title: Factors Associated With Pathogenicity of Anti-Glomerular Basal Membrane Antibodies: A Case Report.
abstract: Antiglomerular basement membrane (GBM) disease is known as a super-acute proliferative glomerulonephritis caused by auto-antibodies targeting the NC1 domain of the α3 chain of type IV collagen.Here, we describe a case of atypical anti-GBM disease presenting as a dialysis-dependent acute renal failure with unusual mild glomerular involvement. We found that immunoglobulin G (IgG) deposits were restricted to the uncommon IgG2 and IgG4 subclasses, and that blood was positive for anti-GBM antibodies by immunofluorescence, but not by Enzyme Linked Immunosorbent Assay (ELISA). The patient was treated with plasma exchanges, corticosteroids, and cyclosphosphamide. He eventually regained a normal renal function.This case demonstrates that biopsy-proven anti-GBM disease can have reduced pathogenicity. Referring to previous studies of anti-GBM detection in the blood from healthy or minimally ill individuals, we discuss the antigenic specificities, the IgG subclasses, and the involvement of complement in this observation.We suggest that anti-GBM disease is a heterogeneous entity and that the study of IgG subclasses by immunofluorescence may help to distinguish categories with different severities.
authors.affiliation: From the Urgences Néphrologiques et Transplantation Rénale (RO, JP, AH, ER, PG); Service d'Anatomie Pathologique (DB, IB); Service de Néphrologie et Dialyses (KD), Hôpital Tenon; Université Pierre et Marie Curie (DB, IB, AH, ER, PG), (Paris 6), Sorbonne Universités, Paris, France; Department of Nephrology (TH), Clinical Sciences in Lund, Lund University, Lund, Sweden; and Laboratoire Jean Dausset (LabEX Transplantex) et Inserm UMRS 1160 (RT), Hôpital Saint Louis, Paris, France.
authors.forename: Rime
authors.initials: R
authors.lastname: Ossman
authors.forename: David
authors.initials: D
authors.lastname: Buob
authors.forename: Thomas
authors.initials: T
authors.lastname: Hellmark
authors.forename: Isabelle
authors.initials: I
authors.lastname: Brocheriou
authors.forename: Julie
authors.initials: J
authors.lastname: Peltier
authors.forename: Ryad
authors.initials: R
authors.lastname: Tamouza
authors.forename: Karine
authors.initials: K
authors.lastname: Dahan
authors.forename: Alexandre
authors.initials: A
authors.lastname: Hertig
authors.forename: Eric
authors.initials: E
authors.lastname: Rondeau
authors.forename: Pierre
authors.initials: P
authors.lastname: Galichon
mesh_terms: D058186:Acute Kidney Injury / Q000209:etiology / Q000276:immunology* / Q000628:therapy; D019867:Anti-Glomerular Basement Membrane Disease / Q000097:blood / Q000150:complications / Q000276:immunology*; D001323:Autoantibodies / Q000097:blood*; D004797:Enzyme-Linked Immunosorbent Assay; D000939:Epitopes; D005455:Fluorescent Antibody Technique; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D008297:Male; D008875:Middle Aged; D006435:Renal Dialysis
chemical_list: D001323:Autoantibodies; D000939:Epitopes; D007074:Immunoglobulin G; C051951:antiglomerular basement membrane antibody