Autoimmune liver disease

Purpose of review To review studies that improve the diagnosis and treatment of autoimmune hepatitis and extend understanding of its pathogenic mechanisms. Recent findings Black patients have more advanced disease and poorer outcomes than white patients. Genome-wide DNA microsatellite techniques have identified multiple regions that may confer susceptibility or resistance to the disease. Preferential inactivation of one parentally-derived X chromosome may favor autoreactivity in women. Acute and chronic hepatitis of undetermined cause can respond to corticosteroid therapy and represent autoantibody-negative autoimmune hepatitis. Outcomes can be improved by continuing therapies until resolution of all features and by early identification of problematic patients with the Model for End Stage Liver Disease. Serum levels of B-cell activating factor correlate with laboratory indices of liver injury. Tacrolimus and mycophenolate mofetil are promising therapies for problematic patients, and the antigenic targets of atypical antibodies to liver/kidney microsome may lead to diagnostic tests for de-novo autoimmune hepatitis after liver transplantation. Summary Ethnic background and genetic predisposition affect the occurrence and outcome of autoimmune hepatitis. Susceptibility and resistance factors across the human genome underscore the genetic complexity of the disease. Outcomes can be improved by better use of current regimens and further evaluation of action-specific immunosuppressive agents.