Clinical Presentation

A 25-year-old woman presents with pruritus.

(A) Endoscopic cholangiography shows diffuse ductal irregularity involving the intrahepatic and extrahepatic ducts with pruning of the distal biliary tree (arrowheads), intraluminal filling defects (large arrow), and involvement of the ductal confluence (small arrow). (B) Contrast-enhanced computed tomography shows intrahepatic ductal dilatation with wall thickening and beading (arrow). (C) More caudal image shows marked wall thickening involving the cecum (large arrow), a diffusely featureless colon (small arrow), and diffuse moderate colonic wall thickening (arrowheads).

Differential Diagnosis

• Sclerosing cholangitis (SC):