Abstract: FR-PO761
Successful Kidney Transplant in a Patient with Overlap C3 Glomerulonephritis and Thrombotic Microangiopathy Treated with Ravulizumab
Session Information
- Post-Transplantation and Case Reports
November 03, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Transplantation
- 2102 Transplantation: Clinical
Authors
- Huidobro, Juan Pablo, Pontificia Universidad Catolica de Chile, Santiago, Chile
- Sepúlveda, Rodrigo, Pontificia Universidad Catolica de Chile, Santiago, Chile
- Carpio, Daniel, Universidad Austral de Chile, Valdivia, Los Ríos, Chile
- Majerson Grinberg, Alejandro, Pontificia Universidad Catolica de Chile, Santiago, Chile
- Jara, Aquiles, Pontificia Universidad Catolica de Chile, Santiago, Chile
Introduction
Overlap of C3 glomerulopathy (C3G) and thrombotic microangiopathy (TMA) has recently been acknowledged as one of the possible manifestations of a dysregulation of the alternative complement pathway. C3G of genetic cause has high risk of recurrence after kidney transplant (KT). Data of KT in patients with overlap C3G and TMA is scarce.
Case Description
A 30-y/o woman with kidney failure secondary to overlap C3G/TMA received a deceased-donor KT 6 years ago with primary graft failure secondary to early recurrence of C3G and TMA. Genetic study showed two pathogenic variants in complement-factor H. Post-KT PRA was 100%. While on dialysis she received immunoglobulin for desensitization. She then received a second deceased-donor KT. Flow cytometry (FC) crossmatch (XM) was negative. Ravulizumab, thymoglobulin and steroids were used for induction and five sessions of plasma exchange were performed (one pre-KT). She developed DGF requiring dialysis for 3-4 weeks after KT. Graft biopsy one-week post-KT revealed signs of ATN without signs of TMA or antibody-mediated rejection (Fig. 1), with negative C4d. DSA anti-HLA antibodies class I and II were positive and FC XM was positive (T and B). She was discharged on dialysis with tacrolimus, mycophenolate and prednisone. Four weeks after KT creatinine dropped to 2.9 mg/dL, leading to interruption of dialysis. Since then, allograft function has improved to creatinine of 1 mg/dL at month 3 post-KT.
Discussion
Overlap C3G and TMA is an infrequent condition with a high risk of recurrence. While C3G component secondary to a genetic cause has no treatment available, TMA can be successfully treated with complement inhibitors. This case shows a successful KT in a patient with overlap of C3G and TMA secondary to genetic cause and could therefore serve as guidance to the management of KT in these patients.