title: Clinicopathological characteristics of typical and atypical anti-glomerular basement membrane nephritis.
abstract: Anti-glomerular basement membrane (GBM) antibody disease is a rare pathological condition that mainly involves renal and/or pulmonary parenchyma. It is characterized by the presence of circulating anti-GBM antibodies accompanied by a linear deposition of immunoglobulins (Ig) detected through immunofluorescence (IF) technique and typical signs and symptoms of organ dysfunction, such as rapidly progressive glomerulonephritis (RPGN) and pulmonary hemorrhage (PH). However, recently atypical forms of anti-GBM disease have been described and the presence of overlapping diseases contributed to make its diagnosis challenging. In this review will be discussed the entire spectrum of renal anti-GBM related conditions, focusing the attention on the differences in terms of pathogenesis, diagnosis and therapy of these disparate entities.
authors.affiliation: Department of Surgery and Translational Medicine, Pathology, University Milan Bicocca, San Gerardo Hospital, Monza, Italy.
authors.forename: Vincenzo
authors.initials: V
authors.lastname: L'Imperio
authors.affiliation: Department of Nephrology, University Milan Bicocca, San Gerardo Hospital, Monza, Italy.
authors.forename: Elena
authors.initials: E
authors.lastname: Ajello
authors.affiliation: Department of Nephrology, University Milan Bicocca, San Gerardo Hospital, Monza, Italy.
authors.forename: Federico
authors.initials: F
authors.lastname: Pieruzzi
authors.affiliation: Department of Pathology, Hospital Sacco, Milan, Italy.
authors.forename: Manuela
authors.initials: M
authors.lastname: Nebuloni
authors.affiliation: Department of Pathology, Hospital Sacco, Milan, Italy.
authors.forename: Antonella
authors.initials: A
authors.lastname: Tosoni
authors.affiliation: Department of Surgery and Translational Medicine, Pathology, University Milan Bicocca, San Gerardo Hospital, Monza, Italy.
authors.forename: Franco
authors.initials: F
authors.lastname: Ferrario
authors.affiliation: Department of Surgery and Translational Medicine, Pathology, University Milan Bicocca, San Gerardo Hospital, Monza, Italy. Fabio.pagni@unimib.it.
authors.forename: Fabio
authors.initials: F
authors.lastname: Pagni
mesh_terms: D019867:Anti-Glomerular Basement Membrane Disease / Q000145:classification / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D001323:Autoantibodies / Q000276:immunology*; D001706:Biopsy; D004359:Drug Therapy, Combination; D005455:Fluorescent Antibody Technique; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007678:Kidney Glomerulus / Q000187:drug effects / Q000276:immunology / Q000473:pathology* / Q000648:ultrastructure; D008854:Microscopy, Electron; D011237:Predictive Value of Tests; D011379:Prognosis; D012307:Risk Factors
chemical_list: D001323:Autoantibodies; D007166:Immunosuppressive Agents; C051951:antiglomerular basement membrane antibody
keywords: Anti-GBM disease; Crescentic glomerulonephritis; Nephropathology; Rapidly progressive glomerulonephritis