Abstract The distribution of HLA-A, B, C and DR antigens was determined in a cohort of 104 unrelated Indian patients with Takayasu arteritis (TA) belonging to the North Indian states of Punjab, Haryana, Uttar Pradesh and Delhi. The data was compared with healthy controls belonging to the same ethnic group. In addition, polymorphism in the MHC class I chain related A (MIC A) gene was studied in a group of 25 TA patients and 40 healthy controls. The data revealed a strong association of the disease with HLA-B5 ( χ 2=22.5, P<1×10 −6, RR=3.08) as well as its two common serological subtypes, B51 ( χ 2=20.5) and B52 ( χ 2=18.5). No particular association was observed with any of the five alleles of the MIC A gene, nor any linkage disequilibrium could be established with these alleles and those of HLA-B locus in this population. The observation suggest that HLA linked genes are definitely involved in the development of Takayasu arteritis and that the disease in Indian subjects is associated with HLA-B5 and its two serological subtypes, B51 as well as B52.