title: Fibrillary glomerulonephritis presenting as rapidly progressive glomerulonephritis.
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.
authors.affiliation: Johns Hopkins Medical Institutions, Baltimore, MD, USA. psharm16@jhmi.edu
authors.forename: Purva
authors.initials: P
authors.lastname: Sharma
authors.forename: Michael
authors.initials: M
authors.lastname: Kuperman
authors.forename: Lorraine
authors.initials: L
authors.lastname: Racusen
authors.forename: Duvuru
authors.initials: D
authors.lastname: Geetha
mesh_terms: D001707:Biopsy, Needle; D003520:Cyclophosphamide / Q000008:administration & dosage; D003937:Diagnosis, Differential; D018450:Disease Progression; D005260:Female; D005921:Glomerulonephritis / Q000175:diagnosis*; D006801:Humans; D007074:Immunoglobulin G / Q000378:metabolism; D007150:Immunohistochemistry; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D007262:Infusions, Intravenous; D007678:Kidney Glomerulus / Q000378:metabolism / Q000473:pathology; D008875:Middle Aged; D020551:Pulse Therapy, Drug; D013997:Time Factors
chemical_list: D007074:Immunoglobulin G; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide