Fibrillary glomerulonephritis presenting as rapidly progressive glomerulonephritis

Am J Kidney Dis. 2012 Jul;60(1):157-9. doi: 10.1053/j.ajkd.2011.12.024. Epub 2012 Mar 6.

Abstract

Fibrillary glomerulonephritis (GN) is an uncommon cause of rapidly progressive kidney failure. We report a case of rapidly progressive kidney failure with kidney biopsy showing crescentic GN on light microscopy and immunofluorescence showing linear/globular glomerular basement membrane (GBM) staining for immunoglobulin G and C3, consistent with anti-GBM disease. However, electron microscopy showed fibrillary deposits in the GBM, suggesting a diagnosis of fibrillary GN. As exemplified by this case, it is important to consider fibrillary GN in the differential diagnosis of crescentic GN with linear immunoglobulin G deposits within the GBM. Electron microscopy is crucial to make this diagnosis.

Publication types

  • Case Reports

MeSH terms

  • Biopsy, Needle
  • Cyclophosphamide / administration & dosage
  • Diagnosis, Differential
  • Disease Progression
  • Female
  • Glomerulonephritis / diagnosis*
  • Humans
  • Immunoglobulin G / metabolism
  • Immunohistochemistry
  • Immunosuppressive Agents / administration & dosage
  • Infusions, Intravenous
  • Kidney Glomerulus / metabolism
  • Kidney Glomerulus / pathology
  • Middle Aged
  • Pulse Therapy, Drug
  • Time Factors

Substances

  • Immunoglobulin G
  • Immunosuppressive Agents
  • Cyclophosphamide