Autoimmune liver disease

PURPOSE OF REVIEW: The aim of this article is to review studies that improve the diagnosis and treatment of autoimmune hepatitis and suggest new drug and molecular interventions. RECENT FINDINGS: Elderly patients have an indolent but aggressive disease that responds well to corticosteroid therapy. Variant syndromes are artificial designations that reflect uncertainties regarding the diagnostic limits of classical disease. Antibodies to cyclic citrullinated proteins and complex assays for antibodies to actin and alpha-actinin may have prognostic value. Defects in the number and function of T regulatory cells may enhance cell-mediated cytotoxicity. HLA DRB113 may be a risk factor in some North American patients, and disease outcome may be influenced by the 'dose' of alleles encoding critical residues. Screening for thiopurine methyltransferase deficiency does not predict azathioprine intolerance. Treatment until normalization of the laboratory and histological features reduces the risk of relapse by 30-50%. Adverse outcomes in pregnancy are associated with antibodies to soluble liver antigen/liver pancreas and Ro/SSA. SUMMARY: Novel serological tests may have prognostic value. Defects in the suppressor activity of regulatory T cells may promote liver injury. Genetic predispositions strongly influence disease occurrence and outcome. Laboratory and histological features should be normal prior to drug withdrawal. Azathioprine toxicities cannot be predicted.