Autoimmune liver disease

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Autoimmune hepatitis can affect diverse ethnic groups, and its clinical expression and outcome can vary accordingly. An asymptomatic presentation may identify patients who respond more readily to medication. Celiac sprue is important to recognize and treat by gluten restriction. Centrilobular necrosis and coincidental destructive cholangitis do not preclude the diagnosis, and antibodies to soluble liver antigen/liver pancreas may identify patients susceptible to relapse. Women, who have non-DRB1*0401 DR4 alleles more commonly than do men, may respond to a diverse range of autoantigens. DRB1*1301 is associated with autoimmune hepatitis in Brazil, especially among children, and it may favor an indigenous triggering agent. Variant syndromes are heterogenous conditions that probably reflect referral biases, and molecular mimicry between foreign and self-antigens is the basis for most theories of pathogenesis. Immunosuppressive medications (eg, cyclosporine, mycophenolate mofetil) have been used empirically with success, and recurrent and de novo disease after liver transplantation must be considered in all patients with graft dysfunction.

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