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Granulomatosis with Polyangiitis (Wegener's)

2011; American Society of Nephrology; Volume: 22; Issue: 4 Linguagem: Inglês

10.1681/asn.2011010081

1533-3450

Ronald J. Falk, Wolfgang L. Gross, Loı̈c Guillevin, Gary S. Hoffman, David Jayne, J. Charles Jennette, Cees G. M. Kallenberg, Raashid Luqmani, Alfred Mahr, Eric L. Matteson, Peter A. Merkel, Ulrich Specks, Richard A. Watts,

Otitis Media and Relapsing Polychondritis

The Boards of Directors of the American College of Rheumatology, the American Society of Nephrology, and the European League Against Rheumatism recommend a gradual shift from honorific eponyms to disease-descriptive or etiology-based nomenclature. The leadership of these three organizations tasked an international group of senior academicians who are experts 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 The move toward a vasculitis terminology based on pathology, rather than historical reference, was triggered by evidence that Dr.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 met on November 7, 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 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 1954.5Granulomatosis with polyangiitis has previously been proposed as an alternative name for Wegener's granulomatosis.6 We recognize 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 that the new name recognizes 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 name for a disease is never easy. We believe that the wider medical and patient communities will accept and adopt the use of granulomatosis with polyangiitis (Wegener's) with the same spirit of international and multispecialty cooperation that led to our arriving at the new name. DISCLOSURES None.