Autoimmune liver disease.
OBJECTIVE
To review studies that improve the diagnosis and treatment of autoimmune hepatitis and extend the understanding of its pathogenic mechanisms.


RESULTS
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.


CONCLUSIONS
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.
Czaja
Albert J
AJ
Division of Gastroenterology and Hepatology, Mayo Clinic College of Medicine, Rochester, Minnesota 55905, USA. czaja.albert@mayo.edu
D000818:Animals; D001323:Autoantibodies / Q000097:blood*; D003937:Diagnosis, Differential; D019693:Hepatitis, Autoimmune / Q000175:diagnosis / Q000276:immunology* / Q000628:therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D016031:Liver Transplantation / Q000379:methods*; D045164:Mesenchymal Stem Cell Transplantation / Q000379:methods*; D011379:Prognosis
D001323:Autoantibodies; D007166:Immunosuppressive Agents