Systemic lupus erythematosus in the first two decades of life

Seventy patients with systemic lupus erythematosus (SLE) in the first two decades of life have been followed from 1958 to 1981. The patients were treated with corticosteroids alone (17 patients) or corticosteroids and immunosuppressives (53 patients). Twenty-one patients (30%) had evidence of CNS lupus. Eighty-seven percent of patients had evidence of renal involvement, and 34% of these patients had diffuse proliferative lupus nephritis (DPLN). A high frequency of pathologic transition was found to occur. Analysis of 20 pregnancies in 12 women with SLE reveals that patients with active SLE and/or renal disease are at a high risk for complications of pregnancy in contrast to patients in remission. The occurrence of infection, aseptic necrosis and cataracts is shown to be related to SLE itself and to its treatment. By life table analysis overall survival was 90% at 1 yr, 85% at 10 yr. and 77% at 15 yr. Survival of patients who entered the study before age 16 and of patients with CNS lupus could not be distinguished from that of the total group. Patients who entered the study from 1974-1981 had 5-yr survival similar to that of the total group. Survival of 21 patients with DPLN did not deviate from that of the entire group until 14 yr after study entry (7 yr if dialysis and transplantation are considered as deaths). In conclusion, the prognosis of SLE and especially of DPLN in the younger patient appears to be better than indicated by prior reports. Thus, analysis of outcome in this disease may require a long duration of follow-up.