Autoimmune hepatitis and overlap syndromes

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Autoantibodies and autoimmune liver diseases

Diagnosis and classification of autoimmune liver diseases is dependent on the detectable autoantibody profile. Although AIH serology may be complex, diagnosis of PBC is closely linked to the demonstration of antimitochondrial antibodies (AMA) with specificity for the E2 subunits of the pyruvate dehydrogenase complex on the inner mitochondrial membrane, the M2 antigen [5], [6], [7], [8]. Sensitivity and specificity of anti-M2 AMA for the diagnosis of PBC is more than 95% dependent on the

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      Therefore, AIH can be defined as a multifactorial polygenic disease, caused by the interaction between a trigger and different environmental factors that occurs in a genetically susceptible individual. The overlap between AIH and other hepatic autoimmune diseases, such as PBC or PSC is well documented by numerous studies, as well as their epidemiologic details and clinical course [3–6]. However, only few studies [7,8] investigated the frequency of concurrent autoimmune diseases other than PBC or PSC in large numbers of patients.

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      Forty-one percent of patients with AIH and concurrent inflammatory bowel disease have cholangiographic changes of PSC,32 50% of children with AIH have findings by ERC that indicate autoimmune sclerosing cholangitis,33 and 1.7% to 10.0% of adults with classical AIH have biliary changes by MRC (albeit associated mainly with advanced hepatic fibrosis).43,44 The histologic findings may disclose portal edema or fibrosis, ductopenia (see Fig. 4), ductal tortuosity, ductular proliferation, cholate stasis, increased stainable hepatic copper, periductal fibrosis, or rarely, fibrous obliterative cholangitis (see Fig. 5).1,16,19,31,53 Patients with AIH who have cholestatic features that cannot be categorized as PBC or PSC have an overlapping cholestatic syndrome that may represent AMA-negative PBC23,54 or small duct PSC (see Table 1).22

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