What is the treatment for PBC?

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There is no cure for PBC. Treatments aim to ease symptoms and to slow the progression of the disease. A liver transplant can be life-saving in the later stages of the disease.

Treatments to ease symptoms

Itch can be a distressing symptom and can be difficult to treat.
Cholestyramine(trade name Questran) is the most commonly used medicine for itch. It works by 'binding' to bile in the gut. (When bile gets into the gut, it travels down to the large intestine where some of it is absorbed back into the bloodstream, and goes back to the liver to be reused. As cholestyramine binds to bile in the the large intestine, it stops this 'reabsorption' and so more bile than usual is passed out with the faeces (stools). This helps to reduce a build up of bile in the liver and bloodstream which often eases itch.) There may be a delay of 1-4 days after starting treatment before itch improves. Other bile-binding medicines are sometimes used.

Other medicines are sometimes used to ease itch if the above are not helpful. For example, rifampacin, naloxone or phenobarbitone. It is not clear how these work, but they do in some cases. UDCA (see below) may also relieve itch. Plasmapheresis (like a plasma exchange) has been used in some severe cases of persistent, severe itch.

Dry skin can make itch worse, so using liberal amounts of moisturiser is useful if you have dry skin. No medicines seem to ease tiredness which is often a main symptom.

Treatments to slow the progression of the disease

There is no medicine that stops or reverses the disease. However, some medicines may slow down the progression of the disease in some cases.
Ursodeoxycholic acid (UDCA) is the most common medicine used with the aim of slowing the progression of the disease. However, research trials indicate that, at best, it only has a modest effect of slowing the disease in some people, and may not help at all in others. However, it may ease itch. It works by changing the make-up of bile in the liver which may reduce the harmful effect of bile on the liver cells.
Immunosuppressive medicinesare sometimes used. For example, penicillamine, azathioprine, methotrexate, cyclosporin, and steroids. These work by suppressing the immune system. But again, there is little clear cut evidence to say how effective they are in treating PBC. They all have a risk of causing significant side-effects.

Your specialist will advise on the pros and cons of trying any particular medicine.

Treatments of associated diseases

As mentioned, various other autoimmune diseases are more common in people with PBC. So, you may need treatment for these too.

Osteoporosis is more common in women with PBC. The prevention and treatment of osteoporosis is the same as for any other woman and is discussed in a separate leaflet called Osteoporosis.

Treatment of cirrhosis and liver failure

See separate leaflet called Cirrhosis for details of cirrhosis.

A liver transplant is an option if the liver becomes badly damaged and fails. This is a major operation and not to be undertaken lightly. However, it can a life-saving measure and the results are often very good. Some people with severe itch which has not responded to any other treatment have a liver transplant even if their liver is not too badly damaged.