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Kidney Week

Abstract: PO2213

Early Recurrence of Fibrillary Glomerulonephritis After Kidney Transplantation

Session Information

Category: Transplantation

  • 1902 Transplantation: Clinical

Authors

  • Niazi, Nicholas S., The University of Texas Health Science Center at San Antonio, San Antonio, Texas, United States
  • Ding, Yanli, The University of Texas Health Science Center at San Antonio, San Antonio, Texas, United States
  • Bhayana, Suverta, The University of Texas Health Science Center at San Antonio, San Antonio, Texas, United States
Introduction

Fibrillary glomerulonephritis (FGN) is a rare glomerulonephritis characterized by glomerular congo red negative nonbranching fibrils on electron microscopy (EM). The optimal treatment for FGN is unclear and the renal prognosis is poor, with up to half of patients progressing to end-stage kidney disease (ESKD) by four years. Kidney transplantation has a variable recurrence rate after transplant ranging from 9 to 50%.

Case Description

A 57-year-old-male with a history of cirrhosis secondary to nonalcoholic fatty liver disease and ESKD secondary to FGN was hospitalized for acute kidney injury. The patient underwent simultaneous liver and kidney transplantation four weeks prior to presentation. The patient underwent induction with basiliximab and started on tacrolimus, mycophenolate mofetil, and prednisone for maintenance immunosuppression. The patient’s serum creatinine had increased from the previous nadir of 2.0 mg/dL to 2.7 mg/dL. Given worsening allograft function, a transplant kidney biopsy was performed. The patient’s biopsy showed evidence of recurrent fibrillary glomerulonephritis, including positive immunohistochemical staining for DNAJB9 and ultrastructural findings of mesangial nonbranching fibrils averaging 21.5 nm in diameter. The biopsy also showed acute tubular necrosis, secondary global and focal segmental glomerulosclerosis, and tubular atrophy and interstitial fibrosis in 20-30% of the cortex. There was no evidence of acute T-cell mediated rejection or antibody-mediated rejection. The patient’s allograft function improved and ranged from 1.5 -1.7 mg/dL at the time of discharge. The patient is now six months from transplant and has stable allograft function and minimal proteinuria.

Discussion

FGN recurrence after kidney transplantation has been described in case reports and case studies. In the most recent and largest study, utilizing DNAJB9 and protocolized post-transplant biopsy, the authors showed a recurrence rate of 21% and a median time to recurrence of 10.2 years. Additionally, all biopsies before five years were negative in their cohort. Allograft failure was seen in 33% of patients with recurrent FGN. To our knowledge, this case is the earliest reported recurrence of FGN after transplantation. In patients with a history of FGN, recurrent disease should be considered in the differential of early allograft dysfunction.