Autoimmune hepatitis and overlap syndromes
Section snippets
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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Cited by (52)
Overlap of concurrent extrahepatic autoimmune diseases is associated with milder disease severity of newly diagnosed autoimmune hepatitis
2021, Hepatobiliary and Pancreatic Diseases InternationalCitation Excerpt :Typical hallmarks of liver histology in AIH include interface hepatitis with dense plasma cell-rich lymphoplasmacytic infiltrates. The histological spectrum varies including bridging necrosis and severe inflammatory activity, especially in acute manifestation of the disease [18–24]. At initial diagnosis, the extent of fibrosis varies.
Extrahepatic autoimmunity in autoimmune liver disease
2019, European Journal of Internal MedicineCitation Excerpt :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.
Bile Duct Diseases
2018, MacSween's Pathology of the LiverAutoimmune Hepatitis Overlap Syndromes and Liver Pathology
2017, Gastroenterology Clinics of North AmericaCitation Excerpt :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
Assessment of Liver Histology
2017, Zakim and Boyer's Hepatology: A Textbook of Liver DiseaseEASL clinical practice guidelines: Autoimmune hepatitis
2015, Journal of Hepatology