Primary Sclerosing Cholangitis

Primary sclerosing cholangitis (PSC) is a progressive, cholestatic liver disease defined by diffuse inflammation and fibrosis of the bile ducts and is associated with inflammatory bowel disease. PSC leads to obliteration of the biliary tree and ultimately biliary cirrhosis. PSC has been diagnosed more frequently in the past three decades because of routine liver biochemistry testing in clinical practice and widespread availablility of endoscopic retrograde cholangiopancreatography and magnetic resonance cholangiopancreatography. To date, the etiology of PSC remains unknown and effective medical therapy is not currently available. Patients with PSC have shortened life expectancy due to disease progression and associated co-morbidities such as cholangiocarcinoma and colon cancer. Orthotopic liver transplantation extends the live of patients with advanced stage PSC.