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:
Tiredness and itch are generally the first symptoms to appear, while jaundice is usually associated with the later stages of the disease.
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.
The following factors may increase your risk of primary biliary cholangitis:
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.
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.
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.
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.
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.
