Primary biliary cirrhosis, orprimary biliary cholangitis(PBC), is a long-term liver disease in which thebile ducts in the liver become damaged.

This gradually leads to a build-up of bile in the liver, which can damageit 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.

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 causesthebile ducts tobecome swollen and scarred, obstructingthe flow of bile out of the liver. Bile then starts to build up in the liver, which damages it and may lead to cirrhosis.

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 isa 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 helprelieve the itchiness associated with it. In cases where there is extensive liver damage, a liver transplant may be required.

and the complications of PBC .

Content supplied by the NHS Website

Medically Reviewed by a doctor on 4 Jan 2017