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 1,
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 *,
and
Ingeborg M. Bajema *
*Department of Pathology, Leiden University Medical Center, Leiden, and Department of Internal Medicine, Meander Medical Center, Amersfoort, Netherlands; School of Medicine, Health Policy and Practice, University of East Anglia, Norwich, United Kingdom; Department of Nephrology, Spedali Civili, University of Brescia, Italy; ||Department of Clinical and Experimental Immunology, University Hospital Maastricht, Maastricht, Netherlands; ¶Department of Internal Medicine, Hôpital Cochin, Paris, France; and **Department of Medicine, Massachusetts General Hospital, Boston, Massachusetts
1 To whom correspondence should be addressed. E-mail: R.A.F.de_Lind_van_Wijngaarden{at}lumc.nl.
 |
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.
