Sclerosing Cholangitis

Primary sclerosing cholangitis (PSC) is a chronic inflammatory disease of the biliary tree that is of unknown etiology, causes biliary obstruction, and can lead to chronic cholestasis, episodes of acute cholangitis, biliary cirrhosis, and cholangiocarcinoma. The clinical presentation is variable, ranging from an asymptomatic patient with mild elevation of liver biochemical tests or abnormal imaging to acute cholangitis or complications of cirrhosis and portal hypertension. PSC is typically diagnosed by imaging of the biliary tree with magnetic resonance cholangiopancreatography (MRCP) and occasionally with ERCP. The role of ERCP in PSC is mainly therapeutic: dilating and stenting of dominant biliary strictures, removing biliary tract calculi, and differentiating between benign and malignant strictures. Prophylactic antibiotics should be used during ERCP in PSC patients as the main adverse event is bacterial cholangitis. Cholangiocarcinoma can occur in up to 20% of patients with PSC, but the diagnosis remains difficult; brush cytology has a low sensitivity, although molecular techniques involving detection of chromosomal and DNA abnormalities show promise.