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Primary Biliary Cirrhosis

Primary biliary cirrhosis is a progressive disease that damages and eventually disintegrates the bile ducts leading from the liver to the gallbladder and small intestines. When this occurs, the liver cannot release bile into the digestive system and becomes damaged due to the buildup.

This chronic disease, which affects up to 1 in 4,000 individuals, develops slowly and women are more affected by it than men by a 9:1 ratio. It is typically found in patients between 40 and 60 years old.

  • Causes

    Causes for the disease are unknown. However, researchers believe there is a link to autoimmune conditions, which is where the body’s immune system turns on itself and attacks healthy tissue.

    In addition, researchers have noted genetic links in the disease, as people who have a brother, sister or parent with the disease are more likely to contract it themselves.

  • Symptoms

    In the early stages of primary biliary cirrhosis, patients may not report any symptoms. If they occur, fatigue and intense itching in the limbs and back are the most common. In addition, bile back up in the liver may lead to jaundice, which will appear in advanced stages of the disease.

    In addition to symptoms, other conditions may be prevalent in patients with primary biliary cirrhosis, such as glandular problems (especially near the eyes and mouth), arthritis, renal stones and gallstones. In the later stages of primary biliary cirrhosis, the patient’s bones also may begin to soften, increasing the risk of fractures.

  • Testing and Diagnosis

    Since symptoms usually do not present themselves until the disease’s latter stages, primary biliary cirrhosis is often found through routine blood tests. If initial tests spark a doctor’s concern, additional tests will be scheduled, including:

    • An anti-mitochondrial antibody test – this antibody is detected in nearly 9 of 10 primary biliary cirrhosis patients.
    • An alkaline phosphatase blood test – two tests six months apart will likely determine if the patient has primary biliary cirrhosis.

  • Treatment

    No treatment to reverse primary biliary cirrhosis has been identified. Current therapies include using vitamins, calcium supplements and skin creams to alleviate symptoms.

    The Food and Drug Administration (FDA) has approved only one drug for treating primary biliary cirrhosis: Ursodiol. The drug includes ursodeoxycholic acid, which appears naturally in bile, and helps restore liver functionality. The patient must take Ursodiol once daily for the rest of their life or until they need a liver transplant.

    Doctors also may recommend a balanced diet since patients with cirrhosis often suffer from malnutrition. Also, since liver damage is either apparent or likely, patients should not use alcohol or other illicit substances that may exacerbate their condition.