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Primary Biliary Cholangitis/Cirrhosis

What is primary biliary cholangitis/cirrhosis (PBC)?

PBC is a chronic disease that can, little by little, destroy some of the tubes linking your liver to your gut. These tubes are called bile ducts.

The bile ducts carry a fluid called bile to your gut (also known as your intestine or bowels). When PBC damages your bile ducts, bile can no longer flow through them. Instead it builds up in the liver, damaging the liver cells and causing inflammation and scarring.

Over the years this damage becomes widespread. The liver becomes less able to repair itself, leading to a condition known as cirrhosis. The damage caused to your liver cells by cirrhosis can result in the failure of your liver carry out all of its jobs properly.

Who is at risk of PBC?

Nine out of ten people who get PBC are women. No one knows why this is. In particular the following women are most at risk:

  • women who are middle aged or older
  • women who have a family history of PBC.

What are the causes of PBC?

PBC is believed to be an autoimmune disease. This is a disease where the body attacks itself. In this case, your body’s defence against germs and infections – the immune system – mistakes the cells lining the bile ducts as ‘foreign’ and attacks them.

Why this happens is still not known. Some medical specialists believe that something may trigger your body into making this mistake. Possible causes of this ‘trigger’ could be:

  • an infection
  • some form of toxin (poison) taken in from the environment.

Occasionally PBC comes to light during or just after pregnancy. It is not clear whether pregnancy may spark it off or, because pregnant women are under close medical supervision, the symptoms of PBC are first noticed during pregnancy.

This external trigger probably starts PBC only in people who are already at risk because they have inherited certain genes from their parents.

Symptoms

Some people with PBC will never get any symptoms of the disease. Clear symptoms of PBC are constant tiredness (for some people this can be severe) and intense itching in any part of the body. Itching, also known as pruritus, may be a result of your liver’s inability to process bile. It is thought that bile acids are not the cause of the itching but rather other chemicals that are retained in the body. As with tiredness, the severity of the itching will vary from person to person. Severity is not an indication of the amount of liver damage.

Other symptoms that may develop usually include the following:

  • dry eyes and/or dry mouth
  • constant or variable ache or discomfort in the upper right hand side, below your ribs
  • indigestion, nausea or poor appetite
  • arthritis (inflammation of the joints)
  • pain in the bones
  • mottled palms with red or pink blotches
  • diarrhoea
  • dark urine and/or pale stools
  • jaundice – yellowing of the skin and whites of the eyes.

Tiredness and itching are generally the first symptoms to appear while jaundice is usually associated with the later stages of the disease.

Diagnosis

Doctors can tell whether you have PBC on the basis of symptoms and a range of tests, including blood tests.

Antibody test: Most people with PBC have something in their blood called antimitochondrial antibody (AMA). An antibody is a chemical made by the body to attack an ‘invader’. Though doctors are not really sure why, the presence of AMA in your blood is an important sign that you have PBC.

Liver function tests (LFTs): This type of test is performed to gain an idea how the different parts of your liver are functioning. The Liver Function Tests are made up of a number of separate examinations, each looking at different properties of your blood.

In PBC, doctors will be looking for increased levels of both alkaline phosphatase (ALP), an enzyme released into the blood by damaged bile ducts, and the immunoglobulin IgM. The liver enzymes alanine transaminase (ALT) and aspartate transaminase (AST) are also monitored, although these are a measure of any leakiness or damage relating primarily to liver cells rather than the bile duct cells.

Ultrasound scan: This is used to check the condition of the bile ducts and to rule out the possibility that your symptoms could be a sign of a different liver problem.

Liver biopsy: If tests show you have PBC you may need a liver biopsy to see how serious the condition is.

Treatment

There are a number of treatments for the symptoms of PBC. Some of them help with any unpleasant symptoms, such as dry eyes, and others slow the progress of the disease. At this time, doctors cannot cure PBC or completely stop its slow destruction of the bile ducts. Because of this PBC is a common reason for liver transplants in the UK.

Managing symptoms

Itching skin: colestyramine (sold as Questran) may be prescribed by your doctor to help ease itching. Taken orally, colestyramine works by preventing re-absorption of the chemicals that cause the itching. It can take days or even weeks before this becomes effective.

Some people taking colestyramine have problems such as changed bowel habits and bloating. Your doctor may prescribe ‘Questran light’ to reduce these side effects. If colestyramine does not help, a hospital specialist may try other medicines such as rifampicin and naltrexone.

Itching is made worse by dry skin so it is very important to keep your skin well moisturised.

Dry eyes and dry mouth: the combination of dry eyes and a dry mouth (sicca syndrome) might be soothed by such treatments as artificial tears and saliva, lubricating gels and oestrogen creams. You may find that lozenges from your pharmacist will help with the dryness in your mouth.

