Abstract: SA-PO865
Recurrence of C3 Deficiency Related Membranoproliferative Glomerulonephritis After Kidney Transplantation
Session Information
- Transplantation: Clinical - Case Reports
November 05, 2022 | Location: Exhibit Hall, Orange County Convention Center‚ West Building
Abstract Time: 10:00 AM - 12:00 PM
Category: Transplantation
- 2002 Transplantation: Clinical
Authors
- Bernardo, João Filipe, Centro Hospitalar Universitario Lisboa Norte EPE, Lisboa, Lisboa, Portugal
- Gonçalves, Sara, Centro Hospitalar Universitario Lisboa Norte EPE, Lisboa, Lisboa, Portugal
- Godinho, Iolanda, Centro Hospitalar Universitario Lisboa Norte EPE, Lisboa, Lisboa, Portugal
- Abreu, Fernando, Centro Hospitalar Universitario Lisboa Norte EPE, Lisboa, Lisboa, Portugal
- Neves, Marta R.A., Centro Hospitalar Universitario Lisboa Norte EPE, Lisboa, Lisboa, Portugal
- Melo, Maria Joao, Centro Hospitalar Universitario Lisboa Norte EPE, Lisboa, Lisboa, Portugal
- Goncalves, Joao Albuquerque, Centro Hospitalar Universitario Lisboa Norte EPE, Lisboa, Lisboa, Portugal
- Santana, Alice, Centro Hospitalar Universitario Lisboa Norte EPE, Lisboa, Lisboa, Portugal
- Lopes, Jose António, Centro Hospitalar Universitario Lisboa Norte EPE, Lisboa, Lisboa, Portugal
Introduction
C3 deficiency is hereditary disease leading to ineffective opsonization and defective complement lytic activity, increasing infection susceptibility. Affected patients may develop an immune-complex mediated membranoproliferative glomerulonephritis (MPGN) due to inadequate immune complexes clearance. Theoretically possible, the MPGN disease recurrence in the kidney graft of C3 deficient patients has never been described and its impact on graft survival is unknown.
Case Description
29-year-old caucasian male with complete absence of C3 factor due to a homozygous loss function mutation. He had a history of frequent respiratory infections and one episode of meningitis in childhood. At the age of 20 he presented hematuria, proteinuria (1,3g/24h) and elevated serum creatinine (pCr 2,3mg/dL). Kidney biopsy were compatible with MPGN secondary to IC deposition. He rapidly progressed to end stage kidney disease, starting haemodialysis at the age of 22, and was submitted to a kidney transplant five years later: Maastricht II cardiac death donor with 6 HLA mismatches, no donor specific antibodies (DSA) and induction therapy was thymoglobulin. Despite delayed graft function, one month after discharge the patient had a serum creatinine of 2,1mg/dL. There were no infectious complications but 18 months after transplantation he developed microscopic hematuria, proteinuria (4,1g/24h) and progressive kidney allograft dysfunction without DSA, without cytomegalovirus or polyoma virus infection. Kidney allograft biopsy revealed lobulated glomeruli, thickening of the capillary wall, endocapillary cellularity, mesangial expansion, “full house” pattern (deposition of IgG, IgA, IgM, C1q and C4 in the mesangium and capillary wall) and no C3. Based on this, we assumed a recurrence of IC mediated MPGN secondary to C3 deficiency. Despite initiation of antiproteinuric therapy, graft function deteriorated and nine months after he was started on hemodialysis.
Discussion
MPGN recurrence after kidney transplantation of C3 deficient patients seems to occur early and graft survival is considerably shorter. As C3 is mainly synthesized in liver, combined liver-kidney transplantation maybe a better option, in order to restore C3 plasma circulating pool and prevent MPGN recurrence.