Primary Biliary Cirrhosis

Primary biliary cirrhosis is a chronic (long-term) condition which affects the liver. Primary biliary cirrhosis causes gradual damage of the bile ducts and this eventually means that bile cannot flow normally from the liver, which causes bile to collect in the liver and the liver to become damage.

A rare condition it is estimated that around 24 per 100,000 people in the UK are diagnosed with primary biliary cirrhosis. The condition is much more common in women than men (90% of cases involve women) and tends to affect people aged between 30 and 65. In general most cases are diagnosed in women over the age of 40.

What causes primary biliary cirrhosis?

The precise source of primary biliary cirrhosis is unidentified, though experts suggest that genetic and environmental factors are possible causes of the disease. Many experts also believe that primary biliary cirrhosis is an autoimmune condition, which means that the body’s immune system has an abnormal response to a trigger. In the case of individuals with primary biliary cirrhosis, it is the case that the immune system releases white blood cells known as T-cells to the bile ducts. T cells are part of the body’s defence system and are usually used to attack illnesses, infections and other threats. However, in cases of PBC the T-cells damage the bile ducts and cause scarring, which eventually interrupts the flow of bile from the liver and causes damage to the organ.

It is not known why some people suffer from autoimmune conditions and research in this area is ongoing.

There is also evidence to suggest that certain risk factors increase the chance of an individual developing primary biliary cirrhosis. These risk factors include:

• Family history: people who have a close relative with PBC have a slightly higher risk of developing the condition.
• Sex: females are much more prone to incur PBC than males.

Triggers for the disease have also been identified. These triggers may cause PBC to develop in people who are genetically predisposed to the disease, although evidence is limited. Possible triggers include:

• Hormone replacement therapy (HRT).
• Smoking.
• Living near a waste site.
• Infections.

Symptoms of primary biliary cirrhosis

In many cases individuals with PBC do not develop symptoms until the liver has suffered considerable damage, which is known as asymptomatic primary biliary cirrhosis. In around 50 percent of cases symptoms do appear and they tend to vary according to the stage of the disease, as follows:

Early stage:

• Fatigue: this is the most common symptom and many people find that they struggle to cope with a full day of work and have an urge to sleep during the day.
• Itchiness: this can be mild, moderate or severe and may affect many areas of the body.
• Dry mouth and eyes.

Advanced stage:

• Jaundice: this is when the whites of the eyes and skin appear yellow, which is caused by the liver being unable to get rid of bilirubin.
• Diarrhoea.
• Unpleasant smelling stools.
• Fluid retention inside the abdomen, which can make the stomach appear bloated and distended.
• Oedema: this occurs when fluid collects in the legs and feet.

It is possible for primary biliary cirrhosis to cause complications, which may include:

• Osteoporosis (when the bones become weakened).
• Liver cancer.

How is primary biliary cirrhosis diagnosed?

In many cases primary biliary cirrhosis is diagnosed when an individual is having blood tests for another condition, which often happens because the symptoms can be general in the early stage. A blood test is usually sufficient to reach a diagnosis but additional tests may also be carried out, including a liver biopsy which involves having a tiny piece of tissue removed for analysis under a microscope. The blood sample will be analysed for the presence of anti-mitochondrial antibodies, which are detectable in around 95% of people who have primary biliary cirrhosis. The blood tests may also show a high level of bilirubin, a yellow substance that is usually passed through the liver. If the bile ducts are damaged the levels of bilirubin in the body will be higher than usual.

Treatment for primary biliary cirrhosis

There is no cure for primary biliary cirrhosis and the main aims of treatment are to prevent damage to the liver and ease symptoms. The main treatment for preventing damage to the liver is ursodeoxycholic acid (UDCA), which helps to reduce inflammation of the liver, the level of bilirubin in the blood, the levels of liver enzymes and alter the composition of bile.

Vitamins, calcium supplements and hormone therapy may be recommended to help ease symptoms. Some people with PBC also suffer from autoimmune hepatitis, a condition which causes the liver to become inflamed. If this is the case steroids will be prescribed to reduce inflammation.

In extreme cases, where the liver is brutally damaged and your life is in danger, a liver transplant could be the only option. A liver transplant involves replacing the damaged liver with a healthy liver from another person. Transplants are complex operations that carry risks, but they can save and prolong lives. The major problem with transplants is that there is a shortage of donor organs and waiting lists can be very long.

Self-help

People with PBC will be advised about their lifestyle and steps they can take to reduce symptoms and prevent further damage to their liver. These lifestyle changes include:

• Avoiding alcohol or cutting down on alcohol consumption. You should try not to exceed recommended daily guidelines, which are 2 units per day for women and 3 units per day for men.
• Eating a healthy diet and maintaining a stable, healthy weight.
• Giving up smoking (if this is applicable).