Interventions for steroid-resistant nephrotic syndrome: a systematic review

In a systematic review and meta-analysis of randomized controlled trials (RCT), we aimed to evaluate the benefits and harms of all interventions for children with steroid-resistant nephrotic syndrome (SRNS). Nine RCTs involving 225 children were included. Cyclosporin when compared with placebo or no treatment significantly increased the number of children who achieved complete remission [3 trials, 49 children, relative risk (RR) for persistent nephrotic syndrome 0.64, 95% confidence intervals (CI), 0.47-0.88]. There was no significant difference in the number of children who achieved complete remission between oral cyclophosphamide with prednisone and prednisone alone [2 trials, 91 children, RR 1.01, 95% CI 0.74-1.36], between intravenous cyclophosphamide and oral cyclophosphamide [1 study, 11 children, RR 0.09, 95% CI 0.01-1.39], and between azathioprine with prednisone and prednisone alone [1 trial, 31 children, RR 1.01, 95% CI 0.77-1.32]. No RCTs were identified comparing combination regimens comprising high-dose steroids, alkylating agents or cyclosporin with single agents, placebo, or no treatment. Further adequately powered and well-designed RCTs are needed to confirm the efficacy of cyclosporin and to evaluate regimens of high-dose steroids with alkylating agents or cyclosporin for SRNS.