The purpose of this study was to establish the actual survival rate of a cohort of 51 patients with systemic lupus erythematosus (SLE) all of whom were followed during 12 years from 1976 to 1988 and to evaluate the prognostic significance of the severe complications of the disease. Forty-eight were females. The mean age was 28.5 at onset, 36.2 at diagnosis and 42 at entry; 80% had four or more 1982 ARA criteria at diagnosis and 96% in 1988. Survival rates were: 96% at two years, 86.3% at five years and 74.5% at twelve years. The main cause of death was infection (62%); SLE was directly responsible in only one case. Manifestations considered as severe occurred in almost two-thirds of the cases without any concordance in the time sufficient to recognize different severe forms of the disease.