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    Sclerosing cholangitis

    Primary sclerosing cholangitis; PSC

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

    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.

    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

    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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    • Digestive system

      illustration

    • Bile pathway

      illustration

      • Digestive system

        illustration

      • Bile pathway

        illustration

      A Closer Look

        Tests for Sclerosing cholangitis

          Review Date: 8/11/2011

          Reviewed By: Linda J. Vorvick, MD, Medical Director, MEDEX Northwest Division of Physician Assistant Studis, University of Washington, School of Medicine; and George F. Longstreth, MD, Department of Gastroenterology, Kaiser Permanente Medical Care Program, San Diego, California. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

          The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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