Symptoms & Causes of Primary Biliary Cholangitis (Primary Biliary Cirrhosis)

What are the symptoms of primary biliary cholangitis?

The most common symptoms of primary biliary cholangitis are

  • feeling tired
  • itchy skin

Other symptoms may include

As the disease gets worse, symptoms may include

Many people have no symptoms when they are first diagnosed with primary biliary cholangitis. Doctors diagnose about 60% of people with primary biliary cholangitis before symptoms begin.3 People with primary biliary cholangitis and no symptoms are identified through blood tests. Some people do not have symptoms for years after they have been diagnosed with primary biliary cholangitis.

Tired woman sitting in front of a laptop.The most common symptoms of primary biliary cholangitis are feeling tired and having itchy skin.

What causes primary biliary cholangitis?

Experts aren’t sure what causes primary biliary cholangitis. Studies suggest that certain genes make some people more likely to develop the disease. In people with these genes, factors in the environment may trigger an autoimmune reaction that causes their immune system to attack the small bile ducts in the liver, causing primary biliary cholangitis.

Possible environmental triggers include

  • infections
  • cigarette smoking
  • exposure to certain chemicals


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. 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 NIDDK is carefully reviewed by NIDDK scientists and other experts.