Primary sclerosing cholangitis is a chronic cholestatic liver disease caused by diffuse inflammation and fibrosis that can involve the entire biliary tree. The cause is unknown, but it is presumed to be immune mediated, and there is a close association with inflammatory bowel disease, particularly ulcerative colitis. The disorder tends to affect men (male:female, 2:1), some presenting with fatigue, intermittent jaundice, weight loss, right upper quadrant pain, and pruritus, but many are asymptomatic at diagnosis, which is made incidentally when a persistently raised serum alkaline phosphatase is discovered, usually in the clinical setting of ulcerative colitis. Serum biochemical tests usually indicate cholestasis, but diagnosis is based on three criteria: (1) generalized beading and stenosis of the biliary system on cholangiography; (2) absence of choledocholithiasis or a history of bile duct surgery; and (3) exclusion of bile duct cancer, usually by prolonged follow-up. There is no curative medical treatment. Pruritus is initially managed with cholestyramine, with second-line treatments including rifampicin and naltrexone. Orthotopic liver transplantation is the only option available for young patients with advanced liver disease.
Primary sclerosing cholangitis, the biliary tree, and ulcerative colitis.