What is Primary Biliary Cirrhosis?

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Primary Biliary Cirrhosis (PBC) is a slow, chronic liver disease which can cause progressive destruction of the bile ducts in the liver. The bile ducts are the channels through which bile flows. The body attacks the cells lining the bile ducts within the liver as if they are foreign to the body itself, i.e. attacking ones own cells. This damage causes poor drainage of bile acids, which leak outwards and damage the normal liver cells. This causes inflammation and scarring which may, after many years become extensive. This widespread damage and scarring is commonly called cirrhosis.

Many people who have PBC may never develop cirrhosis. Cirrhosis is often assumed to mean damage to the liver caused by excessive alcohol consumption. This is not the case - cirrhosis simply means an advanced form of scarring, which changes the structure of the liver cells, and can be associated with a whole range of liver and biliary diseases.

What causes PBC?

The cause of PBC is at present unknown, although it is thought to be an autoimmune disease whereby the body's immune system malfunctions and turns on itself Another example of this is a common form of thyroid disease in which the thyroid cells are destroyed. Like many liver diseases, it requires further considerable research.

PBC predominantly affects females at a ratio of approximately nine women to every one man, although PBC in men behaves in an identical way to that in women. PBC is more common in families where one member is known to be affected. There are a small number of recorded family cases — often a mother and daughter — and probably about one patient in thirty to seventy has a close relative with the disease.

Laboratory research has indicated that people with PBC display several abnormalities of the immune system, which may be an important factor in determining a cause. What initially sparks off the bile duct damage is unknown. PBC can sometimes become apparent during or just after pregnancy. The trigger could be an infection, a toxin, stress, or some hereditary factor It cannot be spread by sexual intercourse and there is no evidence of infection. Generally, it presents in women aged between thirty and fifty-five although, at present, the peak incidence is fifty to seventy years.

How common is PBC?

It is thought that PBC is most common in Scandinavia, Scotland and North East England, although it is found in people all over the world. In each of these areas it has been shown that as many as one woman in a thousand between the ages of thirty and eighty may have PBC although in many the illness may be silent (asymptomatic) and will never become serious.

As there are no good identifiable risk factors, it is not yet known whether it is possible to prevent the disease.

What are the symptoms of PBC?

Some PBC sufferers display no outward symptoms. Others may suffer from one or more of the following:

• Chronic fatigue and lethargy - mild or severe
• Intense itching of the skin which can affect any part of the body
• Palms mottled with red or pink blotches
• Frequent indigestion or nausea, poor appetite
• Sore joints/bone pain
• Easy bruising or bleeding
• Dry eyes and/or mouth
• Persistent ache or discomfort in the upper right hand side of the abdomen
• Weakness in wrists and/or hands
• Dark urine and/or pale stools
• Diarrhoea
• Jaundice - yellowing of the skin or eyes - is a sign of progression
  

How is PBC diagnosed?

The presence of liver disease is detected by liver function tests on a single sample of blood. People with PBC nearly always have the specific antibody, AMA (antimitochondrial antibody), and finding this in the blood is useful in reaching a diagnosis. As physical obstruction is a relatively common cause of jaundice, quite often the bile ducts need to be X-rayed or visualised by ultrasound to confirm there is no such blockage lower down the biliary tree. Diagnosis of PBC can be confirmed with a biopsy which involves taking a very small sample of cells from the liver with a needle. This also helps to determine the extent of any liver damage.

Is there a treatment for PBC?

Although there is presently no cure for PBC, there have been some major breakthroughs in the treatment of the illness in the last few years.

Ursodeoxycholic Acid (250mg) has recently been approved in the UK for PBC. More information is available from either your family doctor or hospital specialist.

A range of treatments is now available which have been found to ease the symptoms. Drugs that dampen down the body's immune system and slow the progression of the disease are also under investigation.

