Primary biliary cirrhosis (PBC) is a long-term liver disease that damages the small bile ducts in the liver.
This gradually leads to a build-up of bile in the liver, which then damages the liver.
Symptoms of PBC often include:
- fatigue (extreme tiredness)
- itchiness of the skin
- dry eyes and mouth
Read more about the symptoms of PBC.
Bile and bile ducts
Bile is a liquid produced inside the liver and used by the digestive system to help digest fats and remove waste from the body. It is transported out of the liver into the small bowel through a series of small tubes called bile ducts.
In PBC, for reasons not fully understood, the immune system (the body’s natural defence against infection and illness) gradually damages the bile ducts. Eventually, the damage leads to a blockage that obstructs the flow of bile. The amount of bile in the liver builds up to a dangerous level, causing scarring of the liver (cirrhosis).
Who is affected?
PBC is a rare liver condition. In England and Wales, PBC is estimated to affect one in every 3,000 people. However, the rates of PBC in England are higher than in some other parts of the world. The reason for this is unknown.
PBC mainly affects women (about 90% of all cases). The condition usually occurs in people aged 30-65, and is normally diagnosed after a person has reached 40.
The exact causes of PBC, and why it mainly affects women, are unknown.
Read more about the causes of PBC.
Treating PBC
PBC is usually a progressive condition, which means damage to the liver can steadily get worse over time. The rate at which PBC progresses varies between individuals. It can take decades in many cases. If not treated, the liver can become damaged to such an extent it no longer works. This is known as liver failure and can be fatal.
There is no cure for PBC but there are medicines that help relieve the symptoms and slow the progression of the condition. In cases where there is extensive liver damage, a liver transplant may be required.
However, over the last 20 years, the need for liver transplants as a result of PBC has been decreasing. This may be due to the use of a medicine called ursodeoxycholic acid (UDCA).
Read more about how PBC is treated.
