Epidemiology and genetics of large-vessel vasculitides. Report 1. Epidemiological and molecular genetic aspects of Takayasu arteritis

Takayasu arteritis (TA) is a rare systemic disease characterized by granulomatous inflammation affecting large arteries, primarily the aorta and its branches. First described in Japan, where the disease has a high prevalence, TA was subsequently reported, though less frequently, in other ethnic groups. Rare cases of familial aggregation suggest the involvement of genetic factors in the pathogenesis of TA. During nearly 45 years of genetic research on TA, a strong association has been established with the HLA-B5(52) antigen/HLA-B*5201 allele, as well as with other HLA and non-HLA molecular genetic markers.