Managing your condition

A medication called ursodeoxycholic acid (URSO, also referred to as UDCA) has helped some people. Made from naturally occurring bile acid, it seems to work through several mechanisms by protecting the liver from the harmful chemicals in the bile. Although early studies failed to show any direct impact on survival rates, new evidence suggests that URSO may help to keep people alive for longer and delay liver transplantation.

A number of other medicines are also now being tried and your medical team may ask for your assistance in this. At present, however, a liver transplant is the only option for people who have advanced PBC.

Will I need a liver transplant?

A liver transplant is usually only recommended if other treatments are no longer helpful and your life is threatened by end stage liver disease. It is a major operation and you will need to plan it carefully with your medical team, family and friends.

Liver transplantation works well for people with PBC. It is possible to get PBC in your new liver, but it may take up to 15 years before the disease becomes significant.

Looking after yourself

PBC affects people in very different ways. Because of this it is very difficult to say what impact PBC may have on your life.

For example, many people with PBC may live with very few problems for many years, even decades. On the other hand, PBC can have a major impact on people’s day-to-day lives early on by causing unpleasant symptoms such as very itchy skin.

If your liver has become damaged it can have a major impact on your health. For example:

  • it may reduce your body’s ability to digest food properly, especially fats
  • it may mean your body does not get enough energy, making you always feel tired (fatigue)
  • you may not cope very well with toxins such as alcohol and some medicines.

Eating a healthy balanced diet and exercising to maintain a healthy weight are recommended. Some people with PBC may need to eat a special diet.  If you have any questions about your diet, talk to your GP. You can ask to be referred to a registered dietician for some personal advice.

Problems with fat: Some people with PBC have difficulty digesting fat. This leads to a type of diarrhoea called steatorrhoea (see above). Some people may also feel sick and bloated. If this happens to you, apart from seeing a registered dietician, you may find that a low fat diet helps to reduce the diarrhoea, abdominal pain and any other discomfort from the steatorrhoea.Fat is essential and should not be cut out of your diet completely without proper advice from a dietician. Fat is an important source of energy, so if you do have to cut it out you may need to eat more carbohydrates for more energy.

Some people may need to have energy supplements and injections of fat-soluble vitamins. Others are prescribed medium chain triglycerides (MCT) which are fats that are easier to digest.

Heartburn:  PBC may cause some people to experience an unpleasant taste in their mouth, or heartburn – a severe pain in the chest that can be mistaken for heart problems. Stomach acids escaping into your food pipe (oesophagus) are the usual cause of this sensation. If you get heartburn you may find that eating small amounts often helps. Avoid big meals and spicy food. It is a good idea to always carry something to eat, preferably food high in carbohydrate such as a barley sugar or boiled sweet.

Thinning bones (osteoporosis): As we get older our bones often become thinner and weaker. This is more common among women, especially after the menopause. PBC may make this worse. You can help to stop this problem by doing plenty of weight-bearing exercise and eating food with plenty of calcium such as skimmed milk.Sometimes, despite these efforts, medicine is needed. You may be advised to take calcium supplements, a ‘bone enhancing’ medicine called a biophosphate or a course of hormone replacement therapy (HRT), for example. Bone loss can be detected with a special type of X-ray called a “Dexa Scan”.

Alcohol and smoking are dangerous to anyone with liver problems.

Complementary and alternative medicines

Many complementary and alternative medicines are available that may ease the symptoms of liver disease. But certain medications used in non-liver related dissease can damage the liver. At present, healthcare professionals are not clear on the role and place of some therapies in managing liver disease. More research needs to be done on the use of these therapies. You may wish to discuss the use of these therapies with your doctor.

Support

Please visit the support section of our website for information on Support groups in your area or visit our Useful Links section for other organisations who may be able to offer information and support.

UK PBC Project

UK-PBC is a collaborative venture between NHS Trusts across the UK, patient groups and UK and international companies, aimed at improving our understanding of PBC and the way it impacts on the lives of patients.
http://www.uk-pbc.com

 

Download Primary Biliary Cirrhosis PBC 0407.pdf

Last Updated November 2008
Reviewed by: Professor Oliver James, Pro Vice Chancellor, Faculty of Medical Sciences, Medical School, University of Newcastle; Professor James Neuberger, DM FRCP, Liver Unit, Queen Elizabeth Hospital, Birmingham; Professor William Rosenberg, Professor of Hepatology, School of Medicine, Southampton University; Colette Thain, MBE, PBC Foundation (UK).

 

 

 

 

Further Information

The Trust has been donated the use of this video discussing the physiology of Primary Biliary Cholangitis/Cirrhosis – it is aimed at medical students but after asking for feedback, some patients have also said they find it useful. It can be quite technical, so please discuss any content that you are unsure about with your medical team.