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Efficacy of Calcineurin Inhibition in Children With Steroid-Resistant Nephrotic Syndrome

Agnes Trautmann1, Jonas Hofstetter1, Beata Lipska-Ziętkiewicz2

  • 1Division of Pediatric Nephrology, University Center for Pediatrics and Adolescent Medicine, Heidelberg, Germany.

Kidney International Reports
|October 27, 2025
PubMed

Insights

Calcineurin inhibitor (CNI) treatment effectively reduces proteinuria in children with steroid-resistant nephrotic syndrome (SRNS), especially in non-genetic forms. However, sustained remission is challenging, highlighting the need for careful CNI management in pediatric SRNS.

Area of Science:

  • Pediatric Nephrology
  • Pharmacology
  • Immunology

Background:

  • Steroid-resistant nephrotic syndrome (SRNS) poses significant challenges in pediatric kidney disease management.
  • Calcineurin inhibitors (CNIs) are a cornerstone treatment for SRNS, but their long-term efficacy and safety profiles require further elucidation, particularly in diverse genetic subtypes.
  • Understanding CNI treatment dynamics is crucial for optimizing outcomes in children with SRNS.

Purpose of the Study:

  • To evaluate the efficacy and long-term impact of calcineurin inhibitor (CNI) treatment in children with steroid-resistant nephrotic syndrome (SRNS).
  • To analyze remission rates, kidney survival, and the influence of CNI dosage and trough levels in both genetic and non-genetic forms of SRNS.
  • To identify predictors of CNI responsiveness and assess the risk of breakthrough proteinuria and relapse.

Main Methods:

  • A cohort of 278 children with SRNS receiving first-line CNI treatment was analyzed.
  • Competing risk analysis, Kaplan-Meier, and Cox regression were used to estimate remission, kidney failure, and survival.
  • Multivariable linear-mixed effects modeling assessed the impact of CNI dosage and trough levels on proteinuria.

Main Results:

  • CNI treatment significantly reduced proteinuria in non-genetic SRNS (84%) and genetic SRNS (58%), with higher CNI doses correlating with proteinuria reduction.
  • Sustained remission was achieved in 24% of non-genetic SRNS cases and 3% of genetic SRNS cases.
  • Kidney survival at 15 years was notably higher in CNI-responsive non-genetic SRNS children (92%) compared to non-responsive ones (42%).

Conclusions:

  • CNI therapy demonstrates real-world efficacy in reducing proteinuria in pediatric SRNS, particularly in non-genetic forms.
  • Long-term remission remains a challenge, with significant risks of breakthrough proteinuria and post-discontinuation relapse.
  • The study underscores the importance of CNI dose-response relationships and provides critical insights into long-term outcomes for genetic and non-genetic SRNS.
Abstract

Keywords:
calcineurin inhibitorgenetic kidney diseasenephrotic syndromesteroid resistance

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