Like most chronic liver diseases, PBC can impair the capacity of the liver to break down toxic drugs and chemicals. These include prescribed and over the counter medicines, alcohol and even foods. Proper advice on a healthy and balanced diet is, therefore, important, as a good dietary intake is advisable for maintaining health for people with liver diseases. Eating little and often is a good guideline for those who have PBC as this ensures that the bile acids always have something to work on and this helps to alleviate digestive problems.

As the liver may not be creating the correct balance of minerals and vitamins, the diet should be rich in calcium, vitamins and proteins. As in virtually everybody, foods containing unsaturated fats (such as oily fish) are probably preferable to fatty foods, which are high in saturated fats, but a normal fat intake must be continued. If there is steatorrhoea (fatty stools), specific advice must be sought from the doctor

Medication in PBC

Just because you have PBC, it does not mean that you cannot have other problems! You may need, from time-to-time, treatment for other conditions. You should always remember to tell your doctor or dentist, that you have PBC. There are few medicines that you should avoid because of PBC, but the dose may need to be modified.

For simple aches and pains it is, however best to avoid Aspirin and Aspirin containing medicines. If you have minor aches and pains, then Paracetamol up to four tablets a day is usually safe. If the pain persists, or you require stronger pain killers, then you should see your doctor. You may wish to take vitamin tablets and provided you follow the instructions on the manufacturers labels, these do not usually cause any problems.

Itching

Patients with PBC often have itching. The most common medicine used to relieve the itching is called Cholestyramine. The common trade name for this is Questran. Some people on Questran have side-effects such as altered bowel habit, or bloating. If this is a problem, ask your doctor whether Questran Light would be an alternative for you. This is usually associated with fewer side-effects. You must be careful when taking Questran that it does not interfere with absorption of other drugs. Again, you should ask your doctor about the best way of taking the Questran. There are other treatments for itching and if Questran does not work for you, then you should consult your doctor Remember it may take several weeks for Questran to work

Osteoporosis

There are many reasons why people may have osteoporosis (thinning of the bones). Being female, middle-aged and having liver disease are each a good reason for developing osteoporosis. You should talk to your doctor about the risks of developing osteoporosis and measures you can take to reduce the bone loss. Amongst the measures you may wish to consider are increasing exercise (particularly when you are weight-bearing) and taking additional calcium. Again, you should talk to your doctor about the best way of taking calcium, but it is often better to take it is in tablet form at night. Some women may benefit from hormone replacement therapy (HRT). Whilst HRT does have benefits in reducing bone loss and in reducing the chance of a heart attack, there are additional risks from HRT and you should discuss with your doctor whether HRT is appropriate for you and, if so, how you should take it.

Other symptoms which may be more common in PBC include dry eyes and a dry mouth. There is no specific treatment for these side-effects: artificial tears and artificial saliva may give symptomatic relief. Some women have dryness in the vagina and if this causes problems, it can be helped by taking lubricants such as Replens which can be obtained from your chemist.

Liver transplantation in PBC

PBC takes a long time to develop and it affects different people in many different ways. Most have few or no symptoms for many years and some live with a benign form of the illness with little or no discomfort. Many do have symptoms, but never reach end-stage PBC. For those few who do reach this stage, liver transplantation may be considered, especially where quality of life is deteriorating.

If medical treatment no longer controls the disease and the person has severe liver failure, transplantation will be considered. Because PBC progresses slowly, transplantation can be carefully planned and those with PBC have very good results compared to all liver transplant patients. Over six hundred successful liver transplants are performed annually in the UK for various liver diseases, which dramatically improve people's quality of life.

Self care

Supportive care, an altered lifestyle and self-help can be beneficial. For some, changes may have to be considered; e.g., giving up work or particular activities because of tiredness and/or lack of concentration. Tiredness is a physical symptom of PBC and you may find that pacing your daily activities will help to preserve stamina and energy. Exercise is to be encouraged whenever possible, although this should only be undertaken when energy permits. Do remember physical capabilities are very individual and vary widely from person to person. Gentle walking and/or swimming can be beneficial.