The strongest evidence that favors a causal relationship between the pathogenesis of PBC and AMA reactivity is derived from experimental models of PBC; for example, one of the female SJL mice demonstrates a breakdown in tolerance against PDC antigens with anti-PDC antibody production followed by PBC-like biliary duct lesions, which is also known as experimental autoimmune cholangitis (5). Here, PDC is linked to primary biliary cholangitis.