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
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
Written and medically reviewed by the StethoPrep medical team.