Primary Biliary Cholangitis (PBC)

What is primary biliary cholangitis (PBC)?

Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is a chronic disease of the liver characterized by a slow, progressive damage to the bile ducts located in the liver. Bile is a substance produced by the liver that is responsible for the digestion of fat and fat soluble vitamins in the intestine, and damage to these ducts prevents the flow of bile out of the liver and causes a buildup of bile and other substances within the liver.  The accumulation of these substances can lead to scarring (fibrosis) and advanced scarring (cirrhosis), which ultimately can lead to liver failure.

What are the symptoms of PBC?

Many people can go years without any symptoms of PBC. Early symptoms include:

• Fatigue, often severe
• Persistent itching of the skin, especially on the bottoms of the feet
• Darkening of the skin
• Small white bumps under the skin mainly around the eyes
• Dry mouth
• Muscle and joint pain

Advanced symptoms include:

• Yellowing of the skin (jaundice)
• Swelling of feet and lower legs
• Chronic diarrhea
• Internal bleeding of stomach and throat
• Fragile bones
• Underactive thyroid
• Swollen distended abdomen due to fluid accumulation (ascites)

What causes PBC?

The exact cause of PBC is unknown. The condition is mainly thought to be due to problems with the immune system that cause the body to attack the bile ducts of the liver, and it is thought that an environmental cause may trigger this autoimmune response.  Examples of these triggers include exposure to toxins or infection. Though not known to be an inherited disease, PBC is more common in individuals with a family history. PBC is not related to alcohol consumption.

Are there treatment options available for PBC?

There is no known cure for PBC. Current available treatments can dramatically slow disease progression, help with symptoms, and help avoid complications, so early detection is key. Treatments include:

• Medications to help with the elimination and proper traveling of bile through the liver. These medications include Ursodiol (brand name, Actigall) and Ubeticholic acid (brand name, Ocaliva)
• Medication to help with itching
• Vitamin supplements to help with jaundice
• Avoidance of alcohol
• Liver transplant for advanced cases

Where can I find more information about PBC?

• American Liver Foundation
• Canadian Liver Foundation
• Mayo Clinic
• NIH
• PBCers

PBC Clinical Practice Guidelines