There is currently no cure for primary biliary cirrhosis (PBC), so the aim of treatment is to:
- prevent liver damage (as much as possible)
- relieve symptoms, such as itchiness
Ursodeoxycholic acid
Ursodeoxycholic acid (UDCA) is a widely used treatment for people with PBC. UDCA is thought to help prevent liver damage by changing the composition of the bile and reducing inflammation (swelling) of the liver. Studies have shown that UDCA can:
- reduce the level of bilirubin the blood – bilirubin is a yellow substance that is present in your blood in excess amounts if something is wrong with your liver or bile ducts
- reduce the levels of liver enzymes – these proteins are produced by the liver if the bile ducts and liver are damaged
- reduce the need for a liver transplant
A 2008 review of a number of studies found that UDCA did not benefit people with PBC. However, it is possible that the studies included were too short to demonstrate the benefit of UDCA over time, or that the dose of UDCA was not high enough.
An alternative treatment that is more effective than UDCA in preventing liver damage has yet to be found.
UDCA is usually taken as a single dose at night. It does not improve the other symptoms of PBC, such as itchy skin or fatigue (extreme tiredness).
Once you start taking UDCA, it is likely that you will need to take it for the rest of you life. Your liver function may be tested to monitor your condition. You will be assessed after a year to see if you need additional treatment. If you are responding fully to the UDCA, then it is likely that the PBC will remain under control and will not shorten your life span.
Side effects
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. After this initial weight gain, most people do not gain any more weight from UDCA. Other side effects include:
- loose stools
- thinning hair
- nausea (feeling sick)
- vomiting
Corticosteroids
PBC sometimes occurs alongside autoimmune hepatitis. This is a very rare cause of chronic (long-term) hepatitis, where the white blood cells attack the liver.
If you have autoimmune hepatitis, you may be treated with corticosteroids as well as UDCA. Corticosteroids are medicines that contain steroids, a type of hormone. They work by suppressing your immune system (the body’s natural defence system) and reducing inflammation.
You will usually be treated with a type of corticosteroid called prednisolone. For more information, see the British Liver Trust: autoimmune hepatitis.
Colestyramine
Colestyramine, which used to be called cholestyramine, is a medicine that is widely used to treat the itchiness associated with PBC.
Colestyramine reduces the amount of bile in the blood, which is thought to be the main cause of the itchiness. It usually comes in sachets of powder that can be dissolved in water or fruit juice. It is a good idea to take the powder with fruit juice, because it has an unpleasant taste.
You may need to take colestyramine for several days before your symptoms begin to improve.
If you are 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 may need to take UDCA at least one hour before colestyramine, or four to six hours afterwards.
This also applies to any other medicines you are taking. Take them at least one hour before colestyramine, or four to six hours afterwards. Your GP should be able to give you further advice about this.
Side effects
Constipation is a common side effect of colestyramine. This usually improves once your body gets used to the medicine. Other side effects of colestyramine include:
- diarrhoea
- nausea
- vomiting
- bloating
If you are 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 circumstances, taking additional vitamin supplements may prove beneficial. Your GP can advise you about whether you would benefit from taking vitamin supplements.
Alternative medicines for itching
See your GP if your itchiness does not improve after taking colestyramine. Alternative medicines are available.
For example, rifampicin, which is usually used as an antibiotic, has been used to treat itching in some people. However, it can cause serious side effects, such as liver failure. For this reason, rifampicin is usually only prescribed by a specialist, such as a hepatologist (liver specialist), not your GP.
It is thought that the female hormone, oestrogen, may make itching worse. Oestrogen is found in some types of hormonal contraceptives, such as the combined contraceptive pill. Therefore, you may wish to consider using a different type of contraceptive, such as condoms.
Oestrogen levels are also increased during pregnancy, so your itching may be worse when you are pregnant.
Managing fatigue
At the moment, no medicines are specifically recommended for treating the fatigue caused by PBC. Your GP may rule out other causes for your fatigue, such as anaemia (a lack of red blood cells) or depression, but fatigue is a difficult symptom to treat.
Although you should exercise whenever possible, you may need to pace yourself and limit your daily activities to a manageable level in order to cope with your fatigue.
Liver transplant
A liver transplant may be recommended if it is thought that the liver damage may reach the stage where your life is at risk.
Planning for a liver transplant often begins before significant damage to the liver has taken place. There are two reasons for this:
- the average waiting time for a liver transplant is 149 days, so it is important to start searching for a suitable donor as soon as possible
- the better your general state of health, the greater the chance of a successful transplant, so a transplant should ideally be carried out while you are still relatively healthy
Like all organ transplants, liver transplants carry a risk of complications. The most serious complication is that your immune system may reject the donated liver, which could be fatal. You may need to take immunosuppressants (medicine to suppress your immune system) for the rest of your life.
Before having a liver transplant, make sure that you discuss the benefits and risks of transplantation with your treatment team, and that you fully understand them. See the Health A-Z topic about Liver transplants for more information.
Success rates
The success rates of liver transplants for people with PBC are favourable. For example, one study of 121 people who had a liver transplant for PBC found that:
- 90% were still alive after five years
- 87% were still alive after 10 years
- 80% were still alive after 15 years
In this study, PBC reoccurred in 15% of people, although some estimates suggest that the condition may reoccur in up to a quarter of people after a liver transplant.
The itchiness always improves after having a liver transplant, but the fatigue often does not improve.
