Article Text

Download PDFPDF

Granulomatosis with polyangiitis (Wegener's): An alternative name for Wegener's granulomatosis
  1. Ronald J Falk1,
  2. Wolfgang L Gross2,
  3. Loïc Guillevin3,
  4. Gary Hoffman4,
  5. David R W Jayne5,
  6. J Charles Jennette6,
  7. Cees G M Kallenberg7,
  8. Raashid Luqmani8,
  9. Alfred D Mahr9,
  10. Eric L Matteson10,
  11. Peter A Merkel11,
  12. Ulrich Specks12,
  13. Richard Watts13
  1. 1Department of Internal Medicine, University of North Carolina, Chapel Hill, NC, USA
  2. 2Department of Rheumatology, University at Lübeck, Lübeck, Germany
  3. 3Department of Internal Medicine, Université Paris Descartes, and Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Paris, France
  4. 4Department of Rheumatologic and Immunologic Diseases, Cleveland Clinic Foundation, Cleveland, OH, USA
  5. 5Department of Nephrology, Addenbrooke's Hospital, Cambridge, UK
  6. 6Department of Pathology and Laboratory Medicine, University of North Carolina, Chapel Hill, NC, USA
  7. 7Department of Rheumatology & Clinical Immunology, University Medical Center Groningen, University of Groningen, The Netherlands
  8. 8Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Sciences, University of Oxford, Oxford, UK
  9. 9Department of Internal Medicine Université Paris Diderot - Paris 7, and Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris, Paris, France
  10. 10Departments of Medicine and Health Sciences Research, Mayo Clinic College of Medicine, Rochester, MN, USA
  11. 11Department of Medicine, Boston University School of Medicine, Boston, MA, USA
  12. 12Department of Medicine, Mayo Clinic, Rochester, MN, USA
  13. 13Department of Rheumatology, Ipswich Hospital NHS Trust, Ipswich, UK; and Norwich Medical School, Norwich, UK
  1. Correspondence to Peter A Merkel, Boston University School of Medicine, 72 East Concord Street, Boston, MA 02118, USA; pmerkel{at}bu.edu

Statistics from Altmetric.com

The Boards of Directors of the American College of Rheumatology (ACR), American Society of Nephrology (ASN) and the European League Against Rheumatism (EULAR) have recommended a gradual shift from honorific eponyms to disease-descriptive or aetiology-based nomenclature.

The leadership of these three organizations tasked an international group of senior academicians expert in the care of patients with vasculitis and engaged in research in the field to provide the medical community with proper descriptive terms instead of the names for Wegener's granulomatosis, Churg–Strauss syndrome, and Behçet's syndrome. The move towards a vasculitis terminology based on pathology, rather than historical reference, was triggered by evidence that Dr Friedrich Wegener was a member of the Nazi party before and during World War II.1

As the first step towards a vasculitis nomenclature that is free of eponyms, the authors of this article held a meeting on 7 November 2010 and reached consensus on an alternative name for Wegener's granulomatosis. As physicians whose clinical and research work focuses on vasculitis, we represent the diverse opinions of our international colleagues within the multiple medical specialties that have strong interests in vasculitis. This article announces the newly proposed name, outlines the reasons for seeking a new disease name, and explains the rationale for the proposed name.

The alternative name for Wegener's granulomatosis is granulomatosis with polyangiitis (Wegener's) which can be abbreviated as GPA. The parenthetical reference to Wegener's will be phased out after several years as the new usage becomes more widely known.

Granulomatosis with polyangiitis was initially described by Klinger in 1931 as a variant of polyarteritis nodosa, and then in greater detail as a separate syndrome by Wegener in two articles appearing in 1936 and 1939.2,,4 The term Wegener's granulomatosis was introduced into the English-language literature by Drs Godman and Churg in 1954.5 Granulomatosis with polyangiitis has previously been proposed as an alternative for Wegener's granulomaotsis.6

We recognise the difficulty inherent in seeking a replacement term for a long-established disease name for this complex multisystem illness with highly variable clinical presentations. Although this replacement term is neither perfect nor encompasses all aspects of the pathophysiology and clinical spectrum of the disease, the new term is nonetheless fit for the intended purpose for several important reasons: inclusion of the word granulomatosis means the new name recognises the history of the disease name as well as a main feature of the pathology, and the word polyangiitis both reflects the frequent vasculitic involvement of multiple types of vessels and retains the nomenclature used by the Chapel Hill Consensus Conference for vasculitic involvement in a related condition called microscopic polyangiitis.7 The new term will not preclude its incorporation into a more detailed revised nomenclature and classification scheme for the vasculitides that may be developed in the future. Finally, we propose inclusion of the parenthetical term (Wegener's) for several years to help smooth the adoption of the new name, avoid confusion in the medical literature and facilitate electronic searches.

Changing a disease name is never easy. We believe that the wider medical and patient communities will accept and adopt the name granulomatosis with polyangiitis (Wegener's) with the same spirit of international and multispecialty cooperation that led to our arriving at the new name.

References

View Abstract

Footnotes

  • To be simultaneously published in Arthritis & Rheumatism, Journal of the American Society of Nephrology and Annals of the Rheumatic Diseases

  • Provenance and peer review Not commissioned; not externally peer reviewed.

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.