Primary focal segmental glomerulosclerosis in children: prognostic factors

To study the clinical course and the predictors of outcome in children and adolescents, 39 patients with nephrotic syndrome and primary focal segmenta l glomerulosclerosis (FSGS) were followed for a mean of 84.6 months. Thirty -six patients were treated with prednisone, either alone or in conjunction with cyclophosphamide. The clinical course was one of sustained remission i n 4 patients, frequent relapse in 13, persistent non-nephrotic proteinuria in 5, and persistent nephrotic syndrome in 17; 2 patients had stable renal failure and 8 had progressive renal failure, 5 of them evolving to end-stag e renal failure (ESRF). Resistance to prednisone was recorded in 76.6% of p atients. The use of cyclophosphamide plus prednisone was of benefit in 42.8 % of patients; 22.2% of the prednisone-resistant patients achieved remissio n of the nephrotic syndrome. A Kaplan-Meier analysis revealed a survival ra te of 92% after 5 years, 86% after 10 years, and 76% after 15 years. Using both univariate and multivariate, analysis, persistent nephrotic syndrome w as associated with progression to ESRF and the remission of proteinuria wit h maintenance of renal function. As the outcome of the nephrotic syndrome i n FSGS is significantly improved by remission of proteinuria, it is conceiv able that immunosuppressive medication may be used in conjunction with pred nisone in patients with steroid resistance.