What is Primary Biliary Cirrhosis?

PBC is a chronic disease that slowly destroys some of bile ducts (tubes draining the bile from the liver into intestine).

When bile ducts are damaged, bile can’t flow and builds up in the liver, damaging the liver cells, causing inflammation and scarring, called liver cirrhosis.

What are the symptoms of PBC?

Some people with PBC will never get any symptoms of the disease.

Others develop tiredness (for some people this can be severe) and intense itching in any part of the body.

Other symptoms that may develop usually include the following:

  • dry eyes and/or dry mouth
  • ache or discomfort in the right upper side of the abdomen, below your ribs
  • indigestion, nausea or poor appetite
  • arthritis (inflammation of the joints)
  • pain in the bones
  • diarrhoea
  • dark urine and/or pale stools
  • jaundice – yellowing of the skin and whites of the eyes.

Who is at risk of PBC?

Nine out of ten people who get PBC are women.
No one knows why this is. In particular the following women are most at risk:

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

What are the causes of PBC?

PBC is believed to be an autoimmune disease.
This is a disease where the body attacks itself.
Possible causes of this ‘trigger’ could be:

  • an infection
  • some form of environmental trigger

How is PBC diagnosed?

Blood test for specific antibody

Most people with PBC have something in their blood called antimitochondrial antibody (AMA).

Liver function tests (LFTs)

In PBC, doctors will be looking for increased levels of alkaline phosphatase (ALP), and the immunoglobulin IgM. The liver enzymes alanine transaminase (ALT) and aspartate transaminase (AST) are also monitored.

Radiologic scans

Ultrasound scan is painless test to check the condition of liver in general.

More specific test is MRI and MRCP scan which allows to visualize bile ducts in more details.

Treatment

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 (Ursofalk) has helped some people. However, a liver transplant is the only option for people who have advanced PBC.

Managing unpleasant symptoms

Itching skin: colestyramine (sold as Questran) may be prescribed by your doctor to help ease itching. Some people taking this medication have problems such as changed bowel habits and bloating. Itching is also made worse by dry skin. It is very important to use plenty of moisturizer.

Dry eyes and dry mouth can be treated with artificial tears and saliva, lubricating gels. Lozenges will help with the dryness in mouth.

Will I need a liver transplant?

A liver transplant is usually only recommended if other treatments are no longer helpful and the liver showed decompensated cirrhosis. However, liver transplantation works well for people with PBC. It is possible to get PBC in your new liver, but it may take up about 15 years before the disease becomes significant.

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