Pediatric patients with autoimmune hepatitis have manifestations and consequences that are different from their adult counterparts. Viruses and drugs may cause autoimmune hepatitis by triggering a final common pathway of pathogenesis. DRB1ast;0301 and DRB1ast;0401 are the principal susceptibility allele for type 1 autoimmune hepatitis. A lysine residue at DRbeta;71 of the HLA molecule influences prognosis. Cytotoxic CD8plus;cells are the sole effectors withing the lives of patients with autoimmune hepatitis, but the mechanisms of clonal expansion are unclear. In the woodchuck hepatitis model, a host-dependent immunoreactive state prior to viral infection is a requisite for chronic hepatitis. The target antigen of perinu-clear anti-neutrophil cytoplasmic antibodies in type 1 autoimmune hepatitis may be actin. Antinuclear antibodies are associated with a better long-term prognosis than seronegativity but seropositie patients who have antibodies to double-stranded DNA by immunosassay frequently fail to respond to corticosteroid therapy. Cytochrome P450 1A2 may be the hepatic autoantigen of the autoimmune polyglandular syndrome. Empiric corticosteriod therapy can be effective in patients with predominant autommune features and concurrent true hepatitis C virus infection.
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