title: [Cholestatic liver diseases].
abstract: Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are important causes of cholestatic liver disease. IgG4-associated cholangitis (IAC) also belongs to the same entity. Overlap syndromes combine characteristics of cholestatic liver diseases and autoimmune hepatitis. The diagnosis of PBC is based on the detection of anti-mitochondrial antibodies. PBC is frequently associated with other autoimmune disorders. The treatment of choice is ursodeoxycholic acid. PSC is frequently associated with inflammatory bowel disease (IBD). The cholangiography shows characteristic bile duct lesions. Bile duct strictures and bacterial cholangitis should be treated by dilatation and antibiotics, respectively. Cirrhosis may ultimately develop in PBC and PSC. In advanced PBC or PSC, liver transplantation might be indicated. The clinical course of IAC is similar to PSC. In contrast to PSC, however, there is no association with IBD.
authors: Abteilung Innere Medizin II, Medizinische Universitätsklinik, Freiburg i. Br.
authors: Daniel
authors: D
authors: Grimm
authors: Robert
authors: R
authors: Thimme
mesh_terms: D001706:Biopsy; D002758:Cholangiography; D002760:Cholangiopancreatography, Endoscopic Retrograde; D015209:Cholangitis, Sclerosing / Q000175:diagnosis / Q000209:etiology / Q000473:pathology / Q000628:therapy; D002779:Cholestasis / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D003937:Diagnosis, Differential; D019693:Hepatitis, Autoimmune / Q000175:diagnosis / Q000209:etiology / Q000473:pathology / Q000628:therapy; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D008099:Liver / Q000473:pathology; D008105:Liver Cirrhosis, Biliary / Q000175:diagnosis / Q000209:etiology / Q000473:pathology / Q000628:therapy; D008111:Liver Function Tests; D011379:Prognosis
chemical_list: D007074:Immunoglobulin G