Young age at onset, renal involvement, and arterial hypertension are of adverse prognostic significance in juvenile systemic lupus erythematosus.

Objective: To look for associations between mortality, clinical or laboratory data, and age at disease onset in systemic lupus erythematosus patients aged 16 years or younger at disease onset.

Patients and methods: The medical records of patients seen at the Clinics Hospital, State University of Campinas, São Paulo, Brazil, between 1979 and 1995 were reviewed retrospectively. All 59 included patients (48F/11M) fulfilled four or more American College of Rheumatology criteria for systemic lupus erythematosus. Patients with discold, drug-induced or neonatal lupus, or other systemic connective tissue diseases were excluded. Patients were studied individually then classified in three groups based on age at disease onset, as follows: Group I, < or = 9 years of age; Group II, 10-14 years of age; and Group III, 15-16 years of age. Clinical and laboratory abnormalities and mortality were compared in the three groups.

Results: The most frequent clinical manifestations were joint symptoms (91.5%), renal involvement (71.1%), malar rash (61%), alopecia (61%), fever (59.3%) and photosensitivity (52.5%). Laboratory findings included antinuclear antibody in 94.9% of cases. LE cells in 71.1%, low serum complement in 65.3%, anti-DNA in 63.4%, hematuria in 62.7%, and proteinuria in 61%. The mortality rate was 23.7% (9F/5M) overall, 18.7% in females, and 45.4% in males (P = 0.07). The cause of death was infection in eight patients (57.1% of decedents), central nervous system disease in five (35.7%), and renal insufficiency in one (7.2%). Disease onset before 15 years of age (P = 0.026), renal involvement (P = 0.03), and arterial hypertension (P = 0.002) were predictive of mortality. Mortality was not influenced by gender or race.