title: Hypotheses on the etiology of antineutrophil cytoplasmic autoantibody associated vasculitis: the cause is hidden, but the result is known.
abstract: The first description of what is now known as antineutrophil cytoplasmic autoantibody-associated necrotizing vasculitis appeared more than 140 yr ago. Since then, many aspects of the pathogenic pathway have been elucidated, indicating the involvement of antineutrophil cytoplasmic autoantibodies, but why antineutrophil cytoplasmic autoantibodies are produced in the first place remains unknown. Over the years, many hypotheses have emerged addressing the etiology of antineutrophil cytoplasmic antibody production, but no exclusive factor or set of factors can so far be held responsible. Herein is reviewed the most influential hypotheses regarding the causes of antineutrophil cytoplasmic antibody-associated vasculitis with the aim of placing in an epidemiologic background the different hypotheses that are centered on environmental and genetic influences.
authors: Department of Pathology, Leiden University Medical Center, Albinusdreef 2, P.O. Box 9600, 2300 RC Leiden, Netherlands. R.A.F.de_Lind_van_Wijngaarden@lumc.nl
authors: Robert A F
authors: RA
authors: de Lind van Wijngaarden
authors: Leendert
authors: L
authors: van Rijn
authors: E Christiaan
authors: EC
authors: Hagen
authors: Richard A
authors: RA
authors: Watts
authors: Gina
authors: G
authors: Gregorini
authors: Jan Willem Cohen
authors: JW
authors: Tervaert
authors: Alfred D
authors: AD
authors: Mahr
authors: John L
authors: JL
authors: Niles
authors: Emile
authors: E
authors: de Heer
authors: Jan A
authors: JA
authors: Bruijn
authors: Ingeborg M
authors: IM
authors: Bajema
mesh_terms: D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology*; D014890:Granulomatosis with Polyangiitis / Q000209:etiology* / Q000276:immunology*; D006801:Humans; D014657:Vasculitis / Q000209:etiology* / Q000276:immunology*
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic