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Primary Sclerosing Cholangitis
 PSC

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%)

Clinical Findings

  • Asymptomatic; onset of symptoms is insidious
  • Diarrhea
  • Fever, chills
  • Jaundice
  • Pruritus
  • Elevated serum alkaline phosphatase, aspartate aminotransferase and ascorbate levels

Imaging Findings

  • ERCP considered study of choice but magnetic resonance cholangiopancreatography (MRCP) is less invasive and approaches accuracy of ERCP
  • Early changes may include ulceration of the common bile duct
  • Irregular narrowing with saccular dilatation proximal to strictured ducts leads to beaded  (beads-on-a-string) appearance of ducts
  • Usually involves intra- and extrahepatic ducts simultaneously
  • MRCP findings
    • High T2 in wedge-shaped areas with dilatation of ducts
  • US may show thickening of gallbladder wall, thickening and dilatation of the bile ducts

Differential Diagnosis

  • Secondary causes of cholangitis which can mimic PSC
    • Biliary calculi
    • Infection
    • Pancreatitis

Treatment

  • Endoscopic dilatation of some strictures has been tried
  • Definitive treatment is liver transplantation

Complications

  • Cirrhosis
  • Portal hypertension
  • Liver failure (as long as 10-15 years after diagnosis)
  • Cholangiocarcinoma

Prognosis

  • Ten-year survival of 65%

Primary Sclerosing Cholangitis

Primary Sclerosing Cholangitis

Primary Sclerosing Cholangitis. Images from Endoscopic Retrograde Cholangiopancreatography (ERCP) show alternating areas of stricture (black arrows) and dilated segments of the intrahepatic ducts (white arrows) characteristic of the "beaded" appearance seen with primary sclerosing cholangitis.
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MedScape   Primary Sclerosing Cholangitis -- Approach to Diagnosis  Steele, I; Levy, C; Lindor, K