Understanding Primary Biliary Cholangitis

Reviewed by: HU Medical Review Board | Last reviewed: May 2024

Primary biliary cholangitis (PBC) is a chronic condition that affects the liver. Chronic means that it lasts for life. It is also progressive, which means it can get worse over time. However, treatment may be able to prevent or delay complications.1,2

PBC causes inflammation that impacts the liver’s bile ducts. The liver makes bile to help with digestion. Bile ducts are tube-like structures that transport bile between the organs in your digestive system, starting in the liver and ending in the small intestine, where the bile helps with digestion. If the bile ducts do not work properly, bile can back up in the liver. This can cause serious liver damage over time.1-4

What causes primary biliary cholangitis?

PBC is an autoimmune condition. This means it is caused by the immune system mistakenly attacking the body. In people with PBC, the immune system attacks cells in the bile ducts.1,3

It is not clear why some people develop PBC. Research shows that it may be related to genetics. If someone in your family has an autoimmune condition, you are more likely to develop one. PBC may also be triggered by environmental factors like chemicals or infections.1,3

What are the symptoms of primary biliary cholangitis?

People may not notice symptoms of PBC early on. When they do, the most common early symptoms of PBC are extreme tiredness (fatigue) and itchy skin (pruritus). Other, less common, symptoms include:1,3,5

  • Stomach pain
  • Dry mouth or eyes
  • Yellow skin, also called jaundice
  • Bone, muscle, and joint pain
  • Patches of dark skin
  • Small yellow or white bumps under the skin or around the eyes

As the disease progresses, it can start to impact more of your body systems, which may cause complications. When PBC affects digestion, it is harder to break down fats. This is called fat malabsorption.1,3,5

PBC also can cause:1,3,5

  • Liver scarring (cirrhosis)
  • Thinning of the bones (osteoporosis)
  • High blood pressure in veins near the liver (portal hypertension)

Who does primary biliary cholangitis impact?

PBC is not a common disease. It mostly impacts women, and it is most common in women between the ages of 45 and 65. In the United States, about 65 in 100,000 women and 15 in 100,000 men have PBC.1,3

How is primary biliary cholangitis diagnosed?

Some people may not have any symptoms of PBC. In this case, the condition might be accidentally discovered from routine test results. If your doctor suspects PBC based on existing symptoms, they will recommend an antimitochondrial antibody (AMA) test. This blood test looks for high levels of an antibody associated with PBC. About 95 percent of people with PBC have a positive AMA test.1,3,6

If your AMA test is positive, your doctor may do other tests to rule out other conditions and show whether your liver is damaged. These tests may include:1,3,6

  • Liver function tests
  • Liver biopsies
  • Imaging tests
  • Other blood tests

How is primary biliary cholangitis treated?

There is no cure for PBC, but medicine can slow down disease progression. Ursodeoxycholic acid (UDCA) is a type of bile salt treatment that helps move bile out of the liver. It helps about half of people with PBC. Another biosalt for PBC is called obeticholic acid. Other types of drugs that target certain processes in the body may also help slow down PBC.1,3,7

Your doctor may also recommend other drugs to treat specific symptoms commonly linked to PBC. For example, antihistamines may help with itchy skin. If your liver function continues to decline, you may be put on a liver transplant waiting list. While PBC can return after a liver transplant, it often progresses more slowly following a transplant.1,3

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