Pathology · Hepatobiliary and Pancreatic Pathology

A 45-year-old man with primary sclerosing cholangitis undergoes liver biopsy for elevated alkaline phosphatase. Biopsy shows periductal 'onion-skin' fibrosis around bile ducts with eventual duct obliteration. This disease is most strongly associated with:

  • A Ulcerative colitis
  • B Autoimmune hepatitis
  • C Crohn disease
  • D Celiac disease
Correct answer: A. Ulcerative colitis

Explanation

Primary sclerosing cholangitis (PSC) has a strong association with ulcerative colitis, present in approximately 70–80% of PSC cases, making it one of the most important extra-intestinal manifestations of IBD. The characteristic 'onion-skin' periductal fibrosis leads to progressive biliary strictures, cholestasis, and eventual biliary cirrhosis. PSC is also a significant risk factor for cholangiocarcinoma, and patients with concomitant UC have an additional increased risk for colorectal carcinoma.

Reference: Robbins & Cotran Pathologic Basis of Disease, 10th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

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