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Rheumatology Advance Access originally published online on March 31, 2009
Rheumatology 2009 48(6):622-625; doi:10.1093/rheumatology/kep057
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© The Author 2009. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

HLA-DR4, DR13(6) and the ancestral haplotype A1B8DR3 are associated with ANCA-associated vasculitis and Wegener's granulomatosis

Patricia M. Stassen1, Jan W. Cohen-Tervaert2, Simon P. M. Lems3, Bouke G. Hepkema3, Cees G. M. Kallenberg4 and Coen A. Stegeman1

1Department of Nephrology, University Medical Center Groningen, University of Groningen, Groningen, 2Department of Internal Medicine, Division of Clinical and Experimental Immunology, Maastricht University Medical Center, Maastricht, 3Laboratory for Transplantation Immunology and 4Department of Clinical Immunology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.

Correspondence to: Patricia M. Stassen, Department of Internal Medicine, Maastricht University Medical Center, PO Box 5800, 6202 AZ, Maastricht, The Netherlands. E-mail: p.stassen{at}mumc.nl


   Abstract

Objectives. As the HLA system is involved in recognition of self and non-self, an association with the development of ANCA-associated vasculitis (AAV) seems probable. In this study, the relation between HLA antigens and AAV and it's severity were investigated.

Methods. Consecutive patients diagnosed with AAV at our centre, who were followed for at least 2 years, were included. The frequency of HLA antigens of AAV and WG patients was compared with 5872 healthy blood donors from the same region and with 4000 healthy Dutch controls originating from a Eurotransplant database.

Results. From 304 AAV patients, sufficient data were available. We found DR13(6) to be less prevalent and both DR4 and the ancestral haplotype A1B8DR3 more prevalent in patients with AAV compared with controls, particularly in patients with WG. In addition, DR1 was less prevalent in patients with WG in comparison with controls. Further, DR8 was more prevalent in patients with CSS compared with other forms of vasculitis and controls. There were no associations between HLA antigens and disease characteristics or course of AAV or WG.

Conclusions. AAV is associated with increased prevalence of DR4 and the ancestral haplotype A1B8DR3 and with decreased prevalence of DR13(6), particularly in patients with WG. In patients with WG, prevalence of DR1 was decreased, whereas in patients with CSS DR8 was increased. No associations between HLA antigens and disease characteristics or course of AAV were found.

KEY WORDS: Autoinflammatory conditions, Vasculitis, Immunogenetics and HLA, Major histocompatibility complex, Physiology, ANCA-associated vasculitis, Wegener's granulomatosis

Submitted 10 July 2008; revised version accepted 24 February 2009.
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