Brief Report

Pediatric Nephrology

, Volume 21, Issue 10, pp 1476-1479

First online:

Recurrence of proteinuria 10 years post-transplant in NPHS2-associated focal segmental glomerulosclerosis after conversion from cyclosporin A to sirolimus

  • Britta HöckerAffiliated withDivision of Pediatric Nephrology, University Children’s Hospital Email author 
  • , Tanja KnüppelAffiliated withDivision of Pediatric Nephrology, University Children’s Hospital
  • , Rüdiger WaldherrAffiliated withGroup Practice of Pathology
  • , Franz SchaeferAffiliated withDivision of Pediatric Nephrology, University Children’s Hospital
  • , Stefanie WeberAffiliated withDivision of Pediatric Nephrology, University Children’s Hospital
  • , Burkhard TönshoffAffiliated withDivision of Pediatric Nephrology, University Children’s Hospital

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Abstract

Mutations in the NPHS2 gene, which encodes podocin, are associated with steroid-resistant nephrotic syndrome in childhood. Renal histology frequently presents focal segmental glomerulosclerosis (FSGS). Post-transplant recurrence of proteinuria in patients affected by homozygous or compound heterozygous NPHS2 mutation is encountered rarely (1–2%) compared to 30% recurrence in nonhereditary FSGS. We report on a pediatric kidney transplant recipient with NPHS2-associated nephrotic syndrome and FSGS, who developed biopsy-proven recurrence of FSGS 10 years post-transplant in temporal association with conversion from cyclosporin A (CsA)- to sirolimus (SRL)-based immunosuppression, due to histological evidence of severe CsA-induced nephrotoxicity. Reswitch of the immunosuppressive regimen from SRL to CsA led to a noticeable decrease of proteinuria and to stabilization of graft function. We conclude that patients with hereditary FSGS are not entirely protected from post-transplant recurrence of proteinuria, even in the long term. The close temporal relationship of FSGS recurrence with CsA withdrawal and conversion to SRL suggests that caution should be exercised in the use of CsA-free immunosuppression also in patients with NPHS2-associated FSGS.

Keywords

Focal segmental glomerulosclerosis NPHS2 gene Cyclosporin A Sirolimus Podocin Nephrotic syndrome Proteinuria