The name Primary Biliary Cirrhosis (PBC) is somewhat deceiving since cirrhosis only occurs in the final stage of this liver disease, after decades of inflammation

Primary Biliary Cirrhosis Has A New Name (Cholangitis)The name Primary Biliary Cirrhosis (PBC) is somewhat deceiving since cirrhosis only occurs in the final stage of this liver disease, after decades of inflammation. Since 1950, due to the late diagnosis of the disease, most PBCpatients had already developed cirrhosis or were in liver failure.During the past twenty years, many improvements for the care of the PBC patient by way of early testing for postive antimitochondrial antibody and elevated liver (enzyme) tests, has led to the early diagnosis and medications that slow the disease progression in many patients. Using current treatments, about 80 - 85% of patients diagnosed with PBC will most likely will never reach the cirrhosis stage or need a liver transplant. The name primary billiary cirrhosis fit the disease for many years, but it does not fit the disease now due to early diagnosis inmost patients.For over 20 years, both patients and the medical community have discussed the PBC name change.In April 2014, adiscussion among a few doctors and advocatesturned into the "PBC Name Change Initiative".Hepatologists, PBC researchers, advocates, patients, caregivers and support groups worldwide endorsed the name change initiative. For the next year, there was a constant stream of meetings, e-mails, phone calls and surveys among PBC experts, patients, advocates and stakeholders. May 2015, the American Association for the Study of Liver Disease (AASLD) and in the fall of 2014 the European Association for the Study of the Liver (EASL) approved the new name primary biliary cholangitisto replace primary biliary cirrhosis.

What's in a Name?

Primary is not secondary to any other cause, specifically the original condition or set of symptoms in disease processes. Biliary pertains to the bile, to the bile ducts, or to the gallbladder.

Cholangitis (k'lan-j'tis) is an immune inflammatory response (-itis means inflammation only, does not denote infection) focused on the cells of the biliary system.

Primary biliary cholangitis (PBC) gradually causes liver destruction of the biliary system with inflammation from the immune response. PBC is not directly linked to cirrhosis since stages 0 - 1 portal, 2 periportal and 3 septal do not indicate cirrhosis. In some patients, after many years (decades) of destruction, the damage will lead to stage 4 cirrhosis. Some patients can live many years in the cirrhosis stage without a liver transplant, death or liver failure.

There were many reasons to change the name primary biliary cirrhosis to primary biliary cholangitis, depending on who you spoke to, patient or medical professional. Below are some of the most important.

1. Simplicity: Changing cirrhosis to cholangitis makes it simple for both patients and doctors to understand that the disease is inflammatory focused on the biliary system. Primary biliary cholangitis follows the correct clinical, biochemical and the histological staging of this disease.2. Clarity: Cholangitisgives the disease name clarity. The type of tests andproceduresvary depending on the PBC disease stage. Certain tests need to be performed beforethe cirrhosis stage, yet other more extensive testsare performed in stage 4 when cirrhosis is present. The new global ICD codes will help make sure patients have the proper tests and proceduresperformed for their exact disease stage. In addition, making sure the patient's insurance company reimburses providers for tests and procedures performed, eliminating the increased or high out-of-pocket costs to the patients. Currently, all PBC patients' medical records include primary biliary cirrhosis as their liver disease diagnosis. Using the new ICD codes onlythose patients (about 15 - 20%) who are actually in the cirrhosis stage 4, will have the new cirrhosis code listed in their medical records. PBC patients (about 80 - 85%) in stages 0, 1, 2 & 3 will no longer be falsely labeled with a cirrhosis stage they do not have and may never have.3. Research Donations: Receiving large research donations are a major problem associated with PBC and the word cirrhosis. Most large donors prefer tofund liver diseases that have not reached the cirrhosis stage, preferring the focus of research concerns preventing cirrhosis and its complications. For donors the primary biliary cirrhosis name indicates cirrhosis, the finaldisease stage. Even after educating the donors, the cirrhosis stigma is still attached and the donation will usually go to another liver disease. Over the years, the stigma has kept PBC research from receiving many large donations. We hope by removing the word cirrhosis it will help bring large donations to PBC research and in finding a cure. Patients and providers all depend on hope to promote research and advance the care of patients.4. Goals: The name change to primary biliarycholangitiswillhelp meetour goalsof the correct diseasedescription, clarity of stages, more awareness and possible large research donations. It will help patients who have felt isolated in their disease due to an incorrect diagnosis, the despair often associated with the term cirrhosis, andthat implied connection, most commonly associatedwith alcohol and or drug use. This will help remove some of the discrimination towards patients, making it easier purchasinglife, health and travel insurance (various countries) and mortgage real estate insurance when needed (UK).

