Abstract

Thirty-six patients with the onset of symptoms of systemic lupus erythematosus before age 20 years (23 aged less than 15 years at onset) were studied during a 15-year period. All had clinical evidence of nephritis. They were followed for a mean of 5 years (range 6 months to 13 years) or until death. Survival was calculated to be 77% at 10 years for those aged less than 15, and 74% for those aged less than 20, from the onset of clinical nephritis. At referral, renal function was already impaired in two-thirds of patients. Renal biopsies showed mild focal or proliferative changes in 19% of patients, membranous lesions in 11%, and diffuse proliferative lesions in 70%. Three (8%) patients died during follow-up, all from sepsis, and 3 (8%) others required chronic haemodialysis for terminal renal failure. The prognosis even of severe lupus nephritis in childhood and adolescence has improved in recent years. Side effects of treatment remain an important cause of death and morbidity.