Immunologic mechanisms have been proposed in the pathogenesis of the p
utative 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 apprecia
tion 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, id
entification of B-cell and T-cell antigenic epitopes involved in autoa
ntibody production, functional analyses of T cells from both periphera
l blood and the inflamed livers of patients, and the immunologic mecha
nisms of hepatocellular necrosis and bile duct injury. Although specif
ic immunologic mechanisms of hepatobiliary injury remain poorly define
d, continued studies are likely to provide the insights necessary to d
evise new diagnostic and therapeutic approaches.