Frequently Asked Questions About Primary Biliary Cholangitis


Marijke Vroomen Durning, RN

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Primary biliary cholangitis (PBC), sometimes called primary biliary cirrhosis, is a progressive, chronic liver disease. Researchers believe PBC is an autoimmune disorder that causes your body to attack healthy cells in the bile duct.

Learn about some of the most common questions people have about primary biliary cholangitis.

How does PBC affect the liver?

Bile is a fluid excreted by your liver to help digest food and absorb fat. It flows from your liver to your digestive system through your bile ducts. Inflamed and scarred bile ducts caused by PBC can restrict the flow and the bile backs up into your liver, causing liver damage. This is called cirrhosis. If the ducts are completely blocked, no bile can flow through.

What are the symptoms of PBC?

The signs and symptoms of primary biliary cholangitis aren’t always noticeable at first. The disease progresses slowly and it may take a few years for symptoms to be obvious. Early symptoms may include:

  • Itchy skin

  • Fatigue

  • Dry eyes

  • Dry mouth

As the disease progresses, symptoms may include:

  • Abdominal pain, especially in the upper right area

  • Generalized pain in your muscles, joints or bones

  • Poor appetite

  • Weight loss

  • Jaundice (yellow tint to your skin and the white of your eyes)

  • Dark yellow colored urine

  • Swelling of your legs, ankles or feet, and abdomen

  • Raised patches of skin around your eyes or in the creases of your skin around your joints

  • Elevated cholesterol levels in your blood

  • Greasy diarrhea

  • Hypothyroidism (underactive thyroid)

What are the risk factors for developing PBC?

Primary biliary cholangitis affects more women than men. About 65 out of every 100,000 people diagnosed with PBC are women while about 12 out of 100,000 are men. Most people diagnosed with the disease are between the ages of 30 and 60 years. Having a parent or sibling with PBC may increase your risk of developing it. There are also some environmental triggers that could cause PBC. They include infections (bacterial, fungal or parasitic), smoking cigarettes and exposure to certain environmental toxins.

How is PBC diagnosed?

Primary biliary cholangitis is diagnosed based on your medical history and the results of several tests. Blood tests check liver enzymes and other blood levels, such as your cholesterol, as well as signs of autoimmune disease. Imaging tests, such as ultrasounds and magnetic resonance imaging (MRI) may show abnormalities in your liver or the bile ducts. An endoscopic retrograde cholangiopancreatography may also be performed. This test involves inserting an endoscope (long tube with a camera on one end) through your mouth to your intestine. The camera sends back images of the ducts. A liver biopsy may also be recommended. Your doctor would remove a tiny piece of liver tissue to be examined in a laboratory.

Are there any complications related to PBC?

If you have primary biliary cholangitis, you are at risk for complications such as high cholesteroland vitamin deficiencies because your liver cannot metabolize some vitamins from the food you eat. Other possible complications include:

  • Gallstones and stones in your bile ducts

  • Osteoporosis (thinning of the bones)

  • Portal hypertension, an increase in pressure in your portal vein, the blood vessel that carries blood to your liver from your gallbladder, pancreas, spleen and gastrointestinal system

  • Enlarged spleen

  • Varices, enlarged veins in your stomach and esophagus, which can cause bleeding

  • Liver cancer

  • Confusion

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Medical Reviewers: William C. Lloyd III, MD, FACS Last Review Date: Jan 11, 2018

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View Sources

Medical References

  1. Primary biliary cirrhosis. Mayo Clinic.
  2. Primary Biliary Cholangitis. American Liver Foundation.
  3. Primary Biliary Cirrhosis (PBC). Merck Manual.
  4. Primary Biliary Cholangitis (Primary Biliary Cirrhosis). National Institute of Diabetes and Digestive and Kidney Diseases. Part of the National Institutes of Health.

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