Skip to Main Content

+

  • • 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

++

Symptoms and Signs

+

  • • Intermittent jaundice

    • Fever

    • Right upper quadrant pain

    • Pruritus

++

Laboratory Findings

+

  • • Elevated alkaline phosphatase

    • Hyperbilirubinemia

    • Leukocytosis

++

Imaging Findings

+

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

+

  • • Cholangiocarcinoma

    • Presence of cirrhosis

++

Rule Out

+

  • • Cholangiocarcinoma

+

  • • 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

++

When to Admit

+

  • • Cholangitis

++

Surgery

+

  • • Balloon dilatation of multiple strictures

    • Resection of dominant stricture followed by biliary reconstruction

    • Liver transplantation (preferably before onset of cirrhosis)

++

Contraindications

+

  • • Cirrhosis

++

Medications

+

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

++

Treatment Monitoring

+

  • • Alkaline phosphatase levels

++

Complications

+

  • • Cholangitis

    • Recurrent strictures

    • Primary nonfunction

    • Allograft rejection

    • Recurrent stricture post-transplant

++

Prognosis

+

  • • 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

++

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]

Want remote access to your institution's subscription?

Sign in to your MyAccess profile while you are actively authenticated on this site via your institution (you will be able to verify this by looking at the top right corner of the screen - if you see your institution's name, you are authenticated). Once logged in to your MyAccess profile, you will be able to access your institution's subscription for 90 days from any location. You must be logged in while authenticated at least once every 90 days to maintain this remote access.

Ok

About MyAccess

If your institution subscribes to this resource, and you don't have a MyAccess profile, please contact your library's reference desk for information on how to gain access to this resource from off-campus.

Subscription Options

AccessSurgery Full Site: One-Year Subscription

Connect to the full suite of AccessSurgery content and resources including more than 160 instructional videos, 16,000+ high-quality images, interactive board review, 20+ textbooks, and more.

$995 USD
Buy Now

Pay Per View: Timed Access to all of AccessSurgery

24 Hour Subscription $34.95

Buy Now

48 Hour Subscription $54.95

Buy Now

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.