Autoimmune hepatitis. Part B: diagnosis.

Diagnostic criteria have been codified by the International Autoimmune Hepatitis Group, and a scoring system can quantify the strength of the diagnosis and over-ride the impact of absent or inconsistent features. The absence of a definable etiologic agent and precise diagnostic test, implies that the diagnosis may be missed or misapplied. Centrilobular (zone 3) necrosis may be an early form of autoimmune hepatitis and this pattern can transform to the classical pattern of interface hepatitis. An acute severe or fulminant presentation is possible, and different ethnic groups may have different manifestations and outcomes. Asymptomatic patients at presentation commonly become symptomatic, and treatment decisions must be based on objective features of disease severity and not the presence or absence of symptoms. Concurrent autoimmune diseases are frequent, and they may constitute an autoimmune polyglandular syndrome associated with a single gene mutation. Emerging autoantibodies of possible prognostic value are antibodies to soluble liver antigen/liver pancreas, asialoglycoprotein receptor, actin, and liver cytosol type 1. HLA DRB1*03, *04, *03-*04, *07, *13 and DQB1*02 are associated with the occurrence, clinical phenotype and outcome of autoimmune hepatitis. Variant syndromes should be suspected if cholestatic features are prominent and conventional treatment is ineffective.