Curbside Consultation of the Liver: 49 Clinical Questions

Mitchell L. Shiffman, MD

Question 35: Should All Patients With Suspected Primary Sclerosing Cholangitis Undergo An Endoscopic Retrograde Cholangiography To Confirm This Diagnosis? (continued)

Michael Fuchs, MD, PhD, FEBG

Primary sclerosing cholangitis (PSC) is a rare chronic cholestatic liver disease characterized by inflammation, obliteration, and progressive fibrosis of bile ducts. This results in strictures of the intrahepatic and/or extrahepatic biliary tree, ultimately leading to biliary cirrhosis and end-stage liver disease. The disease usually affects men, presents during the fourth and fifth decade of life, and will develop in up to 8% of patients with ulcerative colitis. Interestingly, up to 80% of patients with ulcerative colitis will develop PSC.1 PSC can be suspected based on biochemical…