We previously demonstrated association of DRB1*09:01-DQB1*03:03 haplotype, a haplotype common in East Asians but rare in the European populations, with MPA/MPO-AAV, suggesting that a population difference in HLA-class II plays a role in the epidemiology of this disease. Here, HLA-DRB1 is linked to anti-neutrophil cytoplasmic antibody-associated vasculitis.