IMMUNOPATHOGENESIS OF AUTOIMMUNE HEPATOBILIARY DISEASES

Immunologic mechanisms have been proposed in the pathogenesis of the putative autoimmune hepatobiliary diseases: primary biliary cirrhosis, autoimmune hepatitis, and primary sclerosing cholangitis. Advances in our understanding of the human immune response have led to an appreciation of the complexity of interactions that initiate, perpetuate, and regulate autoimmunity. This appreciation has catalyzed the rapid pace of investigation into the immunogenetics of disease susceptibility, identification of B-cell and T-cell antigenic epitopes involved in autoantibody production, functional analyses of T cells from both peripheral blood and the inflamed livers of patients, and the immunologic mechanisms of hepatocellular necrosis and bile duct injury. Although specific immunologic mechanisms of hepatobiliary injury remain poorly defined, continued studies are likely to provide the insights necessary to devise new diagnostic and therapeutic approaches.