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Abstract: SA-PO1131

Success of Intensive Immunosuppression to Prevent Post-Transplant Recurrence of C3 Glomerulonephritis in Children

Session Information

Category: Trainee Case Report

  • 1902 Transplantation: Clinical

Authors

  • Colliou, Eloise, CHU Toulouse, Toulouse, France
  • Maisin, Anne Francoise, APHP, Robert-Debre Hospital, Paris, France
  • Macher, Marie-Alice, APHP, Robert-Debre Hospital, Paris, France
  • Kwon, Theresa, APHP, Robert-Debre Hospital, Paris, France
  • Dossier, Claire, APHP, Robert-Debre Hospital, Paris, France
  • Vrillon, Isabelle, University Hospital of Nancy, Nancy, France
  • Fremeaux-Bacchi, Veronique, APHP, HEGP, Paris, France
  • Deschênes, Georges, APHP, Robert-Debre Hospital, Paris, France
Introduction

C3 glomérulonephritis (C3GN) is characterized by the dysregulation of complement alternative pathway and is frequently associated with autoantibodies stabilizing the C3 or C5 convertase. There is currently no specific treatment and the renal prognosis is poor with a high risk of early recurrence post-transplant, leading to graft loss in more than 50% of cases, despite the use of Eculizumab.

Case Description

Two patients received a living donor transplant and one a deceased donor. Bi-nephrectomy was performed 5 +/-2 months before transplantation. Steroids (60mg/m2/d) and MMF (1500mg/m2/d) were started at least 1 month prior to transplant. Eculizumab was started 14 days before transplant in the 2 patients with a living donor and the 3rd patient was on long-term treatment. One session of immunoadsorption was performed 8h prior the renal graft for all. They received an induction by thymoglobulin or Basiliximab followed by Tacrolimus [T0 : 8-10 ng/ml], associated with MMF [AUC 50-60 µg.h.ml], steroids and Eculizumab. No early recurrence occurred. Protocolar renal biopsies at 3 months only showed C3 deposits in 1 patient without any inflammation. After a follow-up of 3, 6 and 15 months, none of the patients developed proteinuria or hematuria under Eculizumab. One patient developed a BK virus replication (4.8 log) at M4 that led us to decrease immunosuppression, this was complicated with acute cellular rejection (1B) at M5. eGFR at last follow-up was 55 ml/min/1,73m2 in this patient and 80-86 ml/min/1,73m2 in the two other patients.

Discussion

Intensive immunosuppression pre and post-transplant may prevent post-transplant recurrence of C3GN.