Hypotheses on the etiology of antineutrophil cytoplasmic autoantibody associated vasculitis: the cause is hidden, but the result is known

Robert A F de Lind van Wijngaarden, Leendert van Rijn, E Christiaan Hagen, Richard A Watts, Gina Gregorini, Jan Willem Cohen Tervaert, Alfred D Mahr, John L Niles, Emile de Heer, Jan A Bruijn, Ingeborg M Bajema
Clinical Journal of the American Society of Nephrology: CJASN 2008, 3 (1): 237-52
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.

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