Immunosuppressive Therapy in Children With Steroid-Resistant Nephrotic Syndrome

Abstract

Background: Treatment of steroid-resistant nephrotic syndrome (SRNS) remains a huge challenge in pediatric patients. Immunosuppressive agents including cyclosporine A (CsA), tacrolimus (TAC) and mycophenolate mofetil (MMF) are recommended for the management of children with SRNS. The aim of this study was to compare the efficacy of CsA, TAC and MMF in children with SRNS and provide guidance for clinical practice. Methods: This is a retrospective analysis of the records of 70 children with SRNS recruited from a children hospital over a period of seven years. They were treated with CsA, TAC and MMF as initial immunosuppressive therapy in addition to steroids. Complete or partial remission was considered a good response. Results: Five (41.7%) of 12 children who were on CsA therapy achieved remission at 6 months and 5 (41.7%) at 12 months. Nine (19.1%) of 47 patients treated with TAC achieved remission at 6 months, 20 (42.6%) at 12 months and 6 (12.8%) within or over 24 months. The remission achieved at 6 months and 12 months was 4 (36.4%) and 2 (18.2%) respectively in MMF group. The relapse rates in children who had achieved remission were 30.0%, 45.7% and 50.0% in CsA, TAC and MMF group respectively. Conclusions: Based on similar baseline characteristics, CsA and TAC as initial therapy for SRNS have a better remission and relapse rates whereas MMF shows a rapid remission effect.

Immunosuppressive therapy in children with primary nephrotic syndrome: single center experience, Karachi, Pakistan

“…It was noted that MMF was more effective in FR/SD rather than SRNS (95% vs 66%) but not better than CS. More or less similar results have been reported by others [18, 25–27]. However, 50% of children required addition of MMF but there was no difference in response (91% vs 87%).…”

Long-term therapy with cyclosporine and alternate-day prednisone in patients with idiopathic Steroid Resistant Nephrotic Syndrome (SRNS)

“…In recent years many studies came up confirming our observations. Li J et al concluded that CsA and Tac as initial therapy for SRNS have a better remission and relapse rate [55]. Genetic screening should be done prior to therapy in familial SRNS and ideally in sporadic SRNS also with initial resistance.…”