title: Fibrillary glomerulonephritis masquerading as rapidly progressive glomerulonephritis with pseudo-linear glomerular basement membrane staining.
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.
authors.forename: Amr
authors.initials: A
authors.lastname: El-Husseini
authors.forename: Juan-Carlos
authors.initials: JC
authors.lastname: Aycinena
authors.forename: Bennet
authors.initials: B
authors.lastname: George
authors.forename: Stuart
authors.initials: S
authors.lastname: Jennings
authors.forename: Virgilius
authors.initials: V
authors.lastname: Cornea
authors.forename: B Peter
authors.initials: BP
authors.lastname: Sawaya
mesh_terms: D019867:Anti-Glomerular Basement Membrane Disease / Q000175:diagnosis* / Q000473:pathology; D001323:Autoantibodies / Q000032:analysis; D005260:Female; D050533:Glomerular Basement Membrane / Q000473:pathology; D005921:Glomerulonephritis / Q000175:diagnosis* / Q000473:pathology; D006801:Humans; D008875:Middle Aged
chemical_list: D001323:Autoantibodies; C051951:antiglomerular basement membrane antibody