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Primary Biliary Cirrhosis (PBC)

What is Primary Biliary Cirrhosis (PBC), previously known as Primary Biliary Cirrhosis?

Primary Biliary Cholangitis (PBC) is a chronic disease that can, little by little, destroy some of the tubes (called bile ducts) that link 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 can become 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.


Some people with PBC will never experience any symptoms of the disease. Common 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 typically include the following:

  • dry eyes and/or dry mouth
  • constant or variable ache or discomfort in the upper right hand side, below the ribs
  • indigestion, nausea or poor appetite
  • arthritis (inflammation of the joints)
  • pain in the bones
  • diarrhea
  • dark-colored 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.

What causes PBC?

This disease is called primary biliary cholangitis because the it attacks the bile (biliary) ducts inside the liver. This leads to scarring (fibrosis) and then to cirrhosis. Many experts consider it an autoimmune disease in which the body turns against its own cells. 

Who are at risk of PBC?

The following factors may increase your risk of primary biliary cholangitis:

  • Gender: most people with PBC are women.
  • Age: PBC is most likely to occur in people 30 to 60 years old.
  • Genetic factors: Those with a family member who has had PBC are more likely to develop it themselves
  • Geography: PBC is most common in northern Europe and North America.
Testing for PBC

Antibody test

Most people with PBC have anti-mitochondrial antibodies (AMA) in their blood. An antibody is a chemical made by the body to attack an 'invader'. The presence of AMAs in your blood is a possible 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 diagnosing 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 mainly a measure of damage to liver cells rather than to the bile duct cells.



Ultrasound uses high-frequency sound waves to produce images of structures inside your body.

Magnetic resonance cholangiopancreatography (MRCP) 

This special magnetic resonance imaging (MRI) exam creates detailed images of your organs and bile ducts.

Magnetic resonance elastography (MRE)

MRI is combined with sound waves to create a visual map (an elastogram) of internal organs. The test is used to detect any hardening of your liver that might be a sign of cirrhosis.

Liver Biopsy

If the diagnosis is still uncertain, your doctor may perform a liver biopsy. A small sample of liver tissue is removed through a small incision using a thin needle. It's examined in a laboratory, either to confirm the diagnosis or to determine the extent (stage) of the disease.
Treatment (provided by NUH)

There are a number of treatments for the symptoms of PBC. Some of these may help with any unpleasant side effects (such as dry eyes) while other treatments aim to slow the progress of the disease.

Controlling your condition

A medication called ursodeoxycholic acid has proven effective for some patients. Although early studies did not 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. For people who have advanced PBC, however, a liver transplant is their only option.

Managing unpleasant symptoms

Itching skin: cholestyramine (also known as Questran) may be prescribed to help ease the itch. Taken orally, cholestyramine works by preventing re-absorption of the chemicals that cause itching. It can take days or even weeks to become effective.

Some people taking cholestyramine have problems such as changed bowel habits and bloating. Doctors may prescribe 'Questran light' to reduce these side effects. If cholestyramine does not help, a hospital specialist may try other medicines. Itching is made worse by dry skin, thus it is very important to apply plenty of moisturiser.

Dry eyes and dry mouth may be soothed by treatments such as artificial tears and saliva, lubricating gels and oestrogen creams. Lozenges from the pharmacist can help with the dryness of the mouth.

Will I need a liver transplant?

A liver transplant is usually only recommended if other treatments are no longer effective and one's life is threatened by end-stage liver disease. It is a major operation that requires careful planning with one's medical team, family and friends.

Liver transplantation can provide positive outcomes for those with PBC. It is possible to develop PBC in the new liver, but it may take up to 15 years before the disease progresses significantly.

When to seek medical advice

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

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