses to the more severe SLE. This is more likely to happen in patients with lesions both above and below the neck.

• Drug-induced lupus is caused by certain medications, the most common being: procainamide (Pronesyl), used for heart rhythm abnormalities; hydralazine (Hydra-Zide), used for high blood pressure; and isoniazid (Nydrazid), used for tuberculosis. Drug-induced lupus usually doesn't affect the kidneys or central nervous system and typically improves when the drug is discontinued.

• Neonatal lupus, a very rare condition in newborns, results from the passage of autoantibodies from the mother to her baby, specifically anti-Ro/SSA or anti-La/SSB, which can affect the skin, heart and blood of the fetus and newborn. The most common symptom of neonatal lupus is an uncomplicated rash that appears within the first several weeks of life, which may persist for about six months before disappearing. Less frequently, fetuses with neonatal lupus develop a congenital heart block. However, many of these babies do well with a pacemaker at birth. If a fetal heart condition is identified early enough during pregnancy, it might be possible to treat it with steroids or immunoglobulins.

In general, women are far more likely than men to develop autoimmune disorders, and SLE certainly fits that paradigm, occurring 10 times more frequently among adult women than among men. It is also more common in African-Americans, American Indians and Asians than in Caucasians. Although lupus can develop at any age, it is usually diagnosed in women during their childbearing years.

If you have a parent, child, or sibling with lupus, your risk of developing the disease is somewhat higher, although your health care professional probably won't test you for the disease unless you develop symptoms. There is no known cure for SLE, but there are treatments designed to minimize symptoms and effects.

There is a genetic component in lupus, but it is a complicated kind of inheritance, involving many genes interacting to determine a person's risk. Genes are blueprints for the proteins that do the work of the body, and to protect the body from invaders, the immune system involves a huge army of these proteins. Small inherited differences in any one of these proteins might cause it to underreact or overreact in an immune system maneuver. Any one small difference is unlikely to cause a health problem. Therefore, it is thought that people who develop lupus may have inherited a number of small differences and not necessarily the same differences from patient to patient. Why do these differences exist? If every human had an identical immune system, it is likely that some infection would have destroyed our species a long time ago. Diversity in these small inherited blueprints for our proteins is essential for the survival of the human race. In lupus, it may be that a person was simply dealt a bad hand. Multiple areas of the genome are being investigated for potential contributions to some or all lupus cases.

The number and type of lupus symptoms vary widely among patients. Symptoms also tend to wax and wane, with patterns of inactive disease bracketed by lupus "flares."