Condition Basics

What is primary biliary cholangitis (PBC)?

Primary biliary cholangitis (PBC) used to be called primary biliary cirrhosis. It's a type of liver disease caused by damage to the bile ducts in the liver. PBC permanently damages the liver as tissue is replaced with scar tissue. As more scar tissue develops, the structure and function of the liver are affected.

What causes it?

The exact cause of PBC is unknown. But it may be related to a problem with the immune system, which is the body's natural defense system.

What are the symptoms?

Many people who have primary biliary cholangitis do not have symptoms. When symptoms do occur, they may include:

• Itching (pruritus).
• Fatigue.
• Yellowing of the skin and the white part of the eyes (jaundice).
• Discomfort in the upper right part of the abdomen.
• Dry eyes and mouth.

How is it diagnosed?

PBC is suspected based on a medical history, physical exam, and lab tests that show high levels of the enzyme alkaline phosphatase (ALP) and antimitochondrial antibodies (AMA) in the blood. Your doctor may also conduct an imaging test such as an ultrasound, which shows images of the liver and other tissues inside the body. Your doctor may also do a biopsy to remove a small piece of tissue from the liver.

How is PBC treated?

Treatment for PBC focuses on:

• Reducing symptoms.
• Preventing and treating the complications of the disease.
• Preventing other conditions that may cause more liver damage.

Two medicines can be used to treat PBC. They are ursodeoxycholic acid (UDCA) and obeticholic acid (OCA). Both medicines help move bile out of the liver into the small intestine. And OCA decreases the amount of bile acids made by the liver.

Because people with PBC have a high risk of getting osteoporosis, your doctor may suggest that you take calcium and vitamin D supplements. Your doctor may also have you take a medicine called a bisphosphonate.

People who have end-stage PBC may need a liver transplant. But PBC can come back after a liver transplant.

How can you care for yourself?

There are things you can do at home. For example, if your doctor prescribed medicines, take them as instructed. Don't drink alcohol. If your eyes feel dry, try eyedrops. Sugar-free gum or candy can help increase saliva if your mouth is dry. And stay up to date on your vaccines.