Primary sclerosing cholangitis (PSC) is characterized by fibrous obliteration of bile ducts producing an 'onion-skin' periductal fibrosis pattern. PSC is most strongly associated with:

• A Ulcerative colitis ✓
• B Crohn's disease involving the small bowel
• C Autoimmune hepatitis and anti-smooth muscle antibodies
• D Gallstones causing biliary obstruction

Explanation

PSC is strongly associated with ulcerative colitis (70–80% of PSC patients have UC), though the courses of the two diseases are independent. The 'onion-skin' periductal fibrosis is pathognomonic on biopsy. PSC is complicated by secondary biliary cirrhosis and cholangiocarcinoma. Anti-smooth muscle antibodies are associated with autoimmune hepatitis type 1, not PSC (which may have pANCA).

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

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.