Primary biliary cirrhosis Dr Trisha Macnair Primary biliary cirrhosis (PBC) is a slow, progressive liver disease in which the bile ducts are attacked by the body's own immune system.

In this article

What causes it? What are the symptoms?

Who's affected? What's the treatment?

Advice and support

In PBC, the bile ducts (tiny tubes throughout the liver that drain bile into the gallbladder) become inflamed and scarred, causing bile acids to leak into the liver tissue, eventually resulting in cirrhosis.

Although the exact cause isn't yet known, genetic factors play a part in this and other autoimmune diseases and it's likely a complex array of genes are involved.

For example, the activity of many genes known to be linked to liver inflammation, fibrosis and regeneration is increased in PBC, but it's not clear whether this genetic overactivity is the result of some other trigger factor, such as an infection or toxin, or is the cause itself.

Symptoms usually appear in mid-life, from 30 years of age, but sometimes there are few symptoms and PBC is discovered accidentally.

Possible symptoms include tiredness, which can be extreme, nausea and poor appetite, and skin problems such as blotchy red palms, easy bruising and intense itching.

People with PBC may also experience dryness of the eyes and mouth, pain in the joints and bones, persistent abdominal pain on the right (up towards the ribs, over the liver), and yellowing of the skin and eyes known as jaundice (characterised by dark urine and pale faeces).

PBC affects nine times more women than men, and is more likely to occur in families where one member is already known to be affected.

Although found all over the world, PBC is more common in northern Europeans, with as many as one in 1,000 women affected (although the illness may be mild and not cause symptoms).

Several genes commonly found in those with autoimmune disease have been identified but no single PBC gene is known.

Diagnosis is easily made using a blood test for anti-mitochondrial antibody (AMA), which is nearly always positive, combined with the pattern of symptoms.

There's no cure, but treatments can reduce symptoms and slow progression of the disease. Antenatal screening isn't possible.

PBC Foundation

Tel: 0131 225 8586
Email: info@pbcfoundation.org.uk
Website: www.pbcfoundation.org.uk

This article was last medically reviewed by Dr Rob Hicks in July 2006.