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  • • Associated with ulcerative colitis 40-60%, pancreatitis 12-25%, diabetes mellitus 5-10%, and rarely other autoimmune disorders

    • Onset during fourth or fifth decade of life

    • Increased risk for cholangiocarcinoma

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Symptoms and Signs

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  • • Intermittent jaundice

    • Fever

    • Right upper quadrant pain

    • Pruritus

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Laboratory Findings

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  • • Elevated alkaline phosphatase

    • Hyperbilirubinemia

    • Leukocytosis

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Imaging Findings

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  • • Right upper quadrant US, ERCP, and magnetic resonance cholangiopancreatography (MRCP) may show multiple dilatations and strictures of extrahepatic biliary ducts

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  • • Cholangiocarcinoma

    • Presence of cirrhosis

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Rule Out

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  • • Cholangiocarcinoma

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  • • History and physical exam

    • Liver function tests

    • Abdominal US

    • ERCP

    • Liver biopsy if question of cirrhosis

    • Brushings by percutaneous transhepatic cholangiography (PTC) or ERCP if question of malignancy

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When to Admit

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  • • Cholangitis

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Surgery

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  • • Balloon dilatation of multiple strictures

    • Resection of dominant stricture followed by biliary reconstruction

    • Liver transplantation (preferably before onset of cirrhosis)

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Contraindications

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  • • Cirrhosis

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Medications

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  • • Ursodiol (improves liver function and histology but no difference in 5-year clinical outcome)

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Treatment Monitoring

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  • • Alkaline phosphatase levels

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Complications

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  • • Cholangitis

    • Recurrent strictures

    • Primary nonfunction

    • Allograft rejection

    • Recurrent stricture post-transplant

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Prognosis

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  • • 85% survival rate 5 years post transplant

    • 71% actuarial survival at 5 years for resection of dominant stricture (only 20% if cirrhosis present)

    • 43% long-term success with balloon therapy for multiple strictures

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References

Kim WR et al. A revised natural history model for primary sclerosing cholangitis. Mayo Clin Proc. 2000;75:688.  [PubMed: 10907383]
van Hoogstraten HJ et al. Ursodeoxycholic acid therapy for primary sclerosing cholangitis: results of a 2-year randomized controlled trial to evaluate single versus multiple daily doses. J Hepatol. 1998;29:417.
Kubicka S et al: K-ras mutations in the bile of patients with primary sclerosing cholangitis. Gut 2001;48:403.  [PubMed: 11171833]
Ryder SD, Beckingham IJ: ABC of diseases of liver, pancreas, and biliary system. Other causes of parenchymal liver disease. BMJ 2001;322:290.  [PubMed: 11157536]

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