Now we begin the education phase of the cirrhosis name change.Hepatologists, PBC researchers, advocates, patients and support groups are getting ready for an extensive worldwide education campaign. Every outlet availablesuch as hepatology magazines, medical newsletters, blogs, websites, media news releases, e-mails, meetings and conferences will be used to promote the new name primary biliary cholangitis.Please understand the use ofthe new name will not happen over night, but with patients and themedical community working together,the processwill move along much faster and smoother.

Changing the name from cirrhosis to cholangitis was not an easy task. It took a lot of time and energy of many medical professionals, advocates and patients working together for a common goal. We owe our sincerest thanks to all those involved in making the PBC name change a success and reality. Special thanks to Dr. Robert Gish for his total commitment and dedication to the initiative, from start to finish. We reach out to you to support, advocate and embrace this name change and to now expend your energies on education, support of patients with PBC, and provide assistance to those leading research efforts.

Feel free to share the name change information with your family, friends and colleagues who might be interested in Primary Biliary Cholangitis. Thanks for your help and support.Sincerely,

Linie Moore

PBCers Organization, President

http://pbcers.org

https://www.facebook.com/PBCersOrg

Some of the worldwide PBC experts involved in the name change initiative

Ian R. Mackay, MD - Monash University, Australia

Xiong Ma, MD - Renji Hospital, School of Medicine, ChinaRaoul Poupon, MD - Service dHpatologie, Saint-Antoine Hospital, France

Andreas Feige, MD - Asklepios Klinik Nord Ochsenzoll, Hamburg, Germany

Ansgar W. Lohse, MD - University Medical Center Hamburg-Eppendorf, Germany

Bernd Lowe, MD - University Medical Center Hamburg-Eppendorf and Schon Clinic, Germany

Christina Weiler-Normann, MD - University Medical Center Hamburg-Eppendorf, Germany

Matthias Rose, MD - Charite-Universitatsmedizin GermanyChristoph Schramm, MD - University Medical Center Hamburg-Eppendorf, Germany

Inka Wahl, PH.D. - University Medical Center Hamburg-Eppendorf and Schon Clinic, Germany

Shiv Kumar Sarin, MD - Institute of Liver and Biliary Sciences, Jawahar Lol Nehru University, India

Pietro Invernizzi, MD - Humanitas Clinical and Research Center, Italy

Masao Omata, MD - University Medical Information Network (UMIN), Japan

Atsushi Tanaka, MD - Teikyo University School of Medicine, Japan

Osama Yokosuka, MD - Chiba University, Japan

Ulrich Beuers, MD - University of Amsterdam, Netherlands

Albert Pars, MD - Hospital Clinic IDIBAPS, CIBERehd, University of Barcelona, SpainDavid E.J. Jones, MD - Newcastle University, UK

James Neuberger, MD - Queen Elizabeth Hospital, UKBruce Bacon, MD - Saint Louis University School of Medicine, MO, USAAdrian Di Bisceglie, MD - National Institutes of Health (NIH), Saint Louis University Liver Center, MO, USAChris Bowlus, MD - University of California School of Medicine at Davis, CA, USAM. Eric Gershwin, MD - University of California School of Medicine at Davis, CA, USARobert G. Gish, Stanford University, CA, St Josephs Hospital and Medical Center, AZ USA Kris Kowdley, MD - Swedish Medical Center, WA, USAEdward Krawitt, MD - University of Vermont Medical Center, VT, USAKeith Lindor, MD - College of Health Solutions, Arizona State University, Arizona, USAJohn M. Vierling, MD - Baylor College of Medicine, Texas, USA

Support Groups

Ingo van Thiel, European Liver Patients Association (ELPA)

Robert Mitchell-Thain, PBC Foundation

Linie Moore - PBCers Organization