What causes PBC?

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The exact cause is not known. It is thought that PBC is an autoimmune disease. The immune system normally attacks bacteria, viruses, and other 'germs' with antibodies, white blood cells, etc. In people with autoimmune diseases, the immune system attacks tissues of the body. It is not clear why this happens. Some people seem to have a tendency to develop autoimmune diseases. In such people, something might trigger the immune system to attack the body's own tissues. The trigger is not known.

In people with PBC, the immune system attacks cells that line the small bile ducts in the liver. This causes inflammation and damage in and around the bile ducts in the liver.

People with PBC have an increased chance of developing other autoimmune diseases. For example, Sjogren's Syndrome, thyroid disease, Raynaud's Phenomenon, scleroderma.

Who gets PBC?

PBC is an uncommon condition. It affects about 1 in 1000 people in the UK. About 9 in 10 cases occur in women. It most commonly develops in women aged between 30 and 60.

By Rob Mansfield