Treatment - Primary biliary cholangitis (primary biliary cirrhosis)

The aim of treatment for PBC is to slow down the liver damage and reduce your symptoms.

Self-help

All types of liver disease, including PBC, can be helped by following some general health advice.

You should:

• stop smoking
• eat a balanced diet
• stop drinking alcohol, or only drink a small amount, depending on how much liver damage you have

Following this advice could:

• help prevent PBC getting worse
• mean all the treatments for PBC are available to you if you need them – for example, a liver transplant

Avoid certain medicines

The damage to your liver can affect its ability to process certain medicines.

This means you may need to avoid certain medicines – for example, non-steroidal anti-inflammatory drugs (NSAIDs) like aspirin and ibuprofen.

Ask your doctor about any medicines you should avoid. Tell a pharmacist or any healthcare professional treating you that you have PBC.

Ursodeoxycholic acid

Ursodeoxycholic acid (UDCA) is the main treatment for PBC.

It can help delay liver damage in most people, particularly if you start taking it in the early stages of the condition. But it does not improve symptoms such as fatigue.

Once you start taking UDCA, it's likely you'll need to take it for the rest of your life.

You may need regular blood tests to monitor your liver function, and you'll be assessed after a year to see if you've responded to UDCA. If not, you may need additional treatment.

Side effects

Side effects of UDCA can include diarrhoea and feeling and being sick.

Obeticholic acid

Obeticholic acid (OCA) is a treatment that works by improving bile flow and reducing inflammation.

It may be offered as an option for treating PBC, either:

• in combination with UDCA – if UDCA is not working well enough
• on its own – for people who cannot take UDCA

People who may need OCA will first need to be assessed by a specialist team with experience in treating PBC.

Side effects

A possible side effect of OCA is itching. If itching is a problem, your dose may be lowered or you may be offered medicine to treat the itching.

If you have very advanced liver disease, you may be prescribed a lower dose of OCA.

Treating itchiness

Colestyramine (previously called cholestyramine) is a medicine widely used to treat the itchiness associated with PBC.

It usually comes in sachets as a powder that can be dissolved in water or fruit juice. It's a good idea to take the powder with fruit juice because it has an unpleasant taste.

You may need to take colestyramine for a few days before your symptoms begin to improve.

If you're taking UDCA as well as colestyramine, you should avoid taking them at the same time because colestyramine will affect how your body absorbs UDCA.

You should take UDCA at least 1 hour before colestyramine, or 4 to 6 hours afterwards.

This also applies to any other medicines you're taking. A GP can give you further advice about this.

Constipation is a common side effect of colestyramine, although this usually improves once your body gets used to the medicine. Other side effects can include bloating and headache.

If you're taking colestyramine long term, it may affect your body's ability to absorb vitamins A, D and K and folic acid from food. Taking additional vitamin supplements may be beneficial.

A GP can advise you about whether you would benefit from taking vitamin supplements.

Alternative treatments for itching

See a GP if your itchiness does not improve after taking colestyramine.

Alternative medicines are available, such as an antibiotic called rifampicin and a medicine called naltrexone.

These are usually only prescribed by the specialist that looks after your PBC – for example, a gastroenterologist or hepatologist (liver specialist).

Regularly using moisturisers to stop your skin becoming dry may also help reduce itchiness.

Managing fatigue

No medicines are currently available to specifically treat fatigue associated with PBC.

A GP may want to rule out and treat any other possible causes for your tiredness, such as iron deficiency anaemia (a lack of red blood cells), sleep apnoea, or depression.

You should try to exercise whenever possible, but you may need to pace yourself and limit your daily activities to a manageable level.

Adjusting your daily routine around fatigue (which is often worse later in the day) can make a big difference to your quality of life.

If you have difficulty sleeping (insomnia), sleep hygiene measures may help. These include avoiding caffeine, nicotine and alcohol late at night, and ensuring you have a comfortable sleeping environment.

Treating dry mouth and eyes

If you have a dry mouth and eyes, a GP may recommend eyedrops containing "artificial tears" or saliva substitute products.

You should take care of your teeth and gums and visit a dentist regularly because having a dry mouth can increase your risk of tooth decay.

Liver transplant

A liver transplant may be recommended if it's thought the liver damage may eventually put your life at risk.

Planning for a liver transplant often begins before significant damage to the liver has taken place. This is because the waiting time for a liver transplant may be longer than 3 months for adults, so it's important your specialist doctor considers assessment and listing for transplantation as soon as possible.

Having a liver transplant will cure the itchiness and other symptoms, but you may still have fatigue.

Like all organ transplants, liver transplants carry a risk of complications. Your immune system may reject a donated liver, which could be life threatening.

Medicines that suppress the immune system are very effective at preventing this, but they need to be taken for life to reduce this risk.

Most people live more than 10 years after having a liver transplant.

But having a liver transplant does not always completely cure PBC. Some people who have a liver transplant for PBC develop the condition in their new liver. This is known as recurrent PBC.