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Primary Biliary Cirrhosis (PBC)

Primary Biliary Cirrhosis (PBC)

Topic Overview

What is primary biliary cirrhosis (PBC)?

Primary biliary cirrhosis (PBC) is a kind of cirrhosis caused by damage to the bile ducts in the liver. Much like other forms of liver disease, PBC permanently damages the liver as tissue is replaced with scar tissue (fibrosis). As more scar tissue develops, the structure and function of the liver are affected.

What causes PBC?

The exact cause of PBC is unknown. But it may be related to a problem with the immune system, which is the body's natural defense system.

What are the symptoms?

Many people who have primary biliary cirrhosis do not have symptoms. When symptoms do occur, they may include:

  • Itching (pruritus).
  • Fatigue.
  • Yellowing of the skin and the white part of the eyes (jaundice).
  • Discomfort in the upper right part of the abdomen.
  • Dry eyes and mouth.
  • Vaginal dryness.

With more advanced liver damage, people may have complications related to cirrhosis such as:

  • Fluid buildup in the abdomen (ascites).
  • Bleeding of enlarged veins in the esophagus, stomach, and rectum (variceal bleeding).
  • Premature thinning of the bones (osteoporosis).

How is PBC diagnosed?

Primary biliary cirrhosis is suspected based on a medical history, physical exam, and lab tests that show high levels of the enzyme alkaline phosphatase and antimitochondrial antibodies (AMA) in the blood. Your doctor may also conduct an imaging test such as an ultrasound, which shows images of the liver and other tissues inside the body. He or she may also do a biopsy to remove a small piece of tissue from the liver. Biopsy is the best way to definitively diagnose PBC.

How is it treated?

Treatment for primary biliary cirrhosis is difficult, because the immune system appears to be involved. As with other forms of cirrhosis, treatment focuses on reducing symptoms, preventing and treating the complications of the disease, and preventing other conditions that may cause additional liver damage.

The medicine ursodiol is usually given soon after a diagnosis of primary biliary cirrhosis. Ursodiol helps move bile out of the liver and into the small intestine. If it is used early enough, ursodiol can improve liver function and may keep you from needing a liver transplant. The medicine may also help you live longer.1

Medicines such as cholestyramine, rifampicin, or naltrexone may be used to help with itching caused by PBC.

If you have fatigue, your doctor will try to find the cause of your tiredness. It may not be caused by PBC. Some medicines have been tried to help with fatigue in PBC, but none have been proved to work well.

PBC can also cause dry eyes and mouth. The best way to help dry eyes is to use eye drops (artificial tears) when your eyes feel dry. A dry mouth can be helped by chewing gum or hard candy to increase saliva. You can also use a saliva substitute. If that doesn't help, there are some medicines that can be used.

Because people with PBC have a high risk of getting osteoporosis, you should have periodic bone mineral density scans. Your doctor may suggest that you take calcium and vitamin D supplements and perhaps a medicine called a bisphosphonate.

Liver transplantation may be the treatment of choice for people who have end-stage primary biliary cirrhosis, although primary biliary cirrhosis can recur after a liver transplant.

Other Places To Get Help

Organizations

American Gastroenterological Association
Web Address: www.gastro.org

American Liver Foundation (ALF)
Web Address: www.liverfoundation.org

National Digestive Diseases Information Clearinghouse
2 Information Way
Bethesda, MD  20892-3570
Phone: 1-800-891-5389
Fax: (703) 738-4929
TDD: 1-866-569-1162 toll-free
Email: nddic@info.niddk.nih.gov
Web Address: www.digestive.niddk.nih.gov
 

This clearinghouse is a service of the U.S. National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), part of the U.S. National Institutes of Health. The clearinghouse answers questions; develops, reviews, and sends out publications; and coordinates information resources about digestive diseases. Publications produced by the clearinghouse are reviewed carefully for scientific accuracy, content, and readability.


References

Citations

  1. Lindor KD, et al. (2009). Primary biliary cirrhosis. Hepatology, 50(1): 291–308.

Other Works Consulted

  • Angulo P, Lindor KD (2010). Primary biliary cirrhosis. In M Feldman et al., eds., Sleisenger and Fordtran's Gastrointestinal and Liver Disease, 9th ed., vol. 2, pp. 1477–1488. Philadelphia: Saunders.
  • Talwalkar JA, Lindor KD (2006). Primary biliary cirrhosis. In M Wolfe et al., eds., Therapy of Digestive Disorders, 2nd ed., pp. 579–587. Philadelphia: Saunders Elsevier.

Credits

By Healthwise Staff
Primary Medical Reviewer Adam Husney, MD - Family Medicine
Specialist Medical Reviewer W. Thomas London, MD - Hepatology
Current as of March 12, 2014

Current as of: March 12, 2014

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