Abstract: PO2214
Donor-Derived Fibrillary Glomerulonephritis in a Renal Allograft
Session Information
- Transplantation: Clinical - Noninvasive Biomarkers, Immune Regulation, and Fascinomas
November 04, 2021 | Location: On-Demand, Virtual Only
Abstract Time: 10:00 AM - 12:00 PM
Category: Transplantation
- 1902 Transplantation: Clinical
Authors
- Jespersen, Tiana, University of California Davis, Sacramento, California, United States
- Huang, Yihung, University of California Davis, Sacramento, California, United States
- Alnimri, Muna, University of California Davis, Sacramento, California, United States
- Jen, Kuang-Yu, University of California Davis, Sacramento, California, United States
Introduction
Fibrillary glomerulonephritis (FGN) is a rare progressive renal disease that is defined by the presence of randomly oriented non-branching fibrils showing positive immunostaining for DnaJ homolog subfamily B member 9 (DNAJB9). Recurrent FGN in renal allografts have been described with an indolent course. We report a case of donor-derived FGN in a renal allograft.
Case Description
A 73-year-old female with history of end-stage renal disease (ESRD) due to anti-myeloperoxidase antibody-associated pauci-immune glomerulonephritis received a preemptive deceased donor renal transplant from a 62-year-old 79% Kidney Donor Profile Index female. The patient experienced slow graft function and nadir serum creatinine (Cr) of 1.7 mg/dL at 4 months post-transplant with subsequent Cr stabilizing in the 2.0-2.3 mg/dL range. Proteinuria mainly fluctuated between 1-2 g/g. Time-0 biopsy demonstrated mild mesangial widening/hypercellularity with rare glomerular capillary double contours. Ancillary studies revealed positive staining for DNAJB9 and kappa light chain-restriction, consistent with donor-derived FGN. 4-month surveillance biopsy showed similar findings. Background renal parenchyma showed moderate chronicity with prominent chronic vascular disease. At 10-months post-transplant, Cr remains at ~2 mg/dL and proteinuria remained in the 1-1.6 g/g range.
Discussion
FGN carries a poor prognosis with nearly half of patients progressing to ESRD within a few years. A case series of recurrent FGN after kidney transplantation suggests a relatively benign clinical course including a single report of donor-derived FGN (from a living related donor) without proteinuria in the recipient. Our case shows a more severely afflicted allograft that resulted in persistent low-grade but stable proteinuria in the recipient. Suboptimal Cr was also observed following transplant as well, although the allograft had other factors such as chronicity and chronic vascular disease.