Autoimmune liver disease.

Autoimmune hepatitis in children may be associated with sclerosing cholangitis in the absence of inflammatory bowel disease. Wilson disease can have clinical and laboratory features that resemble autoimmune hepatitis, and it may respond initially to corticosteroids. Soluble HLA-DR antigens reflect clinical activity, and they may be useful markers of treatment response. Polymorphisms of the cytotoxic T lymphocyte antigen-4 gene may synergize with other autoimmune promoters or HLA risk factors to increase susceptibility and alter disease expression. DRB1*1301 distinguishes Argentine children from Argentine adults and identifies a unique subgroup. Antibodies to soluble liver antigen/liver-pancreas do not characterize a separate clinical entity. Their target antigen has been isolated, and it shares homologies with a selenocysteine-specific protecting factor (tRNP((Ser)Sec)). CYP2D6 is expressed on the hepatocyte surface, and it can be targeted by antibodies in autoimmune hepatitis and chronic hepatitis C. Perinuclear antineutrophil cytoplasmic antibodies lack sensitivity and specificity for autoimmune hepatitis, and they have diverse antigen specificities. Activation-induced cell death may be impaired in autoimmune hepatitis, and, in contrast to budesonide, mycophenolate mofetil has been effective in a small study of problematic patients.