What Is Primary Biliary Cholangitis?

Primary biliary cholangitis (PBC) is a chronic liver disease that affects the small bile ducts in the liver. These ducts are responsible for transporting bile, a fluid that helps digest fats, out of the liver and into the small intestine. In PBC, the bile ducts become inflamed and damaged, which can lead to liver damage and scarring (cirrhosis).

PBC is a rare disease, affecting about 1 in 1,000 people in the United States. It is more common in women than in men, and it typically occurs between the ages of 30 and 60.

The exact cause of PBC is unknown, but it is thought to be an autoimmune disease. This means that the body's immune system mistakenly attacks its own tissue, in this case the bile ducts.

There is no cure for PBC, but treatment can help slow the progression of the disease and prevent complications. Treatment may include medications to reduce inflammation, ursodeoxycholic acid (a bile acid that can help protect the liver cells), and lifestyle changes such as avoiding alcohol and maintaining a healthy weight.

In severe cases of PBC, liver transplant may be necessary.

PBC is a serious disease, but it can be managed with proper treatment. Most people with PBC are able to live long, full lives.