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Sclerosing cholangitis
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Sclerosing cholangitis

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Primary sclerosing cholangitis; PSC

Sclerosing cholangitis refers to swelling (inflammation), scarring, and destruction of the bile ducts inside and outside of the liver.

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  • Causes

    The cause of this condition is usually unknown.

    The disease may be seen in patients who have:

    • Autoimmune disorders
    • Chronic pancreatitis
    • Inflammatory bowel disease (Crohn's disease and ulcerative colitis)
    • Sarcoidosis

    Genetic factors may also be responsible. Sclerosing cholangitis occurs more often in men than women. This disorder is rare in children.

    Sclerosing cholangitis may also be caused by:

    • Choledocholithiasis
    • Infections in the liver, gallbladder, and bile ducts
  • Symptoms

    The first symptoms are usually:

    • Fatigue
    • Itching
    • Yellowing of the skin and eyes (jaundice)

    However, some people may have no symptoms.

    Other symptoms may include:

    • Enlarged liver
    • Enlarged spleen
    • Loss of appetite and weight loss
    • Repeat episodes of cholangitis
  • Exams and Tests

    Some people do not have symptoms, but blood work shows that they have abnormal liver function. The doctor will look for:

    • Diseases that cause similar problems
    • Diseases that often occur with this condition (especially inflammatory bowel disease)
    • Gallstones

    Tests that show cholangitis include:

    • Abdominal CT scan
    • Abdominal ultrasound
    • Endoscopic retrograde cholangiopancreatography (ERCP)
    • Liver biopsy
    • Magnetic resonance cholangiopancreatography (MRCP)
    • Percutaneous transhepatic cholangiogram (PTC)

    Blood tests include:

    • Liver enzymes (liver function tests)
  • Treatment

    Medications that may be used include:

    • Cholestyramine
    • Ursodeoxycholic acid (ursodiol)
    • Fat-soluble vitamins (D, E, A, K)
    • Antibiotics for infections in the bile ducts
    • Medications that quiet the immune system (prednisone, azathioprine, cyclosporine, methotrexate)

    Surgical procedures:

    • Inserting a long, thin tube with a balloon at the end to open up narrowing (endoscopic balloon dilation of strictures)
    • Placement of a drain or tube for major narrowing (strictures) of biliary ducts
    • Proctocolectomy (for those who have both ulcerative colitis and sclerosing cholangitis)
    • Liver transplant
  • Outlook (Prognosis)

    How well patients do varies. The disease tends to get worse over time and sometimes patients develop:

    • Ascites and varices
    • Biliary cirrhosis
    • Liver failure
    • Persistent jaundice

    Some patients develop infections of the bile ducts that keep returning.

    People with this condition have an increased risk of developing cancer of the bile ducts (cholangiocarcinoma). They should be checked regularly with a liver imaging test and blood tests. Patients who also have inflammatory bowel disease may have an increased risk of cancer of the colon or rectum and should have periodic colonoscopy.

  • Possible Complications

    • Bleeding esophageal varices
    • Cancer in the bile ducts (cholangiocarcinoma)
    • Cirrhosis and liver failure
    • Infection of the biliary system (cholangitis)
    • Narrowing of the bile ducts (strictures)
    • Vitamin deficiencies

Related Information

  BileCrohn's diseaseCirrhosisBiliary system     Crohn's diseaseCirrhosis

References

Gordon FD. Primary sclerosing cholangitis. Surg Clin North Am. 2008;88:1385-1407.

Ross AS, Kowdley KV. Sclerosing cholangitis and recurrent pyogenic cholangitis. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger & Fordtran's Gastrointestinal and Liver Disease. 9th ed. Philadelphia, Pa: Saunders Elsevier; 2010:chap 68.

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Review Date: 7/18/2013  

Reviewed By: George F. Longstreth, MD, Department of Gastroenterology, Kaiser Permanente Medical Care Program, San Diego, California. Also reviewed by David Zieve, MD, MHA, Bethanne Black, and the A.D.A.M. Editorial team.

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