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Abstract: PO1860

An Atypical Case of Fibrillary Glomerulonephritis

Session Information

Category: Trainee Case Report

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Greco, Jessica M., The Ohio State University Wexner Medical Center, Columbus, Ohio, United States
  • Thakkar, Asish, Veterans Health Administration, Columbus, Ohio, United States
  • Cassol, Clarissa Araujo, Arkana Laboratories, Little Rock, Arkansas, United States
  • Satoskar, Anjali A., The Ohio State University Wexner Medical Center, Columbus, Ohio, United States
  • Almaani, Salem, The Ohio State University Wexner Medical Center, Columbus, Ohio, United States
Introduction

Fibrillary glomerulonephritis (FGN) is a rare glomerular disease with multiple disease associations such as hepatitis C, malignancy, and dysproteinemia. Despite these known associations, little is known about the interaction between FGN and other comorbidities. We present a case of FGN that presented years after successful treatment of hepatitis C infection (HCV).

Case Description

The patient is a 72 year old male with history of HCV. He was treated with ledipasvir/sofosbuvir and achieved a sustained virologic response however developed liver cirrhosis and underwent a liver transplant 2 years later. Histological examination of his liver explant was positive for hepatocellular carcinoma however he did not demonstrate any systemic involvement. 1 year after his transplant, he was noted to have a progressively worsening serum creatinine (1.37mg/dl from 0.97mg/dl) and new nephrotic range proteinuria (5.7 gram protein/gram creatinine). Autoimmune serology including ANCA, ANA, and monoclonal protein testing was negative. Complement levels were normal. He underwent a kidney biopsy which demonstrated focal endocapillary hypercellularity (figure 1a), segmental glomerular sclerosis, and mild mesangial and capillary wall staining for IgG, kappa, and lambda, with less C3. Electron microscopy demonstrated mesangial, subendothelial and few isolated subepithelial and intramembranous deposits with a vague fibrillar appearance. DNAJB9 staining was later performed and was positive, confirming a diagnosis of FGN (Figure 1b). After diagnosis of FGN, he underwent extensive malignancy screening which was unrevealing.

Discussion

FGN has a known association with HCV infection. Case series reported positive HCV serology in around 15% of patients with FGN, but none reported FGN after HCV eradication. DNAJB9 staining is specific for FGN and helps establish the diagnosis especially when histology does not demonstrate the classical membranoproliferative or mesangioproliferative patterns of glomerular injury.