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Primary Biliary Cirrhosis (PBC)

Home > Patients & Visitors > Diseases & Conditions > Abdominal > Primary Biliary Cirrhosis (PBC)

Definition

Primary Biliary Cirrhosis (PBC) is a chronic disease that can, little by little, destroy some of the tubes (called bile ducts) which links your liver to your gut.

 

The bile ducts carry fluid called bile to your intestine.

 

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 to carry out all of its function properly.

 

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Symptoms

Some people with PBC will never experience any symptoms of the disease. Clear symptoms of PBC are constant tiredness (this can be severe for some people) and intense itching in any part of the body. As with tiredness, the severity of the itch 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
• diarrhoea
• dark coloured urine and/or pale stools
• jaundice - yellowing of the skin and whites of the eyes.

 

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

 

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Why is it called PBC?

It is called primary biliary cirrhosis because the disease attacks the bile (biliary) ducts inside the liver. This leads to scarring (fibrosis) and then to cirrhosis. In this case, ‘primary' does not mean first. Instead, it indicates that there is no known reason for this damage to happen.


The name PBC may be a little misleading. Many people do not have cirrhosis when they are first diagnosed or for many years afterwards. Indeed, many people with PBC never go on to get cirrhosis.

 

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Who are at risk of PBC?

Nine out of ten people who get PBC are women. In particular, the following women are most at risk:

 

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

 

There are no evidence that PBC can be passed on to others by simple contact, kissing, blood or sexual contact.

 

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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. It is still not known why this happens but some medical specialists believe that something may have triggered your body into making this mistake.

 

Possible causes of this ‘trigger' could be:


• an infection
• some form of environmental toxin (poison)

 

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Testing for PBC

Antibody test

 

Most people with PBC have antimitochondrial antibody (AMA) in their blood. An antibody is a chemical made by the body to attack an ‘invader'. It is known that the presence of AMA in your blood is an important sign that you have PBC.

 

Liver function tests (LFTs)

 

This test is performed to gain an idea on how the different parts of your liver are functioning. The liver function test is 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 alamine 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.

 

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Ultrasound scan

Ultrasound is the same technology used to confirm that all is well in pregnancy. Gel will be applied to your skin, which may feel slightly cold. A probe, like a microphone, will be moved across your skin to send sound waves into the liver area.


The reflected sound waves are picked up through the probe and used to build a screen image of the liver's condition. This painless test 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.

 

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Treatment (provided by NUH)

There are a number of treatments for the symptoms of PBC. Some of them help with any unpleasant side effects, such as dry eyes, and others slow the progress of the disease.

 

Controlling your condition

A medication called ursodeoxycholic acid has helped some people. Although early studies failed to show any direct impact on survival rates, new evidence suggests that URSO may help to keep people alive for a longer period of time and delay liver transplantation. However, a liver transplant is the only option for people who have advanced PBC.

 

Managing unpleasant symptoms

Itching skin: colestyramine (also known as Questran) may be prescribed by your doctor to help ease the itch. Taken orally, colestyramine works by preventing re-absorption of the chemicals that cause 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. Itching is made worse by dry skin. It is very important to use plenty of moisturiser.

 

Dry eyes and dry mouth: the combination of dry eyes and a dry mouth might be soothed by treatments such 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.

 

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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.

 

However, 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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When to seek medical advice

Primary Biliary Cirrhosis is usually diagnosed incidentally or following investigation for the above symptoms. Once diagnosed, the patient is usually seen in the liver clinic at regular intervals to monitor the progress of the condition.

 

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Doctors Listing

Appointment and Enquiry

University Digestive Centre
Kent Ridge Wing 2, Level 4
National University Hospital

 

Appointment Line:
(65) 6772 2505 or (65) 6773 3380
Fax: (65) 6774 1075
Email: udc@nuhs.edu.sg

 

Opening Hours:
8.30am - 5.30pm (Mon-Fri)
Closed on Sat, Sun & Public Holiday

 

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