Antineutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis (AASV) is a group of autoimmune diseases. The human leukocyte antigen (HLA) system is devoted to the pathogenesis of these autoimmune diseases. The present study aimed to investigate the distribution of HLA-DRB1 alleles in Chinese AASV patients and its potential association with clinical and pathologic characteristics. This study included 152 Chinese patients with AASV and 200 healthy controls. Typing of HLA-DRB1 alleles was performed by bidirectional sequencing of exon 2. Compared with normal controls, DRB1*1454 was significantly less prevalent in AASV patients (0/304 vs 14/400, p = 4.6 × 10(-4), p corrected (p(c)) = 0.017), whereas DRB1*1101 was significantly more frequent in patients with MPA (32/214 vs 26/400, p = 6.4 × 10(-4), p(c) = 0.023). In patients with Wegener's granulomatosis (WG) who were PR3-ANCA positive, DRB1*1202 (8/38 vs 20/400, p = 1.3 × 10(-3), p(c) = 0.047) was more prevalent than in normal controls. Compared with normal controls, HLA-DRB1*1454 was less prevalent in AASV patients, whereas DRB1*1101 was significantly more frequent in patients with microscopic polyangiitis. HLA-DRB1*1202 was prevalent among patients with PR3-ANCA-positive WG.
Copyright © 2011 American Society for Histocompatibility and Immunogenetics. Published by Elsevier Inc. All rights reserved.