title: Role of ANCA in the pathogenesis of Wegener's granulomatosis: a short review.
abstract: The presence of autoantibodies to neutrophil cytoplasmic proteinase-3, usually reported as c-ANCA, is virtually diagnostic for Wegener's granulomatosis and related conditions. These antibodies are highly associated with active systemic disease, and may be implicated in pathogenesis. In vitro, c-ANCA can induce a respiratory burst and degranulation of cytokine-primed neutrophils, leading to lysis of cultured endothelial cells. This and other putative pathogenic mechanisms are reviewed.
authors.affiliation: Istituto di Terapia Medica, IRCCS Policlinico S. Matteo, University of Pavia, Italy.
authors.forename: C
authors.initials: C
authors.lastname: Montecucco
mesh_terms: D019268:Antibodies, Antineutrophil Cytoplasmic; D001323:Autoantibodies / Q000032:analysis / Q000276:immunology*; D015415:Biomarkers / Q000032:analysis; D015550:Cell Degranulation / Q000502:physiology; D016207:Cytokines / Q000276:immunology; D004727:Endothelium / Q000276:immunology / Q000473:pathology; D014890:Granulomatosis with Polyangiitis / Q000209:etiology / Q000276:immunology*; D006801:Humans; D009504:Neutrophils / Q000201:enzymology / Q000276:immunology; D016897:Respiratory Burst / Q000502:physiology; D012697:Serine Endopeptidases / Q000276:immunology
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D001323:Autoantibodies; D015415:Biomarkers; D016207:Cytokines; D012697:Serine Endopeptidases