La cholangite sclérosante primitive

Primary sclerosing cholangitis (PSC) is a rare progressive liver disease characterised by bile duct strictures caused by inflammatory and fibrotic damages. PSC patients are usually male patients, with a median age of 40, most often with inflammatory bowel disease. Clinical symptoms of PSC can be absence of any symptom, fatigue and pruritus or end stage liver disease. Diagnosis of PSC can be made if the three following criteria are met: 1) chronic cholestasis, 2) typical radiological or histological features, 3) exclusion of causes of secondary sclerosing cholangitis. Autoimmune IgG4 -related cholangitis is part of the causes of secondary sclerosing cholangitis. Magnetic resonance cholangiography has become the key tool for diagnosis of PSC. Magnetic resonance cholangiography shows typical radiological features of bile ducts and also evaluates impact of bile duct strictures on liver parenchyma. Liver biopsy is essential for diagnosis of small duct PSC, in which magnetic resonance cholangiography is normal, and for the diagnosis of overlap syndrome (PSC+ autoimmune hepatitis). PSC patients are at risk to develop iterative acute cholangitis and secondary biliary cirrhosis. Moreover, PSC is associated with an increased risk of cholangiocarcinoma and gallbladder carcinoma. Whereas diagnostic management of PSC has improved, notably with development of magnetic resonance cholangiography, therapeutic management remains limited. Ursodeoxycholic acid is the only treatment for PSC patients although studies have not shown benefit of this medication on survival of PSC patients. Therapeutic endoscopy should be restricted to symptomatic biliary strictures and should always be performed with concomitant ductal sampling because of high risk of cholangiocarcinoma. Liver transplantation is the therapeutic option for PSC patients with end stage liver disease or seriously reduced quality of live. Despite a risk of PSC recurrence on liver graft, prognosis of PSC patients after liver transplantation is good.