title: Endothelium-neutrophil interactions in ANCA-associated diseases.
abstract: The two salient features of ANCA-associated vasculitis (AAV) are the restricted microvessel localization and the mechanism of inflammatory damage, independent of vascular immune deposits. The microvessel localization of the disease is due to the ANCA antigen accessibility, which is restricted to the membrane of neutrophils engaged in β2-integrin-mediated adhesion, while these antigens are cytoplasmic and inaccessible in resting neutrophils. The inflammatory vascular damage is the consequence of maximal proinflammatory responses of neutrophils, which face cumulative stimulations by TNF-α, β2-integrin engagement, C5a, and ANCA by the FcγRII receptor. This results in the premature intravascular explosive release by adherent neutrophils of all of their available weapons, normally designed to kill IgG-opsonized bacteria after migration in infected tissues.
authors.affiliation: Institut National de la Santé et de la Recherche Medicale INSERM U845, Université Paris Descartes, Sorbonne Paris Cité, France.
authors.forename: Lise
authors.initials: L
authors.lastname: Halbwachs
authors.forename: Philippe
authors.initials: P
authors.lastname: Lesavre
mesh_terms: D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000473:pathology* / Q000503:physiopathology; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000502:physiology*; D018821:CD18 Antigens / Q000502:physiology; D002448:Cell Adhesion / Q000502:physiology; D002450:Cell Communication / Q000502:physiology*; D004730:Endothelium, Vascular / Q000473:pathology* / Q000502:physiology; D006801:Humans; D007678:Kidney Glomerulus / Q000098:blood supply; D007684:Kidney Tubules / Q000098:blood supply; D009504:Neutrophils / Q000473:pathology* / Q000502:physiology
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D018821:CD18 Antigens