[Primary biliary cirrhosis]

To discuss exhaustively: clinical, serological and pathologic aspects of primary biliary cirrhosis (CBP); recent advantages in the knowledge of the disease's pathogenesis, therapeutical approaches that still appear less preferable than liver transplantation.

We reviewed the most important recent studios on CBP.

More than 90% of patients have antimitochondrial antibodies, so that CBP can be considered an autoimmune disease. Inciting agents could be either PDC E2-like or MHC molecules. An autoaggressive mechanism against biliary ducts could be the following: the neo-antigen on the surface of biliary tract cells could be the target for the immunological damage; anti PDC-E2 antimitochondrial antibodies could be secondary to the cellular damage and to the release of mitochondrial enzymes.

In primary biliary cirrhosis, several circumstances (specificity of biliary-ductal destruction, lymphocyte infiltration of portal tract, abnormal expression of HLA-DR on surface of biliary epithelium cells) suggest that the epithelial cells of intrahepatic biliary ducts are the target of a severe and isolated immune response. The identification of the E2-like PDC antigen is a significant achievement in the knowledge of the pathogenetic process. On this basis, it is now to investigate possible genetic markers of risk and to clarify the role of T cells in the immunopathogenesis of the illness. The only effective treatment for CBP is liver transplantation.