Primary biliary cirrhosis

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In primary biliary cirrhosis (PBC) the bile ducts in the liver are attacked by the bodyâ€™s own immune system.

Bile ducts are tiny tubes in the liver that remove a substance called bile and send it to the gallbladder, and on to excretion (leaving the body). Bile usually works to break up the fat in food into small droplets, and helps the body to absorb vitamins A, D, E and K from our diet, so it plays an important part in digestion.

When the bile ducts are attacked, they are damaged and swell, and cannot carry out these functions properly. It also means that toxic (poisonous) substances, which are normally excreted from the body through the bile, are instead kept inside the liver. These substances, such as bile acids and minerals, lead to further damage to the bile ducts and the liver tissue.

The liver can normally heal itself without scarring, but if the damage is continuous and severe, the regrowth process goes wrong and scarring occurs, meaning the liver cannot function properly. This severe damage is known as cirrhosis.

Despite its name, not everybody with primary biliary cirrhosis actually progresses to cirrhosis, because this condition often happens very slowly and with no symptoms for years.

Cirrhosis is not always, as is often assumed, related to the over-consumption of alcohol. It often has quite different causes such as inherited liver diseases. However, those with PBC can often not tolerate alcohol because their liver no longer processes it properly.

Primary biliary cirrhosis is a cholestatic condition (tending to diminish or stop the flow of bile). Because the bile can no longer break down fatty substances fully, it is frequently associated with high levels of cholesterol in the blood, which can be a risk factor for heart disease, and may lead to liver failure. Because the liver cannot carry out its usual functions, there are many other potential complications such as osteoporosis.

Women are nine times more likely than men to have this condition, and it most commonly appears between the ages of 30 and 65.