Question 73

Question:

A 25-year-old man presents to the emergency department with fever, jaundice, and pain localized to the right upper quadrant. Subsequent endoscopic retrograde cholangiopancreatography (ERCP) findings show alternating strictures and dilation of the bile ducts with a characteristic “beading” appearance. Both intrahepatic and extrahepatic bile ducts are involved. What is the most likely diagnosis?

Correct Answer: C.  Primary sclerosing cholangitis

Primary sclerosing cholangitis (PSC) is a chronic idiopathic disorder involving inflammation and fibrosis of both intra- and extrahepatic bile ducts. Alternating dilation and stricture of bile ducts results in characteristic “beading” appearance on ERCP. Up to 70% of patients with PSC suffer from ulcerative colitis.