Coincidental cases of primary sclerosing cholangitis and biliary atresia in siblings?

Familial cases of primary sclerosing cholangitis or biliary atresia have been reported, although genetic influences and immunopathological abnormalities in these diseases are considered to be obscure. We report a case of primary sclerosing cholangitis and biliary atresia in siblings with the observation of HLA-DR antigen expression in the abnormal bile duct epithelial cells.