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Primary biliary cirrhosis

Definition

Primary biliary cirrhosis is irritation and swelling (inflammation) of the bile ducts of the liver. This blocks the flow of bile, which damages the liver cells and leads to scarring called cirrhosis.

Alternative Names

PBC

Causes

The cause of inflamed bile ducts in the liver is not known. However, primary biliary cirrhosis is an autoimmune disorder. That means your body's immune system mistakenly attacks healthy tissue.

The disease more commonly affects middle-aged women.

Long-term bile obstruction is believed to lead to liver cirrhosis. The disease may be linked to autoimmune disorders such as:

Symptoms

More than half of patients have no symptoms at the time of diagnosis. Symptoms most often come on slowly and may include:

Exams and Tests

The doctor or nurse will do a physical exam.

The following tests can check to see if your liver is working properly:

  • Albumin blood test
  • Liver function tests (serum alkaline phosphatase is most important)
  • Prothrombin time (PT)
  • Cholesterol and lipoprotein blood tests

Other tests that can help diagnose this disease are:

Treatment

The goal of treatment is to ease symptoms and prevent complications.

Cholestyramine (or colestipol) may reduce the itching. Ursodeoxycholic acid may improve removal of bile from the bloodstream. This may improve survival in some patients.

Vitamin replacement therapy restores vitamins A, K, E and D, which are lost in fatty stools. A calcium supplement or other bone medicines may be added to prevent or treat weak or soft bones (osteoporosis, and rarely osteomalacia).

Liver transplant may be successful if it is done before liver failure occurs.

Outlook (Prognosis)

The outcome can vary. If the condition is not treated, most patients will die without a liver transplant. About a quarter of patients who have had the disease for 10 years will have liver failure. Doctors can now use a statistical model to predict the best time to do the transplant. Other diseases such as hypothyroidism and anemia can also develop.

Possible Complications

Progressive cirrhosis can lead to liver failure. Complications can include:

When to Contact a Medical Professional

Call your health care provider if you have:

References

Afdhal NH. Diseases of the gallbladder and bile ducts. In:Goldman L, Schafer AI, eds. Goldman's Cecil Medicine. 24th ed. Philadelphia, PA: Saunders Elsevier; 2011:chap 158.

Angulo P, Lindor KD. Primary biliary cirrhosis. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger & Fordtran's Gastrointestinal and Liver Disease. 9th ed. Philadelphia, PA: Saunders Elsevier; 2010:chap 89.

Mayo MJ. Natural history of primary biliary cirrhosis. Clin Liver Dis. 2008;12:277-288.

Lindor KD, Gershwin ME, Poupon R, Kaplan M, et al. Primary biliary cirrhosis. American Association for Study of Liver Diseases. Hepatology. 2009;50:291.


Review Date: 5/15/2014
Reviewed By: Jenifer K. Lehrer, MD, Department of Gastroenterology, Frankford-Torresdale Hospital, Aria Health System, Philadelphia, PA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.
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