What we know about primary biliary cirrhosis

Primary biliary cirrhosis (PBC) is an autoimmune liver disease characterized by destruction of the intrahepatic bile ducts. Despite many recent advances in the study of the disease, including the identification of PDC-E2 as the major recognized autoantigen, the pathogenesis of the disease is still unknown, and further study of the disease is confounded by the lack of a suitable animal model. In this review, we compare and contrast what is known about this model autoimmune disease. In fact, despite the view that primary biliary cirrhosis is a model disease, there are some problems, including relatively weak immunogenetic associations, general failure of immunosuppressive treatments, and no evident connection between the antimitochondrial antibody and the pathogenesis of the biliary ductular lesions. Whatever the case, the cloning of the cDNA for the major mitochondrial autoantigen for PBC ushered in the molecular era for autoimmunity in general, and there has been an ongoing stream of immunologically relevant discoveries consequent on this.