Morbidity associated with childhood systemic lupus erythematosus.

A complete analysis of the outcome of childhood onset systemic lupus erythematosus (SLE) requires determination of survival statistics and consideration of disease and treatment morbidity. The course of 32 patients with SLE diagnosed at or prior to age 16 and followed from 1979-1988 was analyzed with emphasis on the morbidity of pediatric SLE. Clinical characteristics were similar to those in previous studies. Five year survival, calculated by life-table analysis, was 85.3%, comparable to recent studies. Treatment consisted of corticosteroids (32 patients), antimalarials (14), cyclophosphamide (9) or azathioprine (2). Eighty-four percent of patients experienced a noninfectious drug related complication. A major infection contributed in all 5 patients who died. Higher total corticosteroid dose was associated with more severe infections. Eighty-eight percent of patients were left with chronic organ dysfunction. Survival in childhood onset SLE has improved, but morbidity remains a significant problem from organ dysfunction and complications of therapy. Our study is the first to address the issue of morbidity in childhood SLE.