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.
It is called PBC because the disease 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.
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:
Tiredness and itch are generally the first symptoms to appear, while jaundice is usually associated with the later stages of the disease.
PBC 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.
Most people with PBC have Anti-mitochondrial 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.
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 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 uses high-frequency sound waves to produce images of structures inside your body.
This special magnetic resonance imaging (MRI) exam creates detailed images of your organs and bile ducts.
MRI is combined with sound waves to create a visual map (elastogram) of internal organs. The test is used to detect hardening of your liver that might be a sign of cirrhosis.
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.
The following factors may increase your risk of PBC:
Most people with PBC are women
It's most likely to occur in people 30 to 60 years old.
You're more likely to get the condition if you have a family member who has or had it.
It's most common in northern Europe and North America.
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.
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.
Cholestyramine (also known as Questran) may be prescribed by your doctor 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 before this becomes effective.
Some people taking cholestyramine have problems such as changed bowel habits and bloating.
Your doctor 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. It is very important to use plenty of moisturiser.
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.
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.
Click here to access our Find A Doctor directory for a list of doctors treating this condition across our NUHS institutions.
