Granulomatosis with Polyangitis (Wegener’s)”: The New Name for “Wegener’s Granulomatosis”
The term “Wegener’s granulomatosis” was introduced to the English language medical literature in 1954. The name was proposed to honor Dr. Friedrich Wegener. Dr. Wegener was an early describer of the disease, but not the first describer. Since the first descriptions, many scientists have contributed to the understanding of the nature of the disease and to its treatment. As more has been learned about the disease, it has become clear that it is a highly complex entity with diverse symptoms, signs, and outcomes, and the name “Wegener’s granulomatosis” does not adequately represent an understanding of the disease.
To reflect improved understanding of the many types of vasculitis, and make the terminology used to describe them more uniform around the world, leading professional organizations have endorsed the goal of adopting new names for some diseases. The goal is to find new names which are more scientifically accurate and don’t use names of physicians or scientists.
Diseases named after people are called “eponyms” and the use of such eponyms is not uniform, and is often confusing. Sometimes an eponym is used in only some countries. For example, “Horton’s Disease” is used in some European countries while “giant cell arteritis” is used for the same disease in many other places.
For some diseases, including Wegener’s granulomatosis and Takayasu’s arteritis, the eponym is named for people who were not the first or necessarily the most important describers of the diseases. Furthermore, these names don’t reflect a basic scientific understanding of the diseases.
In the case of Wegener’s granulomatosis, additional concerns about Dr Wegener’s involvement with the Nazi regime provided further reason to agree on a new name that was also more scientifically descriptive.
The new terminology for “Wegener’s granulomatosis” which has been adopted by the American College of Rheumatology, the American Society of Nephrology, and the European League Against Rheumatism is “granulomatosis with polyangiitis (Wegener’s)”, which can be abbreviated as “GPA”. The reasons for the change in terminology are outlined in a short manuscript recently published simultaneously in the journals of these three organizations (Arthritis and Rheumatism, Annals of the Rheumatic Diseases, and The Journal of the American Society of Nephrology) (1). Several years of thought and discussion by leading members within the academic vasculitis research community from multiple medical specialties went into the decision to adopt this new name.
The goal was that the new term be recognizable and relate to current convention. Hence, it is recommended that the parenthetical term “(Wegener’s)” be included after “granulomatosis with polyangiitis” for several years to help smooth the adoption of the new name, avoid confusion among patients and physicians and others interested in the disease, and facilitate electronic searches. It is anticipated that most medical journals will adopt the new term quickly.
It is never easy to change a name of something with which we are familiar. The change is needed, and the new term can be better linked to related forms of vasculitis such as microscopic polyangiitis (MPA). Other changes in vasculitis terminology will certainly occur as understanding of these diseases improves.
The Vasculitis Foundation will transition to using the term “granulomatosis with polyangitis (Wegener’s)” in Foundation communications and other resources, and is committed to helping its members, and others who seek information from the Foundation, adapt to the name change.
1. Falk RJ, Gross WL, Guillevin L, Hoffman GS, Jayne DRW, Jennette JC, Kallenberg CGM, Luqmani R, Mahr AD, Matteson EL, Merkel PA, Specks U, Watts RA, for the American College of Rheumatology, the American Society of Nephrology, and the European League Against Rheumatism. Granulomatosis with polyangiitis (Wegener’s): An alternative name for Wegener’s granulomatosis. Arthritis Rheum 2011;63(4):863-864.
2. Woywodt A, Haubitz M, Haller H, Matteson EL. Wegener’s granulomatosis. Lancet 2006;367:1362–6.