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Case of the Week 349

What's the most likely diagnosis?

  • 36 year-old undergoing ERCP for RUQ pain

Image from Endoscopic Retrograde Cholangio-pancreaticography

  1. Pancreatic carcinoma
  2. Caroli's disease
  3. Choledocal cyst
  4. Biliary calculus
  5. Primary sclerosing cholangitis

Additional Image - Additional image from ERCP


Additional Image



Image from ERCP




5. Primary sclerosing cholangitis



More (Click Discussion Tab)

Primary Sclerosing Cholangitis

General Considerations

  • Inflammation, destruction and fibrosis of intrahepatic and extrahepatic bile ducts
  • Unknown etiology but thought to be auto-immune
  • A major cause of liver transplants in United States
  • Majority occur in association with inflammatory bowel disease (IBD)
    • Most often ulcerative colitis (UC)
      • 5% of all patients with UC have PSC
      • 75% of all patients with PSC will have UC
  • Most often between 30-60 years of age with a mean of 40
    • More common in men (70%)

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