ISN-Cochrane Webinar: Interventions for idiopathic steroid‐resistant nephrotic syndrome in children

A subset of children presenting with nephrotic syndrome do not respond to steroids <4 weeks (average 12%) and is termed steroid-resistant nephrotic syndrome (SRNS). This is an updated Cochrane systematic review of randomised controlled trials of interventions for idiopathic SRNS in children. Twenty-nine studies (1248 participants) were identified. Most studies were small and of poor quality. Cyclosporin may increase rates of complete remission compared to placebo (RR 3.50, 95% CI 1.09-11.20) or IV cyclophosphamide (RR 3.43, 1.84-6.41) but effects on kidney failure were uncertain/not reported.  

Learning objectives:

- To evaluate the benefits and harms of different interventions used in children with idiopathic nephrotic syndrome, who do not achieve remission following four weeks or more of daily corticosteroid therapy
- How do the findings of this review inform clinical management of steroid resistant nephrotic syndrome in children?
- What are the implications for future research in steroid resistant nephrotic syndrome in children?

Further reading:

• Trautmann A, Vivarelli M, Samuel S, Gipson D, Sinha A, Schaefer F, et al, International Pediatric Nephrology Association. IPNA clinical practice recommendations for the diagnosis and management of children with steroid-resistant nephrotic syndrome. Pediatric Nephrology 2020;35(8):1529-61. 
• Kidney Disease: Improving Global Outcomes (KDIGO) Glomerular Diseases Work Group. KDIGO 2021 Clinical Practice Guideline for the Management of Glomerular Diseases. Kidney International 2021;100(45):Chapter 4: Nephrotic syndrome in children. 
• Tullus K, Webb H, Bagga A. Management of steroid-resistant nephrotic syndrome in children and adolescents. The Lancet Child & Adolescent Health. 2018 Dec 1;2(12):880-90. 
• Trautmann A, Bodria M, Ozaltin F, Gheisari A, Melk A, Azocar M, et al. Spectrum of steroid-resistant and congenital nephrotic syndrome in children: the PodoNet registry cohort. Clinical Journal of The American Society of Nephrology: CJASN 2015;10(4):592-600Inaba A, Hamasaki Y, Ishikura K, Hamada R, Sakai T, Hataya H, et al. Long-term outcome of idiopathic steroid-resistant nephrotic syndrome in children. Pediatric Nephrology 2016;31(3):425-34 
• Trautmann A, Schnaidt S, Lipska-Ziętkiewicz BS, Bodria M, Ozaltin F, Emma F, et al. Long-term outcome of steroid-resistant nephrotic syndrome in children. Journal of the American Society of Nephrology 2017;28(10):3055–65 
• Ding WY, Koziell A, McCarthy HJ, Bierzynska A, Bhagavatula MK, Dudley JA, et al. Initial steroid sensitivity in children with steroid-resistant nephrotic syndrome predicts post-transplant recurrence. Journal of the American Society of Nephrology 2014;25(6):1342–8. 
• Morello W, Proverbio E, Puccio G, Montini G. A systematic review and meta-analysis of the rate and risk factors for post-transplant disease recurrence in children with steroid resistant nephrotic syndrome. Kidney International Reports 2023;8:254-64. 
• Gulati A, Sinha A, Jordan SC, Hari P, Dinda AK, Sharma S, Srivastava RN, Moudgil A, Bagga A. Efficacy and safety of treatment with rituximab for difficult steroid-resistant and-dependent nephrotic syndrome: multicentric report. Clinical Journal of the American Society of Nephrology. 2010 Dec 1;5(12):2207-12.