The eleventh revision of the International Classification of Diseases (ICD-11) has accelerated a trend away from using people’s names and toward a more descriptive or etiologically based nomenclature for diseases. The most recent example is substituting the granulomatosis with polyangiitis (Wegener’s) for the vasculitis currently known as Wegener’s granulomatosis.
“Part of this process is being driven by ICD-11 and associated conversations about illnesses that have no widely accepted name,” says ACR President David Borenstein, MD, clinical professor of medicine in the division of rheumatology at The George Washington University Medical Center, a rheumatologist at Arthritis and Rheumatism Associates in Washington, D.C., and a member of The Rheumatologist’s editorial board. “While this is going on, there is also a push to do away with eponyms and define diseases more by the pathophysiological mechanism.”
Named Diseases Concern Other Specialties
Concerns about named illnesses are also being addressed in other specialty areas.
“Dermatology probably has even more eponym chaos than we do in rheumatology,” says Eric Matteson, MD, chair of the division of rheumatology at the Mayo Clinic in Rochester, Minn. “They have several different names for the same disease and through the auspices of the International League of Dermatology Societies they are working on a more rational nomenclature for ICD-11. The American Academy of Dermatology has been awarded a grant from the National Institutes of Health to develop a lexicon to address these issues.”
Why Not Use Eponyms?
In addition to lack of consistent naming conventions, those involved point to other concerns about identifying a disease process with a specific person. Sometimes a person has had a disease process named after them because they were the first to describe it in the literature. In other instances, the person for which it was named was a later contributor but was better known or more prolific.
H. Klinger first described granulomatosis with polyangiitis in 1931 as a variant of polyarteritis nodosa. Friederich Wegener followed up in 1936 and 1939 with two articles describing it in greater detail as a separate syndrome. Wegener’s name was attached to the disease in English language literature in 1954.
“Eponyms are said to be a way to recognize those who have gone before us and made major contributions that should not be forgotten,” says Dr. Matteson. “While we should remember these people, the need to recognize one’s heroes with naming is a superfluous exercise that doesn’t really help us in medicine.”
There are also historical concerns about the individual that can cause a push to change the name. In this case, some thought this was needed because of Dr. Wegener’s active participation in the medical establishment of the Nazi party during World War II.
Richard Panush, MD, professor of medicine in the Division of Rheumatology at the Keck School of Medicine, University of Southern California in Los Angeles, has an interest in the history of medicine and has contributed to the discussion of issues relevant to the crimes of Nazi physicians. “It is important that professional societies address the issue of moral, ethical, and professional characteristics of the person in cases such as this,” he says.
There is no evidence that Wegener was directly involved in war crimes during his tenure as director of the health authority in Lodz, Poland. He was involved in the Nazi medical establishment’s goal of preserving “racial hygiene.”
“While his connection to the Nazi regime was an early impetus for change, a critical evaluation of how we name diseases is the more important piece here,” says Dr. Matteson. “What we really want to do in medicine is to give diseases more scientifically appropriate names. Granulomatosis with polyangiitis references the two major features of the illness and is substantially more descriptive than the old name.”
ACR and Others Pushing for Change
The ACR cooperated with the European League Against Rheumatism (EULAR) and the American Society of Nephrology when the name change was suggested. A group of experts in the disease representing the associations met and reached a consensus on changing the name to granulomatosis with polyangiitis. They added Wegener’s as a parenthetical during a transition period.
“The changeover may take as long as five years to complete,” says Dr. Matteson. “In the interim we want make sure the disease is easily identifiable and searchable, so Dr. Wegener’s name will remain attached for a while.”
To get the word out about the change, the presidents of all three professional societies published articles in their journals.1 Letters outlining the suggested name change have been sent to the editors of most major journal editors and the National Library of Medicine.
Both Drs. Borenstein and Matteson agree that it will take some time to have the changes take full effect. Some of it will be people wanting to continue with what they learned in medical school, some just what a physician feels comfortable using.
Other changes in naming of diseases are under consideration. Churg-Strauss syndrome and Behçet’s syndrome are two others being reviewed.
“Time moves on and we are going to have to learn different names for things,” says Dr. Borenstein. “Whether it is because a person had a bad past, because it doesn’t make sense from our current understanding of the disease, or other valid reasons, we will be looking at these issues in a thoughtful way.”
Kurt Ullman is a freelance writer based in Indiana.
