Although PBC–AIH overlap syndrome in white individuals has been reported to be associated with positive tests for HLA-B8, DR3, or DR4, which are characterized as HLA haplotypes for AIH [2], both of our familial occurrence cases shared the same HLA haplotype, DRB1*04:05/DRB1*15:02. Here, TNFRSF10A is linked to connective tissue disorder.