title: Pathogenesis of anti-neutrophil cytoplasmic antibody-associated vasculitis: challenges and solutions 2014.
abstract: Anti-neutrophil cytoplasmic autoantibodies (ANCA) with specificity for proteinase 3 (PR3-ANCA) or myeloperoxidase (MPO-ANCA) are a defining feature of ANCA-associated vasculitides (AAV). They play a pivotal role in disease pathophysiology and have strongly improved early diagnosis and treatment of these infrequent, but potentially fatal diseases. Neutrophils and their products are major players in initiating the autoimmune response and tissue destruction in vasculitic as well as granulomatous inflammation. This review highlights recent findings on old and novel players (ANCA, neutrophils, neutrophil extracellular traps, fibroblasts, immune cells and complement) and puts them into context with the current understanding of disease mechanisms in AAV.
authors.affiliation: Medizinische Klinik IV, Department of Nephrology, University Hospital Munich, Munich, Germany.
authors.forename: Ulf
authors.initials: U
authors.lastname: Schönermarck
authors.affiliation: Klinikum Bad Bramstedt, Vasculitis Centre, Bad Bramstedt, Germany.
authors.forename: Elena
authors.initials: E
authors.lastname: Csernok
authors.affiliation: Department of Rheumatology, University Hospital Schleswig-Holstein, Luebeck, Germany.
authors.forename: Wolfgang L
authors.initials: WL
authors.lastname: Gross
mesh_terms: D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000209:etiology* / Q000276:immunology; D015551:Autoimmunity / Q000276:immunology; D003165:Complement System Proteins / Q000276:immunology; D006801:Humans; D009504:Neutrophils / Q000276:immunology
chemical_list: D003165:Complement System Proteins
keywords: ANCA; NETs; pathogenesis; vasculitis