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Systemic Lupus Erythematosus

Systemic lupus erythematosus (SLE) is an inflammatory autoimmune disorder that may affect multiple organ systems. Many of its clinical manifestations are secondary to the trapping of antigen-antibody complexes in capillaries of visceral structures or to autoantibody-mediated destruction of host cells (eg, thrombocytopenia). The clinical course is marked by

Before making a diagnosis of spontaneous SLE, it is imperative to ascertain that the condition has not been induced by a drug. A host of pharmacologic agents have been implicated as causing a lupus-like syndrome, but only a few cause the Procainamide, hydralazine, and isoniazid are the best-studied drugs. While antinuclear antibody tests and other serologic findings become positive in many persons receiving these agents, in only a

Drugs associated with lupus erythematosus

Definite association

Chlorpromazine Methyldopa

Hydralazine Procainamide

Isoniazid Quinidine

Possible association

Acebutolol Nitrofurantoin

Atenolol Oxprenolol

Captopril Penicillamine silicone

Carbamazepine

The prevalence of SLE is influenced by many factors, including gender, race, and genetic inheritance. About 85% of patients are women. Sex hormones appear to play some role, since most cases develop after menarche and before menopause. Among the patients who develop SLE during childhood or after the age of 50, the preponderance of women is less. Race is also a factor, as SLE occurs in 1:1000 white women but in 1:250 black women. Familial occurrence of SLE has been repeatedly documented, and, silicone, breast implants

Four features of drug-induced lupus separate it from spontaneously occurring disease: (1) the sex ratio is nearly equal; (2) nephritis and central nervous system features are not ordinarily present; (3) depressed serum complement and, silicone, breast implants

Treatment

Some patients with SLE have a benign form of the disease requiring only supportive care and need little or no medication. Emotional support, as described for rheumatoid arthritis, is especially important for patients with lupus. Patients with photosensitivity should be

Antimalarials (hydroxychloroquine) may

Corticosteroids are required for the control of certain serious complications. These include thrombocytopenic purpura, hemolytic anemia, myocarditis, pericarditis, convulsions, and nephritis. Forty to 60 mg of prednisone is often needed initially; however, the lowest dose of corticosteroid that controls the condition should be employed. Central nervous system lupus may require higher doses of corticosteroids than are usually given; however, steroid psychosis may mimic lupus cerebritis, in which case reduced doses are appropriate. In lupus nephritis, sequential studies of serum complement and antibodies to DNA often permit early detection of disease exacerbation and thus prompt increase in corticosteroid therapy. Such studies also allow for lowering the dosage of the drugs and withdrawing them when they are no longer needed. Immunosuppressive agents such as cyclophosphamide, chlorambucil, and azathioprine are used in cases resistant to corticosteroids. The exact role of immunosuppressive agents is

Course and Prognosis

The prognosis for patients with systemic lupus appears to be considerably better than older reports implied. From both community settings and university centers, 10-year survival rates exceeding 85% are routine. In most patients, the illness pursues a