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Kidney Week

Abstract: FR-PO1159

Donor-Derived Fibrillary Glomerulonephritis (FGN) in Mate-Kidney Allografts

Session Information

Category: Transplantation

  • 2002 Transplantation: Clinical

Authors

  • Brand, Kenneth, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, United States
  • Xu, Eric Jia Yi, Beth Israel Deaconess Medical Center, Boston, Massachusetts, United States
  • Chopra, Bhavna, Beth Israel Deaconess Medical Center, Boston, Massachusetts, United States
  • Lim, Mary Ann C., Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, United States
  • Henderson, Joel M., Beth Israel Deaconess Medical Center, Boston, Massachusetts, United States
  • Palmer, Matthew, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, United States
Introduction

FGN is a non-congophilic glomerular disease with randomly arranged fibrillary deposits measuring 12-22 nm. Accounting for < 1% of native kidney biopsies, its significance in donor kidneys is unclear. We report the first case of donor-derived FGN in a pair of mate kidney allografts.

Case Description

A 74-year-old male with prior kidney transplant (KT) from 1997, received a preemptive KT. He received thymoglobulin induction, followed by tacrolimus/mycophenolate/prednisone. At 1 month post-KT, suboptimal serum creatinine (sCr) of 5 mg/dL prompted a biopsy which showed no rejection and non-specific findings. After transitioning to belatacept, his sCr at 3 months post-KT remained suboptimal (3 mg/dL). Repeat biopsy showed fibrillary glomerular deposits positive for DNAJB9. Managed conservatively, his sCr is 1.8-2 mg/dL and urine protein-creatinine ratio (UPCR) is 0.2-0.5 g/g at 8 months post-KT.
A 56-year-old male with cirrhosis, hepatorenal syndrome, and IgA nephropathy received the mate kidney as part of simultaneous liver-kidney transplant. He received basiliximab induction, and tacrolimus/mycophenolate/prednisone maintenance. He had immediate liver allograft function, but required hemodialysis for 3 weeks, followed by sCr nadir of 1.4 mg/dL. A biopsy at 5 months post-KT for high sCr of 2.4 mg/dL showed no rejection but revealed DNAJB9-positive mesangial fibrils (Figure). Managed conservatively, his sCr at 8 months post-KT is 1.9-2.1 mg/dL, with UPCR 0.3-0.4 g/g.
The donor was a man in his 60’s, with ulcerative colitis and remote colon cancer history who was found unresponsive. His terminal sCr was 1.39 mg/dL and urinalysis showed +3 protein, 15-20 RBC/hpf.

Discussion

Although long term outcomes are yet to be determined, small observational studies suggest that long-term FGN recurrence in allografts can reach up to 21%, with minimal impact on kidney function. Our report suggests that donor-derived FGN can be conservatively managed without impact to allograft function.

Acknowledgment

Kenneth Brand, MD and Eric Xu, MD are both first authors on this abstract and contributed equally