The aim of treatment for PBC is to slow down the liver damage and reduce your symptoms.
All types of liver disease, including PBC, can be helped by following some general health advice.
Following this advice could:
The damage to your liver can affect its ability to process certain medications.
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 medications you should avoid, and inform any health professional treating you that you have PBC.
Ursodeoxycholic acid (UDCA) is the main treatment for people with PBC.
It can help prevent or delay liver damage in most people – particularly if you start taking it in the early stages of the condition – although it doesn't improve symptoms such as itchy skin or fatigue.
Once you start taking UDCA, it's likely you'll need to take it for the rest of your life.
Regular blood tests may be carried out to monitor your liver function, and you'll be assessed after a year to see if you have responded well to it. If not, you may need additional treatment.
One possible side effect of UDCA is weight gain. People gain an average of 2.3kg (5lbs) during the first year of taking the medication, although most people don't gain any more weight after this.
Other side effects can include diarrhoea, nausea, vomiting and thinning hair.
Obeticholic acid (OCA) is a new treatment that works by improving bile flow and reducing inflammation.
It may be offered as an option for treating PBC, either:
People who may need OCA will first need assessment by a specialist team with experience in treating PBC.
One possible side effect of OCA is itching. If itching is a problem, your dose may be lowered or you may be offered medication to treat the itching.
People with very advanced liver disease may be prescribed a lower dose of OCA, or it may not be recommended.
Colestyramine (previously called cholestyramine) is a medicine widely used to treat the itchiness associated with PBC.
It usually comes in sachets of 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 up to a few weeks 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. Your 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 diarrhoea.
If you're taking colestyramine on a long-term basis, the medicine may affect your body's ability to absorb vitamins A, D and K from food.
In such cases, taking additional vitamin supplements may be beneficial. Your GP can advise you on whether you would benefit from taking vitamin supplements.
See your GP if your itchiness doesn't improve after taking colestyramine.
Alternative medicines are available, such as an antibiotic called rifampicin and a medication 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.
At the moment, no medications are available to specifically treat fatigue associated with PBC.
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, sleep hygiene measures may help. These include avoiding caffeine, nicotine and alcohol late at night, and maintaining a comfortable sleeping environment.
If you have a dry mouth and eyes, your GP may recommend eye drops containing "artificial tears" or saliva substitute products.
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 because:
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. The 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.
Studies have found more than 9 out of 10 people who have a liver transplant for PBC are still alive after a year, and more than 8 out of 10 will live at least another 5 years.
It's now common for people to still be healthy and well more than 20 years after a liver transplant.
There's a risk of PBC developing in your new liver, but this isn't usually a major concern because it often takes a long time to develop.