Primary sclerosing cholangitis

Autoimmune diseases (AIDs) are characterized by a breakdown in immunological tolerance to the self, and by the production of autoantibodies. They are either systemic or organ-specific. The latter group includes immune diseases of the liver, or autoimmune hepatopathies. The target of these autoantibodies may be the hepatocyte, the small bile ducts or the medium or large bile ducts. Classically, a distinction is made between¿ autoimmune hepatitis (HAI);¿ primary biliary cholangitis (PBC);¿ primary sclerosing cholangitis (PSC);¿ Ig G4 lymphoplasmacytic sclerosing cholangitis.There are also overlap syndromes or borderline forms that combine the diagnostic criteria of two or more of these entities.