• Primary Biliary Cirrhosis

    (PBC)

    Definition

    Primary biliary cirrhosis (PBC) is a chronic condition inside the liver. It is swelling in a part of the liver called bile ducts. Over time, this swelling can cause permanent damage to the bile ducts.
    The liver creates a fluid called bile. The fluid is sent out of the liver through bile ducts. The bile then moves to the gallbladder and the small intestine. Bile helps break down food in the intestines. PBC makes it difficult for bile to move out of the liver. The bile is not able to pass through the damaged bile ducts. As a result, the bile backs up into the liver. This leads to liver damage.

    Causes

    The exact cause of PBC is unknown. It appears to be an autoimmune disorder. This means the immune system is attacking health tissue. It is not known what causes an autoimmune reaction. It is likely due to a combination of genetic and environmental factors.

    Risk Factors

    PBC is more common in women. Other factors that may increase your chance of PBC include:

    Symptoms

    Symptoms of PBC include:
    • Fatigue
    • Itchy skin
    • Abdominal pain, especially in right upper abdomen
    • Signs of liver damage:
      • Yellowing of the skin and/or eyes—jaundice
      • Spider veins
      • Xanthelasma—yellow deposits around eyelids

    Diagnosis

    Your doctor will ask about your symptoms and medical history. A physical exam will be done. Blood tests will help to determine the extent of liver problems. They may also help look for causes like a hepatitis infection or autoimmune disorder. A liver biopsy will also help determine how much liver damage has occurred.
    Detailed pictures of the bile ducts may be needed. To get these pictures, your doctor may order:
    Liver Biopsy
    Nucleus Image
    Copyright © Nucleus Medical Media, Inc.

    Treatment

    There is no known cure for PBC. However, a variety of treatments may help to manage symptoms. Treatment can also help to slow the progression of liver damage and reduce the possibility of complications.
    Talk with your doctor about the best treatment plan for you. Treatment options may include:

    Medications

    Certain medications may help control itching. Ursodeoxycholic acid may also help to move bile through the bile ducts.

    Nutrition

    Your doctor may recommend vitamin supplements. The low levels of bile may make it difficult for your body to breakdown food. As a result, you cannot get enough vitamins from food. Vitamins A, D, K, and calcium are commonly recommended.
    A healthy, well-balanced diet helps your overall health. Your doctor may recommend supplements if you are having trouble reaching your nutrition goals. You should also avoid raw shellfish if you have cirrhosis.
    Avoid alcohol or other items that can affect your liver. Talk to your doctor about any medications you are taking. Even some over-the-counter medications can be harmful with cirrhosis.

    Liver Transplant

    A liver transplant is the only complete cure for PBC. It is only considered when other treatments are unable to control symptoms.

    Prevention

    There are no current guidelines to prevent PBC because the cause is unknown.

    RESOURCES

    American Liver Foundation http://www.liverfoundation.org

    National Institute of Diabetes and Digestive and Kidney Diseases ttp://www.niddk.nih.gov

    CANADIAN RESOURCES

    Canadian Digestive Health Foundation http://www.cdhf.ca

    Canadian Liver Foundation http://www.liver.ca

    References

    Heathcote JE. Management of primary biliary cirrhosis. Hepatology. 2000;31(4):1005-1013.

    Lindor KD, Gershwin ME, Poupon R, et al. Primary biliary cirrhosis. Hepatology. 2009;50(1):291-308.

    Metcalf JV, Howel D, James OF, Bhopal R. Primary biliary cirrhosis: epidemiology helping the clinician. BMJ. 1996;312(7040):1181-1182.

    Poupon RE, Balkau B, Eschwege E, Poupon R. A multicenter, controlled trial of ursodiol for the treatment of primary biliary cirrhosis. UDCA-BPC Study Group. N Engl J Med. 1991;324(22):1548-1554.

    Primary biliary cholangitis (PBC). EBSCO DynaMed Plus website. Available at: http://www.dynamed.com/topics/dmp~AN~T114824/Primary-biliary-cholangitis-PBC. Updated August 24, 2016. Accessed September 29, 2016.

    Primary biliary cirrhosis. American Liver Foundation website. Available at: http://www.liverfoundation.org/abouttheliver/info/pbc. Updated October 4, 2011. Accessed May 28, 2013.

    Primary biliary cirrhosis. National Institute of Diabetes and Digestive and Kidney Diseases website. Available at: http://www.niddk.nih.gov/health-information/health-topics/liver-disease/primary-biliary-cirrhosis/Pages/facts.aspx. Updated April 30, 2012. Accessed May 28, 2013.

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