PURPOSE: To study the distribution of human leukocyte antigen HLA-A/B antigens and HLA-DR/-DR/-DP alleles and to investigate the immunogenetic background of Korean patients with Vogt-Koyanagi-Harada (VKH) syndrome and clinical course with different types of HLA. METHODS: Human leukocyte antigen typings were performed in 18 Korean patients with VKH syndrome and in 128 healthy control subjects. HLA-A/B loci serologic typing was performed according to the standard microlymphocytotoxicity technique. DNA was extracted through the salting out method, and HLA-DR phenotyping and HLA DR4, HLA-DR, and HLA-DP subtyping were performed with the polymerase chain reaction-sequence specific oligonucleotide probe (PCR-SSOP) method. RESULTS: Among HLA-A/B antigens typed by the standard microlymphocytotoxicity method, the frequencies of HLA-A31 (RR = 6,1, P < 1 x 10(-2)) and HLA-B55 (RR = 15.8, P < .05) were significantly increased in the patient group compared with the control group. Among HLA-DR/-DQ/-DP alleles subtyped by DNA methods, the frequencies of HLA-DRB1*04 (RR = 45.1, P < 1 x 10(-7)) and HLA-DRB1*07 (RR = 3.2, P < .05) were significantly increased. However, significant decreases in HLA-DRB1*08 (RR = .1, P < .05), HLA-DRB1*13 (RR = .1, P < .05), and HLA-DRB1*14 (RR = .1, P < .05) frequencies were observed. The result of HLA-DR, HLA-DQ, and HLA-DP subtying showed the significant increase in DRB1*0405 (RR = 45.1, P < 1 x 10(-7)), DQA1*0302 (RR = 12.0, P < 1 x 10(-4)), DRB1*0303 (RR = 5.0, P < 1 x 10(-2)), DRB1*0401 (RR = 18.9, P < 1 x 10(-6)), and DPB1*0501 (RR = 3.8, P < .05). However, significant decreases in DQA1*0101 (RR = .1, P < .05), DQA1*0102 (RR = .1, P < 1 x 10(-2)), DaA1*0103 (RR = .1, P < .05), DRA1*0501 (RR = .1, P < 1 x 10-2), DQ2B1*0301 (RR = .1, P < .05), DQB1*0601 (RR = .1, P < .05), DPB1*0201 (RR = .3, P < .05), and DPB1*0401 (RR = .1, P < .05) frequencies were also observed. In patients with DRB1*0405 itself or HLA-DRB1*0405-DRA1*0302-DRB1*0401 haplotype, a reduction in visual acuity and ocular complications was common. CONCLUSIONS: These results suggest that HLA-DRB1*0405 itself or HLA-DRB1*0405-DRA1*0302-DRB1*0401 haplotype is greatly increased and may play the most important role in the development and the clinical course of VKH syndrome in Korean patients, (Am J Ophthalmol 2000;129:173-177. (C) 2000 by Elsevier Science Inc. All rights reserved.)