hepatic and biliary disorders
Primary Sclerosing Cholangitis (PSC)
Introduction
Primary sclerosing cholangitis (PSC) is patchy inflammation, fibrosis, and strictures of the bile ducts that has no known cause. However, 80% of patients with PSC also have inflammatory bowel disease, most often ulcerative colitis. Other associated conditions include connective tissue disorders, autoimmune disorders, and immunodeficiency syndromes, sometimes complicated by opportunistic infections. Fatigue and pruritus develop insidiously and progressively. Diagnosis is by cholangiography (magnetic resonance cholangiopancreatography [MRCP] or endoscopic retrograde cholangiopancreatography [ERCP]). Liver transplantation is indicated for advanced disease. (See also Overview of Biliary Function .) PSC is the most common form of sclerosing cholangitis. Most (70%) patients with PSC are men. Mean age at diagnosis is 40 years. 