title: [Pathogenesis of primary systemic vasculitides (I): ANCA-positive vasculitides].
abstract: The pathogenesis of different types of systemic vasculitis positive for antineutrophil cytoplasmic antibodies (ANCA) remains incompletely understood. ANCA constitute a heterogeneous group of antibodies that are associated with different types of small-vessel vasculitis, including Wegener's granulomatosis (WG), microscopic polyangiitis (MPA) and Churg-Strauss syndrome (CSS). Anti-proteinase 3 ANCA are present in more than 90% of patients with systemic WG, and anti-myeloperoxidase (MPO) ANCA in 50-75% of those with MPA and 40-60 % of those with CSS. The pathogenic role of ANCA has been well documented in vivo: passive transfer of anti-MPO ANCA in an MPO knockout mouse model immunized with MPO is sufficient to induce the disease. In vitro, mouse and human anti-proteinase 3 ANCA can activate neutrophils primed with TNF-a and contribute to vasculitic lesions. T-cells are also involved: type 1 helper cytokines have been detected in tissue lesions of limited forms of WG, while type 2 helper cytokines have been identified in its systemic forms. Eosinophils may play a key role in the development of vasculitic lesions in CSS, although this remains to be proved.
authors.affiliation: Université Paris-Descartes, Faculté de médecine, UPRES EA 1833, site Cochin, Paris.
authors.forename: P
authors.initials: P
authors.lastname: Guilpain
authors.forename: Y
authors.initials: Y
authors.lastname: Chanseaud
authors.forename: M-C
authors.initials: MC
authors.lastname: Tamby
authors.forename: A
authors.initials: A
authors.lastname: Mahr
authors.forename: A
authors.initials: A
authors.lastname: Servettaz
authors.forename: L
authors.initials: L
authors.lastname: Guillevin
authors.forename: L
authors.initials: L
authors.lastname: Mouthon
mesh_terms: D019264:Adoptive Transfer; D000818:Animals; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology*; D015267:Churg-Strauss Syndrome / Q000276:immunology; D016207:Cytokines / Q000276:immunology; D004195:Disease Models, Animal; D004804:Eosinophils / Q000276:immunology; D014890:Granulomatosis with Polyangiitis / Q000276:immunology; D006801:Humans; D007114:Immunization; D051379:Mice; D008807:Mice, Inbred BALB C; D018345:Mice, Knockout; D009000:Monocytes / Q000276:immunology; D009504:Neutrophils / Q000276:immunology; D009195:Peroxidase / Q000276:immunology; D015995:Prevalence; D013601:T-Lymphocytes / Q000276:immunology; D014657:Vasculitis / Q000453:epidemiology / Q000209:etiology* / Q000235:genetics / Q000276:immunology*
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D016207:Cytokines; D009195:Peroxidase