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 PBC patients 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 in most patients.
For over 20 years, both patients and the medical community have discussed the PBC name change. In April 2014,
a discussion among a few doctors and advocates turned 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 cholangitis to
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: Cholangitis gives the disease name clarity. The type of tests and procedures vary depending on the PBC
disease stage. Certain tests need to be performed before the cirrhosis stage, yet other more extensive tests are
performed in stage 4 when cirrhosis is present. The new global ICD codes will help make sure patients have the
proper tests and procedures performed 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 only those 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 to fund 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 final disease 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 biliary cholangitis will help meet our goals of the correct disease description,
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,
and that implied connection, most commonly associated with alcohol and or drug use. This will help remove some
of the discrimination towards patients, making it easier purchasing life, 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 available such 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 of the new name will not
happen over night, but with patients and the medical community working together, the process will 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, China
Raoul 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 Germany
Christoph 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, Spain
David E.J. Jones, MD - Newcastle University, UK
James Neuberger, MD - Queen Elizabeth Hospital, UK
Bruce Bacon, MD - Saint Louis University School of Medicine, MO, USA
Adrian Di Bisceglie, MD - National Institutes of Health (NIH), Saint Louis University Liver Center, MO, USA
Chris Bowlus, MD - University of California School of Medicine at Davis, CA, USA
M. Eric Gershwin, MD - University of California School of Medicine at Davis, CA, USA
Robert G. Gish, Stanford University, CA, St Josephs Hospital and Medical Center, AZ USA
Kris Kowdley, MD - Swedish Medical Center, WA, USA
Edward Krawitt, MD - University of Vermont Medical Center, VT, USA
Keith Lindor, MD - College of Health Solutions, Arizona State University, Arizona, USA
John 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