Primary biliary cirrhosis (PBC) is a type of chronic liver disease that is poorly understood. It can cause:
- fatigue,
- itchiness, and
- scarring of the liver (cirrhosis).
PBC gradually damages the bile ducts and eventually the flow of bile becomes restricted. This leads to a build-up of bile in the liver, and this build-up damages the liver.
Bile and bile ducts
Bile is a yellowish-green liquid that is produced inside the liver. The bile is transported out of the liver into the gallbladder through a series of small tubes, or channels, called bile ducts. Bile is used by the digestive system to help digest fats.
In PBC, the immune system gradually damages the bile ducts. The reason for this is unclear. 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, leading to the liver becoming scarred.
How common is PBC?
PBC is a rare liver condition. In England, it's estimated that for every million people, two to 24 will develop PBC in any given year. The rates of PBC in England are much higher than in other parts of the world, but the reason for this is not known.
PBC mainly affects women (about 90% of all cases) and it occurs mainly during middle age. The average age at which symptoms begin is 50, although new cases have been reported in people as young as 20 and as old as 90.
The exact causes of PBC, and why it mainly affects women, are unknown.
Prognosis
PBC is a progressive condition, which means that the associated damage to the liver will get steadily worse over time. If it's not treated, the liver can become damaged to such an extent that it no longer works (liver failure). This can be fatal (cause death).
There is no cure for PBC, but there are medications that can help to relieve symptoms and slow the progression of the disease. In cases where there is extensive liver damage, a liver transplant may be required to prolong the person's life.
