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 laborato ry data, and age at disease onset in systemic lupus erythematosus patients aged 16 years or younger at disease onset. Patients and methods. The medica l records of patients seen at the Clinics Hospital, State University of Cam pinas, Sao Paulo, Brazil. between 1979 and 1995 were reviewed retrospective ly. All 59 included patients (48F/11M) fulfilled four or more American Coll ege of Rheumatology criteria for systemic lupus erythematosus, Patients wit h discoid, drug-induced or neonatal lupus, or other systemic connective tis sue diseases were excluded. Patients were studied individually then classif ied in three groups based on age at disease onset, as follows: Group I, les s than or equal to 9 years of age; Group II, 10-14 years of age; and Group III, 15-16 years of age. Clinical and laboratory abnormalities and mortalit y were compared in the three groups. Results. The most frequent clinical ma nifestations were joint symptoms (91.5%), renal involvement (71.1%), malar rash (61%), alopecia (61%), fever (59.3%) and photosensitivity (52.5%). Lab oratory 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 infec tion in eight patients (57.1% of decedents), central nervous system disease in five (35.7%), and renal insufficiency in one (7.2%), Disease onset befo re 15 years of age (P=0.026), renal involvement (P=0.03), and arterial hype rtension (P=0.002) were predictive of mortality. Mortality was not influenc ed by gender or race.