Primary Biliary Cholangitis (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.

Nine out of ten people who get PBC are women, although there are reports that more men are being diagnosed of late. 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 other autoimmune diseases, and PBC in particular.

Large studies have shown that smoking is also a risk factor for developing PBC.

Doctors know that PBC is an autoimmune disease that occurs in some people because of a combination of subtle differences in their genetic make-up and environmental factors – so where you live and the environment around you, but also cigarette smoke and other toxins. In an autoimmune disease, the body’s defence
mechanism (the immune system) wrongly identifies its own cells as ‘invaders’ and attacks them. In PBC, the immune system attacks the cells lining the bile ducts.

Doctors understand many of the very subtle genetic changes that are associated with developing PBC, and these are the same gene changes that are seen in many other autoimmune conditions. However, experts are still trying to understand more about the environmental factors that play a part in PBC. Smoking is known to be a risk factor, and some doctors believe that an infection or other environmental toxin may act as a trigger.

Very occasionally, PBC is diagnosed during or just after pregnancy. However, it’s not clear whether pregnancy itself triggers the condition or simply that pregnant women are under close medical supervision, meaning it’s more likely the symptoms of PBC are picked up during this time. Doctors don’t know why PBC is nearly only ever seen in adults, or why it’s much more common in women than in men.

Drinking alcohol does not cause PBC.

PBC: symptoms & Diagnosis

Symptoms

Some people with PBC will never get any symptoms of the disease. 

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.

  • 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.

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.

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

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.

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.

 

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.

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.

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Download current edition:  Primary biliary cholangitis PBC/02/19.pdf

Special thanks to:Professor Gideon Hirschfield, consultant hepatologist with a special interest in PBC, Toronto Centre for liver disease, Canada; Dr Imran Patanwala, consultant hepatologist, Royal Liverpool University hospital; Lay reviewer Loo How (living with PBC)

Further Information

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.

Many complementary and alternative medicines are available that may ease the symptoms of liver disease. But certain medications used in non-liver related disease 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.

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

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