There are clear autoimmune aspects to PBC: there is a strong association with extrahepatic autoimmune diseases such as thyroid disease. Overlap syndromes between PBC and autoimmune hepatitis have been well documented1 , 2 although the response to immunosuppression is at best poor. One of the characteristic features of PBC is the association with disease specific autoantibodies: these include not only the antimitochondrial antibodies (AMA) but also antibodies to components of the nuclear pore complex (such as gp210). The antigens recognised by AMA have been identified (PDC-E2; OGDC-E2; E3BP; PDC-E1α; BCOADC-E2) and are located on the inner aspect of the mitochondrial membrane.3-7