Autoimmune liver disease.
OBJECTIVE
To review studies that improve the diagnosis and treatment of autoimmune hepatitis and presage new drug and molecular site-specific interventions.


RESULTS
Autoimmune hepatitis can present as acute or chronic hepatitis and as allograft dysfunction after liver transplantation. Elderly patients have an indolent but aggressive disease that responds well to corticosteroid therapy, and human leukocyte antigen DR4 characterizes this population. Geographic and ethnic factors influence clinical phenotype and behavior, and defects in T-regulatory cells may enhance cell-mediated cytotoxicity. Treatment response is the best index of prognosis, and normalization of serum aminotransferase abnormalities prevents disease progression. Mycophenolate mofetil, budesonide, rapamycin, and 6-thioguanine have been effective in small clinical experiences. De-novo autoimmune hepatitis can occur in adults and children after liver transplantation, and rapamycin may be an effective treatment.


CONCLUSIONS
Autoimmune hepatitis has a global distribution and diverse clinical manifestations. Phenotypes are affected by regional and ethnic factors which may provide clues to the etiologic agents. Defects in counter-regulatory functions enhance cell-mediated cytotoxicity, and interventions that promise site-specific actions affecting immunocyte differentiation and proliferation are now feasible. Autoimmune hepatitis must be considered in all patients with acute and chronic hepatitis and in all cases of allograft dysfunction after liver transplantation.
Czaja
Albert J
AJ
Division of Gastroenterology and Hepatology, Mayo Clinic College of Medicine, Rochester, MN 55905, USA. czaja.albert@mayo.edu
D001323:Autoantibodies / Q000097:blood; D015415:Biomarkers / Q000097:blood; D006684:HLA-DR Antigens; D059811:HLA-DRB1 Chains; D019693:Hepatitis, Autoimmune / Q000175:diagnosis / Q000209:etiology* / Q000276:immunology / Q000628:therapy*; D006801:Humans; D016031:Liver Transplantation; D011379:Prognosis
D001323:Autoantibodies; D015415:Biomarkers; D006684:HLA-DR Antigens; D059811:HLA-DRB1 Chains