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Clinical practice recommendations for recurrence of focal and segmental glomerulosclerosis/steroid-resistant

Lutz T Weber1, Burkhard Tönshoff2, Ryszard Grenda3

  • 1Faculty of Medicine, University Hospital of Cologne, Children's and Adolescents' Hospital, Pediatric Nephrology, University of Cologne, Cologne, Germany.

Pediatric Transplantation
|December 30, 2020
PubMed

Insights

Recurrence of primary kidney disease, specifically Focal Segmental Glomerulosclerosis/Steroid-Resistant Nephrotic Syndrome (FSGS/SRNS), poses a high risk for graft loss after pediatric kidney transplantation (RTx). Early plasma exchange or immunoadsorption with specific immunosuppressants shows promise for remission.

Area of Science:

  • Nephrology
  • Transplantation Immunology
  • Pediatric Nephrology

Background:

  • Recurrence of primary kidney disease is a significant threat to allograft survival post-pediatric kidney transplantation (RTx).
  • Focal Segmental Glomerulosclerosis/Steroid-Resistant Nephrotic Syndrome (FSGS/SRNS) recurrence can occur in up to 86% of idiopathic pediatric cases, necessitating clear management guidelines.

Framework:

  • The CERTAIN study group conducted a PICO-based literature review to develop evidence-based statements for managing FSGS/SRNS recurrence after pediatric RTx.
  • Consensus recommendations were formulated through rigorous evaluation of existing evidence and expert panel discussions.

Implementation:

  • Routine nephrectomy before transplantation is not recommended.
  • Discouraging living donation due to recurrence risk is not advised.
  • Special consideration is warranted for patients with prior graft loss from FSGS/SRNS recurrence.

Implications:

  • Early plasma exchange (PE) or immunoadsorption (IA), potentially combined with high-dose Cyclosporine A (CsA) and/or rituximab, appears most effective for inducing remission.
  • FSGS/SRNS recurrence significantly impacts graft survival and can lead to graft loss.
  • Recommendations must account for variability in patient populations, pre-transplant history, immunosuppression, and recurrence definitions.

Keywords:
childrenfocal-segmental glomerulosclerosisrecurrencerenal transplantationsteroid-resistant nephrotic syndrome

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