Primary Biliary Cholangitis

Primary biliary cholangitis (PBC) is a liver disease that affects the bile ducts that run through your liver. It slowly degrades those bile ducts, making it harder for bile to flow through. Bile backs up inside your liver, which damages the tissues. Scar tissue gradually replaces healthy tissue and your liver gradually loses its functionality. This is known as cirrhosis. PBC was formerly known as primary biliary cirrhosis.

What does “primary biliary cholangitis” mean?

“Cholangitis” means inflammation in your bile ducts. “Biliary” means of the bile ducts, and “primary” means original. This means that the disease itself is the original cause of inflammation in your bile ducts. There isn’t some other condition causing inflammation, such as an infection or a blockage. Since the inflammation isn’t responding to anything in particular, it doesn’t know when to stop.

How does primary biliary cholangitis affect my body?

Chronic inflammation degrades your body’s tissues over time. While temporary inflammation can be part of the normal healing process, constant inflammation causes overactive healing in the form of scarring. When your bile ducts are scarred, they become narrowed and distorted, which obstructs the flow of bile. Bile backs up into your liver, causing inflammation and eventually, scarring (cirrhosis).

Is primary biliary cholangitis serious?

Primary biliary cholangitis is a chronic and progressive condition, which means it doesn’t go away and can get worse over time. It progresses slowly through several stages. At the beginning, you might not notice it at all. But in the end, it can cause liver failure, which is fatal without a liver transplant. Fortunately, medication helps slow the progress of the disease, and not everyone will reach this stage.

What is the difference between primary biliary cholangitis vs. primary sclerosing cholangitis?

The two conditions are very similar. One major difference is that primary biliary cholangitis (PBC) affects only your intrahepatic bile ducts, the bile ducts within your liver. Primary sclerosing cholangitis (PSC) affects all of your bile ducts, including your extrahepatic ducts. The two conditions also tend to affect different populations. And while you can treat PBC with medication, PSC has no effective treatment.

Who does primary biliary cholangitis affect?

It primarily affects women and people assigned female at birth (AFAB), by a ratio of 10-to-1. In the U.S., it affects around 60 people AFAB per 100,000 and 15 people assigned male at birth (AMAB) per 100,000. Most are diagnosed after the age of 40. It’s more common in Scotland, Scandinavia and Northeast England. It’s also more common in people with a personal or family history of autoimmune disease, suggesting it might be a partly genetic disorder.