Interventions for steroid-resistant nephrotic syndrome: a systematic review

Doaa Habashy1, Elisabeth M Hodson, Jonathan C Craig

  • 1Center for Kidney Research and Cochrane Renal Group, NHMRC Center for Clinical Research Excellence, The Children's Hospital at Westmead, NSW 2145 Westmead, Sydney, Australia.

Insights

Cyclosporin effectively treats steroid-resistant nephrotic syndrome (SRNS) in children, increasing remission rates compared to placebo. Other treatments showed no significant benefit, highlighting the need for further research on combination therapies for SRNS.

Area of Science:

  • Pediatric Nephrology
  • Clinical Pharmacology
  • Systematic Reviews

Background:

  • Steroid-resistant nephrotic syndrome (SRNS) presents a significant therapeutic challenge in pediatric kidney disease.
  • Limited evidence exists regarding the comparative efficacy and safety of various treatment modalities for SRNS.

Purpose of the Study:

  • To systematically review and meta-analyze randomized controlled trials (RCTs) evaluating interventions for pediatric SRNS.
  • To assess the benefits and harms of different treatment regimens in children with SRNS.

Main Methods:

  • Systematic review and meta-analysis of nine RCTs involving 225 children.
  • Data synthesis using relative risk (RR) and 95% confidence intervals (CI) to compare treatment outcomes.

Main Results:

  • Cyclosporin significantly increased complete remission rates compared to placebo or no treatment (RR 0.64, 95% CI 0.47-0.88).
  • No significant differences in remission rates were observed for oral cyclophosphamide vs. prednisone, IV vs. oral cyclophosphamide, or azathioprine vs. prednisone.
  • No RCTs compared combination regimens (high-dose steroids with alkylating agents or cyclosporin) against single agents or placebo.

Conclusions:

  • Cyclosporin demonstrates efficacy in achieving remission in pediatric SRNS.
  • Current evidence does not support the superiority of other tested single-agent therapies over standard care.
  • Further high-quality RCTs are required to confirm cyclosporin's efficacy and explore combination therapies for SRNS.

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