Definition & Facts of Primary Biliary Cholangitis (Primary Biliary Cirrhosis)

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What is primary biliary cholangitis?

Primary biliary cholangitis is a chronic disease in which the small bile ducts in the liver become inflamed and are eventually destroyed. When there are no bile ducts, bile builds up and causes liver damage. Over time, this damage can lead to liver scarring, cirrhosis, and eventually liver failure. Primary biliary cholangitis is believed to be an autoimmune disease in which a person’s own immune system becomes overactive and attacks normal, healthy bile duct cells.

Does primary biliary cholangitis have another name?

Doctors and patients often use the abbreviation PBC for primary biliary cholangitis. The disease used to be called primary biliary cirrhosis.

How common is primary biliary cholangitis?

Researchers estimated that in 2014 about 58 out of every 100,000 U.S. women and about 15 out of every 100,000 U.S. men had primary biliary cholangitis.1

Who is more likely to develop primary biliary cholangitis?

Primary biliary cholangitis is more common in

  • women than in men.
  • people who are middle aged. The average age at diagnosis is 60.2
  • people who are white compared with other racial or ethnic groups.
  • people who have a parent or sibling—particularly an identical twin—with primary biliary cholangitis.
A health care professional talking with a patient who is a woman.Primary biliary cholangitis is more common in women than in men.

What other health problems do people with primary biliary cholangitis have?

People with primary biliary cholangitis may have other autoimmune diseases, including

Women with primary biliary cholangitis may also have frequent urinary tract infections.

What are the complications of primary biliary cholangitis?

Common complications of primary biliary cholangitis include

These common complications can be prevented and treated.

Liver complications

Primary biliary cholangitis can also damage the liver, leading to liver complications.

Cirrhosis. In cirrhosis, scar tissue replaces healthy liver tissue and prevents your liver from working normally. As cirrhosis gets worse, the liver begins to fail.

Portal hypertension. Portal hypertension most often occurs when scar tissue in a liver with cirrhosis partly blocks and slows the normal flow of blood, which causes high blood pressure in the portal vein. However, people with primary biliary cholangitis may develop portal hypertension before they develop cirrhosis. When portal hypertension reaches a certain level, it can cause additional complications, such as

  • swelling in your legs, ankles, or feet, called edema
  • buildup of fluid in your abdomen, called ascites
  • enlarged veins—called varices—in your esophagus, stomach, or intestines, which can lead to internal bleeding if the veins burst
  • confusion or difficulties thinking caused by the buildup of toxins in your brain, called hepatic encephalopathy

Liver failure. Cirrhosis may eventually lead to liver failure, also called end-stage liver disease. With liver failure, your liver is badly damaged and stops working. People with liver failure may require a liver transplant.

Liver cancer. Research suggests that people with cirrhosis caused by primary biliary cholangitis, as well as men with primary biliary cholangitis have an increased risk for liver cancer.

References

Last Reviewed March 2021
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This content is provided as a service of the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), part of the National Institutes of Health. The NIDDK translates and disseminates research findings to increase knowledge and understanding about health and disease among patients, health professionals, and the public. Content produced by the NIDDK is carefully reviewed by NIDDK scientists and other experts.