Primary biliary cirrhosis, or primary biliary cholangitis (PBC), is a long-term liver disease in which the bile ducts in the liver become damaged.
This gradually leads to a build-up of bile in the liver, which can damage it and eventually lead to scarring (cirrhosis).
PBC doesn't always cause any symptoms in the early stages, but some people may experience:
- fatigue (extreme tiredness)
- itchy skin
- dry eyes and mouth
- pain or discomfort in the upper right corner of their tummy
Many people are only diagnosed with PBC after a problem with their liver is picked up during a routine blood test that is carried out for another reason.
Read more about the symptoms of PBC and diagnosing PBC.
What causes PBC?
Bile is a liquid produced inside the liver that is used to help digest fats and remove toxins from the body. It passes out of the liver through small tubes called bile ducts.
In PBC, for reasons not fully understood, the immune system (the body's natural defence against infection and illness) mistakenly attacks the bile ducts.
This gradually causes the bile ducts to become swollen and scarred, obstructing the flow of bile out of the liver. Bile then starts to build up in the liver, which damages it and may lead to cirrhosis.
Read more about the causes of PBC.
Who is affected
It's not clear how many people are affected by PBC in the UK. Estimates vary from around 15,000 to 20,000 people.
The rates of PBC in the UK are higher than in some other parts of the world. The reason for this is unknown.
About 90% of those affected are women and most cases are diagnosed in people aged 40-60, although PBC can be diagnosed at any age from 20 onwards.
How PBC is treated
PBC is a progressive condition, which means the damage to the liver can get steadily worse over time. The rate at which PBC progresses varies between individuals. In some cases, it can take decades.
If not treated, the liver can become damaged to such an extent that it no longer works properly. This is known as liver failure and it can be fatal.
There are medications that can help slow the progression of the condition and help relieve the itchiness associated with it. In cases where there is extensive liver damage, a liver transplant may be required.
Read more about treating PBC and the complications of PBC.