PBC for Healthcare Professionals

PBC (Primary Biliary Cholangitis) is a chronic cholestatic liver disease. It is in the group of autoimmune diseases which include thyroid disease, systemic sclerosis and its variants, rheumatoid arthritis and Sjögren’s Syndrome. Because the associations between the diseases, PBC patients may also have one or more additional autoimmune conditions. In PBC there is a characteristic immune response with antibodies against mitochondrial proteins (the “anti-mitochondrial antibodies” or AMA) being seen in over 95% of patients. As well as giving us important insights into the mechanism of PBC these antibodies are an important diagnostic test in the clinic. 

In PBC, the immune “attack” is on the small intrahepatic bile ducts. This ultimately leads to destruction of the bile ducts – a ‘disappearing bile duct’ syndrome – cholestasis and, in the latter stages of the disease, jaundice and cirrhosis.

Like many other autoimmune conditions, PBC affects mostly women (in Britain, about 90% of diagnosed patients). Generally, it presents between ages thirty and fifty-five although it may be diagnosed at any age in adults. It is, essentially, never seen in children. According to a recent study, as many as 1:1000 women over the age of forty may have PBC. There is no connection between PBC and either alcohol or any of the established hepatitis viruses.

PBC Foundation Leaflets

The PBC Foundation is an organisation that has facilitated research and continues to do so, when it can. There have been projects we have funded, projects we have supported with resources and projects about which we have informed our membership. 

Here are the three leaflets available from the PBC Foundation. The first one is for health professionals, and we hope it is of considerable help to you. The second is for patients affected by PBC. Please feel free to print this leaflet and give it to your patients diagnosed with PBC. Once a patient contacts us, we can provide a wide range of information and support which will ease the burden on the GP practice. The third leaflet is general information about PBC and the work of the Foundation.

PBC Research

The PBC Foundation is an organisation that has facilitated research and continues to do so, when it can. There have been projects we have funded, projects we have supported with resources and projects about which we have informed our membership.

There are many current research projects in the UK, looking at various aspects of PBC, which may be of interest to your patients. UK-PBC is a national research platform that brings PBC research in the UK together. Over 9000 UIK PBC patients have joined the UK-PBC programme to date.

The most successful study we have been involved into date is the original PBC Genetics Study based in Addenbrookes Hospital in Cambridge. This study used the DNA samples from thousands of patients across the UK to help identify the genetic reasons why someone develops PBC. Patient involvement is absolutely key for the success of the research programme and there are many ways in which people can participate. These include opportunities to continue the genetics collection, studies on why Urso doesn’t work for everyone, and most importantly in many ways, opportunities to take part in new trials of drugs for PBC (including symptoms). Everyone who took part in the original genetics study will be notified about this, and new patients are encouraged to join.  If your patients with PBC are interested in this study, they can contact UK-PBC direct on 01223 769088. We would be grateful to you for informing your patients about this study.

Another important study was the development of the PBC-40, conducted in conjunction with the Universities of Liverpool and Newcastle. This study measures quality of life (and the management of) in relation to PBC and its symptoms.

Whilst Ursodeoxycholic Acid is the standard care for those affected by PBC, we know that there is a certain percentage that does not respond to treatment. We are looking at this section of the community in detail: if there are genetic influences, biomechanics, new possible medications, etc., so please check here regularly to keep up to date with developments.