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

What are the symptoms of primary biliary cholangitis?

The most common early symptoms of primary biliary cholangitis are

  • feeling tired
  • itchy skin

Other common early symptoms may include

As the disease gets worse, symptoms may include

  • weakness
  • nausea
  • weight loss
  • diarrhea
  • swelling in the legs, ankles, or feet
  • dark urine
  • yellowish eyes and skin, called jaundice
  • raised patches on the skin called xanthomas

Many people have no symptoms when they are first diagnosed with primary biliary cholangitis. Doctors diagnose up to 6 in 10 people with primary biliary cholangitis before symptoms begin.2 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.

What causes primary biliary cholangitis?

The exact cause of primary biliary cholangitis is unknown. Experts think that a person’s tendency to have an overactive immune system, which may be genetic, and unknown environmental triggers play a role in causing primary biliary cholangitis. Possible environmental triggers include:

  • infections
  • cigarette smoking
  • exposure to certain chemicals


March 2017

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.

The NIDDK would like to thank:
John Moore Vierling, M.D., Baylor College of Medicine