title: Pathogenesis of lung vasculitis.
abstract: Vasculitides that affect the lung represent a diverse group of diseases with various systemic clinical manifestations, and include microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA, formerly Wegener granulomatosis), Churg-Strauss syndrome (CSS), and anti-glomerular basement membrane (anti-GBM) disease (Goodpasture syndrome). The etiologies of these diseases remain largely unknown. Although the pathogenic mechanisms of each differ, these diseases overlap by the presence of anti-neutrophil cytoplasmic autoantibodies in the vast majority of patients with MPA and GPA, and a substantial minority of patients with CSS and anti-GBM disease. This article reviews the current understanding of the pathogenesis of these four disease entities.
authors.affiliation: University of North Carolina Kidney Center, Chapel Hill. patrick_nachman@med.unc.edu
authors.forename: Patrick H
authors.initials: PH
authors.lastname: Nachman
authors.forename: Ashley G
authors.initials: AG
authors.lastname: Henderson
mesh_terms: D019867:Anti-Glomerular Basement Membrane Disease / Q000209:etiology / Q000276:immunology / Q000473:pathology; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000032:analysis / Q000276:immunology*; D015267:Churg-Strauss Syndrome / Q000209:etiology / Q000276:immunology / Q000473:pathology; D014890:Granulomatosis with Polyangiitis / Q000209:etiology / Q000276:immunology / Q000473:pathology; D006470:Hemorrhage / Q000209:etiology / Q000276:immunology / Q000473:pathology; D006801:Humans; D008171:Lung Diseases / Q000209:etiology / Q000276:immunology* / Q000473:pathology; D055953:Microscopic Polyangiitis / Q000209:etiology / Q000276:immunology / Q000473:pathology; D014657:Vasculitis / Q000150:complications / Q000209:etiology / Q000276:immunology*
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic