Hôpital Saint-Antoine, service d’hépatologie, F-75012, INSERM, UMR_S 938, CDR Saint-Antoine, F-75012, Paris, France, Sorbonne Universités, UPMC Univ Paris 06, UMR_S 938, CDR Saint-Antoine, F-75005, Paris, France
Université Paris 7 Diderot, Faculté de Médecine Xavier-Bichat, 16 rue Henri-Huchard 75018 Paris
Primary sclerosing cholangitis (PSC) is a chronic cholestatic disease of unknown origin commonly associated with inflammatory bowel disease and characterized by fibrosing inflammatory destruction of intra- and/or extrahepatic biliary ducts. The course may be variable from one patient to another but PSC is often progressive, leading to biliary cirrhosis and its complications. Although hepatocellular carcinoma may occur in patients with cirrhotic-stage PSC, the hepatobiliary malignancies specific to PSC are cholangiocarcinoma and, in a lesser degree, gall-bladder carcinoma. Patients with associated IBD have also an increased risk of developing colon carcinoma and a surveillance colonoscopy every 1-2 years is recommended. A diagnosis of PSC is made when magnetic resonance cholangiography shows typical findings in patients with a cholestatic biochemical profile and without causes of secondary secondary cholangitis. Many variant forms have been described whose diagnosis necessitates liver biopsy. Currently, there is no established effective medical treatment and liver transplantation is the only life-extending therapeutic alternative for patients with end-stage PSC. The role for ursodeoxycholoc acid (UDCA) is currently debated but its use at a dose of 15-20 mg/kg/day has been approved by the French Health Authorities. Addition of steroids is recommended when autoimmune features are present. Severe extra-hepatic biliary strictures with symptoms or significant cholestasis should be treated with biliary dilatation, with or without stenting. There is no evidence based algorithm for the follow-up of patients with PSC but some regular investigations (biological every 6 months and radiological every year) are recommended.