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Kidney Week

Abstract: TH-PO728

A Case of Fibrillary Glomerulonephritis Presenting as Rapidly Progression Glomerulonephritis in a Patient with Untreated Hepatitis C

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Kaisani, Aadil, Baylor Scott and White Central Texas, Temple, Texas, United States
  • Mahgoub, Mohammed, Baylor Scott and White Central Texas, Temple, Texas, United States
  • Sayeed Khan, Mohammed Ahmed, Baylor Scott and White Central Texas, Temple, Texas, United States
  • Goraya, Nimrit, Baylor Scott and White Central Texas, Temple, Texas, United States
Introduction

Fibrillary glomerulonephritis (FGN) is a rare progressive disease accounting for 0.5% to 1.4% of all native kidney biopsies. The diagnosis is established with renal biopsy demonstrating randomly oriented fibrils ranging 15-25 nm on electron microscopy, and negative Congo red staining. Recent discovery of DNAJB9 as a biomarker has been reliable for diagnosis, although its role in pathogenesis remains unclear. It is strongly associated with underlying malignancy, monoclonal gammopathy, autoimmune disease or infections. Prognosis remains poor with no established clinical guidelines to optimize therapy. Here, we describe a case of FGN presenting with rapidly progressive glomerulonephritis and crescents on biopsy in a patient with untreated Hepatitis C.

Case Description

A 64 yo. M with significant past medical history of hypertension, cirrhosis secondary to untreated Hepatitis C and methamphetamine who presented with dyspnea. Initial work up showed AKI with creatinine of 4.52 mg/dL. Urinalysis displayed hematuria and UPCR of 7.04 g/g, raising suspicion for an underlying GN. Serological work including anti-GBM, ANCA, Cryoglobulin, RF, HIV, Hepatitis A and B were negative. Hepatitis C antibody was positive, with quantitative RNA greater than 300,000 IU/mL. Electron microscopy from biopsy revealed randomly oriented fibrils of ~ 15nm in diameter throughout the glomeruli. IF revealed IgG, C3, kappa/lambda light chains in the mesangium and along the GBM. IHC analysis showed negative Congo red and positive DNAJB9. Of the 46 glomeruli analyzed, 7 demonstrated cellular and fibrocellular crescents. Biopsy findings were consistent with FGN. He was started on pulse dose steroids followed by a taper and cyclophosphamide. Due to worsening renal function and volume overloaded state, he required initiation of dialysis. Despite starting treatment for Hepatitis C, there has been no evidence of renal recovery.

Discussion

Overall prognosis for FGN remains poor with approximately 40-50% of patients progressing to ESRD. Our case highlights the challenges with recognition and management of FGN as a cause of RPGN since clinical presentation is similar to other recognized etiologies. Despite treatment of RPGN and Hepatitis C, our patient had progression of disease emphasizing need for further trials and novel therapies.