title: Pathophysiology of ANCA-associated vasculitides: are ANCA really pathogenic?
abstract: The strong association of antineutrophil cytoplasmic autoantibodies (ANCA) with certain forms of small vessel vasculitis suggests a pathogenic role of these autoantibodies in the disease process. In vitro, ANCA can activate neutrophils and monocytes to produce reactive oxygen intermediates, to release lysosomal enzymes, and to secrete proinflammatory cytokines. More recently, it was demonstrated that antimyeloperoxidase ANCA can induce systemic vasculitis and glomerulonephritis in mice. Taken together, these data provide convincing evidence that ANCA are indeed pathogenic.
authors.affiliation: Department of Clinical and Experimental Immunology, Cardiovascular Research Institute Maastricht, University Maastricht, Maastricht, The Netherlands. jw.cohentervaert@immuno.unimaas.nl
authors.forename: P
authors.initials: P
authors.lastname: Heeringa
authors.forename: J W Cohen
authors.initials: JW
authors.lastname: Tervaert
mesh_terms: D000818:Animals; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000378:metabolism*; D005921:Glomerulonephritis / Q000209:etiology / Q000276:immunology / Q000503:physiopathology; D006801:Humans; D066298:In Vitro Techniques; D051379:Mice; D009000:Monocytes / Q000502:physiology; D018375:Neutrophil Activation; D009195:Peroxidase / Q000276:immunology; D017382:Reactive Oxygen Species / Q000378:metabolism; D014657:Vasculitis / Q000209:etiology / Q000276:immunology / Q000503:physiopathology*
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D017382:Reactive Oxygen Species; D009195:Peroxidase