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Abstract Autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are the three major autoimmune diseases affecting the liver. They are all characterized by the presence of a variety of autoantibodies, some of which are found in all three diseases, whereas others are restricted to one or two of them or are even specific for the particular disease. AIH is a chronic inflammatory liver disease with an unknown cause that is characterized by a higher incidence in females, the presence of serum autoantibodies, hyper IgG globulinemia, and plasma cell infiltration into liver tissue, where periportal or interface hepatitis is the characteristic histological finding. Primary biliary cirrhosis is a chronic cholestatic liver disease, in which interlobular bile ducts are damaged. It is characterized by AMA (90–95% of patients) and hyper IgM globulinemia, and affects predominantly females. The diagnosis of PBC is currently based on three criteria: the presence of AMA in serum which is highly specific for the disease, elevation of biochemical indices of cholestasis for more than 6 months, and histological features in the liver that are indicative of the diagnosis. Primary sclerosing cholangitis is a chronic cholestatic liver disease characterized by the destruction of the intrahepatic and/or extra hepatic bile ducts through chronic inflammation and fibrosis, leading to biliary complications including bile stasis and cirrhosis. |