Takayasu arteritis occurs with a strong predilection for women and particular geographic areas, and as related to the etiology of the disease, association of HLA antigens has been suggested. In the present study, the authors investigated the association of Takayasu arteritis with class I and class II HLA antigens in 59 Korean patients with this disease. Increased frequencies of HLA-Bw52 (chi 2 6.213, P < 0.02), Cw6 (chi 2 4.132, P < 0.05), DR7 (chi 2 4.506, P < 0.04), and DQw2 (chi 2 7.327, P < 0.01) were observed in the patient group as compared to the control group of healthy Koreans. In the Korean population, 2 risk factors in the HLA system for developing this disease appear to be (1) Bw52 and (2) DR7 and a probable haplotype of Cw6, B13, DR7, DQw2. Previous studies of the Japanese population revealed association of Bw52 and class II HLA antigens (DR2, Dw12), which are in linkage disequilibrium with Bw52. It is of interest that in the Korean population, class II antigens (DR7, DQw2), which are not linked to BW52, are associated with the disease. This finding suggests that the disease susceptibility gene of Takayasu arteritis is located between the HLA-B locus and HLA-DR, DQ loci.