Histocompatibility antigens in primary biliary cirrhosis.

Primary biliary cirrhosis (PBC) is characterized by marked perturbation of the immune system. In addition, there are familial associations which suggest genetic background to the disease. We have studied HLA-A, B, and D loci in 15 Japanese women with PBC, and compared them to controls. Although HLA-A and B loci in PBC patients showed similar prevalence to the control population, HLA-DHO was significantly more common, occurring in 69% of patients with PBC and 21% of controls. This suggests a genetic predisposition to PBC, and the HLA-DHO locus association may reflect a genetic abnormality of the immune responses.