Autoimmune liver disease.

PURPOSE OF REVIEW: To review studies that improve the diagnosis and treatment of autoimmune hepatitis and extend the understanding of its pathogenic mechanisms.

RECENT FINDINGS: A simplified diagnostic scoring system has high sensitivity and specificity. Biliary changes detected by MRI are of uncertain nature and significance. New candidate autoantigens have been identified by proteomic analyses. T regulatory cells suppress disease activity; their adoptive transfer is beneficial in animal models. Budesonide in combination with azathioprine is effective frontline therapy. Bone marrow-derived mesenchymal stem cell transplantation may emerge as salvage therapy. Screening for hepatocellular cancer is justified. Racial disparities in disease severity, mortality, and treatment remain unexplained.

SUMMARY: Diagnosis has been simplified and management strategies have been upgraded. Biliary changes have been recognized but are of uncertain nature and significance. New antigens and antibodies have been described. T-cell populations that modulate disease activity have been characterized, and adoptive transfer of T regulatory cells is possible. Budesonide in combination with azathioprine is effective frontline therapy, and therapeutic interventions that target critical pathogenic mechanisms are feasible.