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Kidney Week

Abstract: TH-PO715

Fibrillary Glomerulonephritis with Concurrent Anti-glomerular Basement Membrane Leading to Rapidly Progressive Glomerulonephritis: A Diagnostic Conundrum

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

  • Bell, Abraham, Florida State University, Tallahassee, Florida, United States
  • Maddineni, Gautam, Florida State University, Tallahassee, Florida, United States
  • Milutinovic, Stefan, Florida State University, Tallahassee, Florida, United States
  • Bell, Isaac, Nova Southeastern University, Fort Lauderdale, Florida, United States
  • Khambati, Ibrahim, Florida State University, Tallahassee, Florida, United States
Introduction

Anti–glomerular basement membrane (anti-GBM) disease is a rare small vessel vasculitis that affects the capillary beds of the kidneys and lungs. It is a well-known significant cause of rapidly progressive glomerulonephritis (RPGN). Conversely, fibrillary glomerulonephritis (FGN) typically presents proteinuria, hematuria and more gradual decline in renal function as compared to a rapid one. Herein, we report a case of anti-GBM disease with finding of FGN on renal biopsy; a rare presentation of both entities existing concurrently.

Case Description

57-year-old male with a previous medical history significant for hypertension, hyperlipidemia, recently diagnosed CKD stage 3A with previous reported Creatinine of 1.54 a month before admission who presented with complaints of dyspnea, worsening fatigue, sleepiness, and approximately 20-pound weight gain along with decreased urine output. After initial evaluation, the patient was noted to have acute renal failure, evidenced by a creatinine of 13.08 and a BUN of 109. Chest X-ray did not reveal any acute cardiopulmonary findings. The patient subsequently underwent renal biopsy, which revealed glomeruli demonstrating necrosis or cellular/fibrocellular crescent formation. In addition, many glomeruli demonstrated Bowman's capsule disruption and extensive glomerular deposition of DNA JB 9 positive fibrils. As well as having positive anti-GBM antibodies. The patient received treatment with IV Solu-Medrol, which was then transitioned to oral Prednisone 80 mg in conjunction with oral Cyclophosphamide 100 mg. Furthermore, the patient underwent five sessions of plasmapheresis and required initiation on Hemodialysis in the hospital with plans to continue treatment after discharge.

Discussion

Standard of care treatment of anti-GBM disease usually involves the use of prednisone and cyclophosphamide in conjunction with plasmapheresis however, renal prognosis is poor with patient usually requiring initiation of hemodialysis. Similar treatment modalities are used to treat FGN with the clinical presentation of RPGN however, this is largely anectodal with similar renal outcomes. Our case highlights the rare coexistence of these two GNs and the importance of renal biopsy to aid in diagnostic/therapeutic intervention.