Abstract:The long-term outcome in rapidly progressive glomerulonephritis (RPGN) is a subject of increasing clinical attention. We performed a retrospective study of 64 patients, who were treated between 1972 and 1990 for biopsy-confirmed RPGN (median observation time 3.3 years). The incidence of RPGN displayed a linear increase with age, and 41 percent of the patients were older than 60 years (26/64). Fifty-one of the 64 patients (80%) were treated with immunosuppressives (steroid pulses, cyclophosphamide, azathioprine, prednisolone, plasma exchange). Of the 13 patients not receiving immunosuppresion, 12 were diagnosed as cases of "idiopathic" RPGN. Anti-neutrophil cytoplasmatic antibodies (ANCA) were tested for in 6 of the 64 patients, of whom 2 with systemic immune disease were cANCA positive. In the Kaplan-Meier analysis, the overall 5-year patient survival rate with the 95 percent confidence interval [95% CI] was 70 percent [47%-93%] and did not differ for immunosuppressed and nonspecifically treated patients. Kaplan-Meier probability of life-sustaining renal function was significantly better in 51 immunosuppressed patients (p = 0.03) compared to 13 nonspecifically treated patients, and the efficacy of immunosuppression in patients older than 60 years was comparable to that in younger patients. After 5-years, the proportion of patients with maintained renal function was only 27 percent [0%-57%] in the immunousppressed patients. From the multivariate Cox model, it was evident that immunosuppression had no independent beneficial effect on renal function, whereas the 20 patients with initial oliguria (< 500 ml/d) bad a significantly increased relative risk of 2.0 of losing renal function [1.1-3.6] compared to those without oliguria. In the Cox model, success of immunosuppression was independent of age, but the relative risk of death was at 5.3 significantly higher in patients over 60 years of age [2.0-13.9] compared to those younger than 60 years. Within the observation period, 24 patients died, 10 due to complications of immunosuppression (4 infections, 6 malignancies). We conclude that intensive immunosuppression should be given to all patients with RPGN irrespective of patient's age, but long-term efficacy is limited, complication rate is high, and initial oliguria is the most significant risk factor for loss of renal function.