Primary biliary cholangitis (PBC), formerly named primary biliary cirrhosis, is a chronic liver disease resulting from the destruction of the bile ducts in the liver, which are called intrahepatic bile ducts.

Of the many functions the liver performs, the liver also produces bile that travels through bile ducts to your small intestine, where the bile aids in the digestion process.

If these ducts are destroyed, bile builds up in the liver, causing inflammation and damage such as scarring. Mild stages of scarring can be categorized as fibrosis but can lead to severe scarring such as cirrhosis.

Cirrhosis has many associated complications. Cirrhosis impairs liver function, meaning the liver is no longer able to do the necessary functions the body needs. The severity or level of liver scarring from cirrhosis is categorized as either compensated or decompensated.

The American Liver Foundation reports, “Women are nine times more likely than men to develop PBC. The disease most often develops during middle age and is usually diagnosed in people between 35 to 60 years old. There appears to be a genetic component to developing PBC, as it’s more common among siblings and in families where one member has been affected.” 

What Causes Primary Biliary Cholangitis?

Liver specialists do not know for sure what causes PBC, but many believe it to be an autoimmune disease of the liver. Autoimmune disease is where the body’s own immune system turns against its own cells or parts of the body.

The Mayo Clinic states, “The liver inflammation seen in primary biliary cholangitis starts when certain types of white blood cells called T cells or T lymphocytes start to collect in the liver.”


The American Liver Foundation states these symptoms of PBC:

  • Fatigue
  • Itching of the skin also known as pruritus
  • Abdominal pain
  • Darkening of the skin
  • Small yellow or white bumps under the skin or around the eyes
  • Dry mouth and eyes
  • Bone, muscle and joint pain

With the progression of cirrhosis, other symptoms may develop such as:

  • Yellowing of the skin (Jaundice)
  • Swelling of the legs and feet (edema)
  • (Ascites) accumulation of fluid in the abdomen
  • Internal bleeding in the upper stomach and esophagus from enlarged veins (varices)
  • Thinning of bones (osteoporosis) can also happen in the later stage of PBC.
  • Cirrhosis also causes a high risk for liver cancer.


There is no cure for PBC, but there are medications to help slow the progression of the disease. Medications that are a naturally occurring bile acid known as UDCA help move bile out of the liver and into the small intestine.

This type of medication is taken daily for life. Along with UDCA medication, there are other things a patient can do to help, such as:

  • Drink plenty of water
  • Avoid alcohol of all types
  • Stop smoking
  • Eat a low-sodium diet
  • Reduce excess weight if you are overweight
  • Reduce stress
  • Practice mild exercise
  • Maintain good skincare
  • Have regular dental checkups

This entry was originally published in Life Beyond Hep C, and is reprinted with permission.