Fibrillary glomerulonephritis masquerading as rapidly progressive glomerulonephritis with pseudo-linear glomerular basement membrane staining

Amr El-Husseini, Juan Carlos Aycinena, Bennet George, Stuart Jennings, Virgilius Cornea, B. Peter Sawaya

Research output: Contribution to journalArticlepeer-review

4 Scopus citations

Abstract

Fibrillary glomerulonephritis (FGN) is a rare disorder with poor renal prognosis. It is a heterogeneous disease associated with significant risk of end-stage renal disease (ESRD). Its etiology and pathogenesis have not been clearly identified. We report a case of a patient presenting with hypertensive crisis, nephrotic range proteinuria, and rapidly progressive glomerulonephritis (RPGN). The kidney biopsy demonstrates crescentic GN on light microscopy (LM) and strong pseudo-linear/globular glomerular basement membrane (GBM) staining for immunoglobulin G on immunofluorescence (IF), suggestive of anti-GBM disease. However, circulating anti-GBM antibodies were negative. Electron microscopy (EM) revealed fibrillary deposits in the GBM, confirming the diagnosis of FGN. Review of the literature revealed very few reported similar cases. It appears that severe hypertension and heavy proteinuria, while uncommon in anti-GBM disease, are consistent findings in RPGN form of FGN.

Original languageEnglish
Pages (from-to)231-235
Number of pages5
JournalClinical Nephrology
Volume84
Issue number4
DOIs
StatePublished - 2015

Bibliographical note

Publisher Copyright:
© 2015 Dustri-Verlag Dr. K. Feistle.

Keywords

  • Antiglomerular basement membrane (GBM) disease
  • Fibrillary glomerulonephritis (FGN)
  • Rapidly progressive glomerulonephritis (RPGN)

ASJC Scopus subject areas

  • Nephrology

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