Primary biliary cholangitis (PBC)

What is primary biliary cholangitis?

Primary biliary cholangitis (PBC) is a chronic autoimmune liver disease in which the body’s own immune system causes slow but progressive damage to the small bile ducts (the channels through which bile flows) inside the liver. Over the course of many years, PBC can eventually lead to cirrhosis (scarring of the liver), although this does not happen in everyone with the disease.

What causes PBC?

PBC is an autoimmune disease, in which the body’s own immune system mistakenly attacks the cells that line the small bile ducts inside the liver. No one knows why this happens. Some people with PBC have relatives with the condition. This suggests that people with PBC may have an inherited predisposition to the disease, which is finally triggered by something in the environment. We do not know what the environmental trigger (or triggers) might be. PBC is more common in women, in people with other autoimmune conditions, and amongst those who smoke tobacco.

What are the symptoms of PBC?

Common symptoms associated with PBC include fatigue, itching (‘pruritus’), dryness of the eyes or mouth (sicca complex), and upper abdominal pain. Because of related conditions, some people experience joint pains, diarrhoea and over- or under-active thyroid. Many people with PBC have no symptoms at all.

Is there a treatment for PBC?

There is no cure for PBC but there are very effective treatments. All patients with PBC begin treatment with a medication called ursodeoxycholic acid (UDCA), given at a dose of 13mg to 15mg/kg/day. It may be taken in divided doses or all at once. It is very well tolerated. Most people taking UDCA have no side effects at all. The most common side effects reported by patients are weight gain and loose bowel motions.

In most people with PBC, UDCA is highly effective at controlling the disease. In some people, however, UDCA is less effective. These patients may receive treatment with obeticholic acid (OCA) or bezafibrate in addition to UDCA:

• OCA has recently been licensed for the treatment of PBC and is only available via specialist centres (such as Addenbrooke’s Hospital). It is generally well-tolerated but might cause itching which can be controlled using other medications.
• Bezafibrate is a medication licensed for treatment of high cholesterol, which is used ‘off licence’ for treatment of PBC. (‘Off licence’ means that the medication is used for a different condition than it is licensed for.) It is generally well-tolerated and has few side effects but does require close monitoring with blood tests when it is first started.

What should I expect from Addenbrooke’s Hospital?

You will be under the care of a hepatology consultant. Following your first consultation with the doctor you will have a baseline ultrasound scan (USS), fibroscan, and DEXA scan and you will start treatment with UDCA. Your subsequent management will depend on the stage of the PBC and how you respond to treatment with UDCA. Patients without cirrhosis who respond well to treatment with UDCA (with or without second-line treatment) will be seen every one or two years. Patients with cirrhosis will have an USS of the liver every six months and a clinic appointment every six to 12 months. Regular USSs are important for people with cirrhosis because they have an increased risk of liver cancer; regular scans can help to detect liver cancer at an early and treatable stage. Other investigations may be required as the disease progresses.

We will do our best to keep the monitoring of your PBC up to date. We strongly encourage you to become familiar with the tests you need, however, to ensure these tests happen when they should.

Contacts

If you require any further information about this leaflet or advice about your condition please contact the Addenbrooke’s autoimmune liver disease advice line on 01223 216109

Where can I receive more information and support?

• PBC Foundation (opens in a new tab) 0131 5566811
• British Liver Trust (opens in a new tab) Helpline: 0800 652 7330 – 10:00 (10am) to 15:00 (3pm) Monday to Friday
• UK-PBC (opens in a new tab) - Stratified Medicine in Primary Billiary Cholangitis
• The Psychological Wellbeing Service offers treatments across Cambridgeshire and Peterborough CPFT NHS Trust (opens in a new tab) 0300 300 0055

What research is happening in PBC?

There are opportunities to be involved in clinical trials for new treatments of PBC. Please call 01223 256225 or email Sister Abi Ford if you would like to discuss this.