Definition and Description of Lupus

article syndicated from NIAMS
updated about 1 year ago

Lupus Erythematosus

Care for the patient with lupus erythematosus is a challenge that draws on all the resources, knowledge, and strengths the health care team has to offer. Because of the unpredictable, highly individualized, and frequently changing nature of the disease as well as the intricacy of each patient's needs, it is impossible to predict the treatment for one patient from the outcome of treatment for another. Careful listening to the person's concerns; a cooperative, multidisciplinary approach; and a flexible plan of care will provide the patient with consistent, supportive care and the reassurance that her or his needs are being attended to.

Definition and Description

Lupus means "wolf." Erythematosus means "redness." In 1851, doctors coined this name for the disease because they thought the facial rash that frequently accompanies lupus looked like the bite of a wolf. Lupus can be categorized into three groups: discoid lupus erythematosus, systemic lupus erythematosus, and drug-induced systemic lupus erythematosus.

Discoid lupus erythematosus (DLE) is characterized by a skin rash only. It occurs in about 20% of patients with systemic lupus erythematosus. The lesions are patchy, crusty, sharply defined skin plaques that may scar. These lesions are usually seen on the face or other sun-exposed areas. DLE may cause patchy, bald areas on the scalp and hypopigmentation or hyperpigmentation in older lesions. Biopsy of a lesion will usually confirm the diagnosis. Topical and intralesional corticosteroids are usually effective for localized lesions; antimalarial drugs may be needed for more generalized lesions. DLE only rarely progresses to systemic lupus erythematosus.

Systemic lupus erythematosus (SLE, or lupus) is a chronic, inflammatory, multisystem disorder of the immune system. Lupus: A Patient Care Guide for Nurses and Other Health Professionals is concerned primarily with this form of lupus. In SLE, the body develops antibodies that react against the person's own normal tissue. This abnormal response leads to the many manifestations of SLE and can be very damaging. The course is unpredictable and individualized; no two patients are alike. Lupus is not contagious, infectious, or malignant. It usually develops in young women of childbearing years, but many men and children also develop lupus. African Americans and Hispanics have a higher frequency of this disease than do Caucasians. SLE also appears in the first-degree relatives of lupus patients more often than it does in the general population, which indicates a strong hereditary component. However, most cases of SLE occur sporadically, indicating that both genetic and environmental factors play a role in the development of the disease.

Lupus varies greatly in severity, from mild cases requiring minimal intervention to those in which significant and potentially fatal damage occurs to vital organs such as the lungs, heart, kidney, and brain. The disease is characterized by "flares" of activity interspersed with periods of improvement or remission. A flare, or exacerbation, is increased activity of the disease process with an increase in physical manifestations and/or abnormal laboratory test values. Periods of improvement may last weeks, months, or even years. The disease tends to remit over time. Some patients never develop severe complications, and the outlook is improving for those patients who do develop severe manifestations.

Drug-induced SLE develops after the use of certain drugs and has symptoms similar to those of SLE. The characteristics of this syndrome are pleuropericardial inflammation, fever, rash, and arthritis. Serologic changes also occur. The clinical and serologic signs usually subside gradually after the offending drug is discontinued. A wide variety of drugs is implicated in this form of SLE.

Drugs Implicated as Activators of SLE

Drugs with proven association

Chlorpromazine
Hydralazine
Isoniazid
Methyldopa
Procainamide

Drugs with possible association

Beta blockers (e.g., acebutolol, atenolol, labetalol, metoprotolol, oxprenolol, pindolol, practolol, and propranolol)
Captopril
Carbamazine
Cimetidine
Diphenylhydantoin (phenytoin)
Ethosuximide
Methimazole
Penicillamine
Phenazine
Quinidine

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