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Genetically distinct subsets within ANCA-associated vasculitis.

The New England journal of medicine | 19 Jul 2012

PA Lyons, TF Rayner, S Trivedi, JU Holle, RA Watts, DR Jayne, B Baslund, P Brenchley, A Bruchfeld, AN Chaudhry, JW Cohen Tervaert, P Deloukas, C Feighery, WL Gross, L Guillevin, I Gunnarsson, L Harper, Z Hrušková, MA Little, D Martorana, T Neumann, S Ohlsson, S Padmanabhan, CD Pusey, AD Salama, JS Sanders, CO Savage, M Segelmark, CA Stegeman, V Tesar, A Vaglio, S Wieczorek, B Wilde, J Zwerina, AJ Rees, DG Clayton and KG Smith
Abstract
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a severe condition encompassing two major syndromes: granulomatosis with polyangiitis (formerly known as Wegener’s granulomatosis) and microscopic polyangiitis. Its cause is unknown, and there is debate about whether it is a single disease entity and what role ANCA plays in its pathogenesis. We investigated its genetic basis.
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Concepts
Granuloma, Arthritis, Vascular-related cutaneous conditions, Vasculitis, Autoimmune diseases, Proteinase 3, Anti-neutrophil cytoplasmic antibody, Wegener's granulomatosis
MeSH headings
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, Case-Control Studies, Female, Genetic Predisposition to Disease, Genome-Wide Association Study, Genotyping Techniques, HLA-DP Antigens, Humans, Major Histocompatibility Complex, Male, Microscopic Polyangiitis, Myeloblastin, Polymorphism, Single Nucleotide, Risk Factors, Wegener Granulomatosis, alpha 1-Antitrypsin
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