PRIMARY SCLEROSING CHOLANGITIS (PSC) | ABIM Board Review

This is a snippet from Dr. Emmanuel C. Gorospe’s Gastroenterology lecture in MedStudy’s 2023-2024 Internal Medicine Video Board Review. medstudy.com/products/interna...
Biliary System - Non-Stone Disease: Primary Sclerosing
Cholangitis (PSC)
• Occurs more frequently in males (70%), middle-aged
• Cause unknown; inflammation leads to sclerosis/
scarring throughout biliary tree
• Negative AMA, often +p-ANCA
• 75% of PSC patients have UC (less common in CD)
- 5% of UC patients have PSC, and
2% of CD patients have PSC
• IBD patients with persistent increase in ALP should
be screened
• Diagnosis: usually start with MRCP, not ERCP
(increased risk of cholangitis)
PSC
• Similar to PBC, can see deficiencies in fat-soluble
vitamins
• 8-15% develop cholangiocarcinoma
- MRCP/ERCP can show “dominant stricture” but can
be difficult to distinguish benign dominant stricture
from cholangiocarcinoma
- Biliary brushing/biopsy can help diagnosis, but
not always
• Patients with UC and PSC are at increased risk of CRC compared to UC alone
• Increased risk of GB cancer
Onion Skin Fibrosis
• In PSC on liver biopsy, see fibrous portal expansion
• True “onion skin lesion” is not always seen
• Liver biopsies infrequently done to diagnose PSC;
diagnosis can frequently be made on bile duct imaging
PSC
Tx of PSC
• The only sure treatment of PSC is liver transplantation
• Colectomy can “cure” UC, but will not affect
course of PSC
• Remember: Rule out PSC in any patient with jaundice/
elevated ALP and history of IBD!
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