What is primary biliary cirrhosis?

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PBC is a condition that affects the bile ducts. It is called:

Primary - because the cause is not known. (That is, it is not 'secondary' to any known cause such as alcohol or poison.)
Biliary - because it affects the bile ducts.
Cirrhosis - because as the disease progresses it can damage the liver cells and cause scarring of the liver. Cirrhosis means scarring of the liver and has various causes. PBC is an uncommon cause of cirrhosis.

In PBC, inflammation develops around the small bile ducts within the liver. The inflammation can slowly damage the bile ducts. The bile ducts gradually become blocked due to the inflammation and damage. Bile is not able to flow down any ducts that become blocked. This process tends to get worse very slowly, and the number of damaged and blocked bile ducts gradually increases. If bile cannot flow down bile ducts, the bile builds up in the liver cells. In time, the liver cells also become damaged by the accumulation of bile. Bile may also spill into the bloodstream.

As damage to the liver cells becomes worse, scarring (cirrhosis) may gradually develop in the liver. In time, cirrhosis can cause liver failure and other serious problems. (There is a separate leaflet called 'Cirrhosis' which describes cirrhosis in more detail.)

In the early stages of the disease, the main problem is the build up of substances in the liver and bloodstream that are normally drained into the duodenum as part of bile. Cirrhosis occurs only in the later stages of the disease. The rate of decline from early stages of the disease to the later more serious stages of the disease can vary from case to case.

In fact, the term PBC is controversial because not all people with PBC develop cirrhosis. Also, if cirrhosis does occur, it typically develops several years after the disease first begins (after several years of inflammation of the bile ducts).