Granulomatosis with polyangiitis historical perspective

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Ali Poyan Mehr, M.D. [2]Associate Editor(s)-in-Chief: Krzysztof Wierzbicki M.D. [3]Cafer Zorkun, M.D., Ph.D. [4]

Overview

Granulomatosis with polyangiitis was first discovered by Peter McBride, a Scottish otolaryngologist, in 1897 when describing a case of rapid destruction of the nose and face[1]. In 1907, Heinz Karl Ernst Klinger added information regarding the anatomical pathology of the disease. However, the full presentation of the disease was later discovered in 2 separate reports in 1936 and 1939 by a German pathologist, Friedrich Wegener.

An earlier name for the disease was pathergic granulomatososis.[2] The disease is still sometimes confused with lethal midline granuloma and lymphomatoid granulomatosis, both malignant lymphomas.[3]

As of November 7, 2010, Wegener's granulomatosis has been changed to Granulomatosis with polyangiitis by the American College of Rheumatology, American Society of Nephrology, and the European League Against Rheumatism. The name has been change to characterize the pathology of the disease rather than having it remain as a historical reference, as it has become evident that Dr. Wegener was associated with the Nazi party.[4][5]

Historical Perspective

Heinz Karl Ernst Klinger (1907) would add information on the anatomical pathology, but the full picture was presented by Friedrich Wegener (1907–1990), a German pathologist, in two reports in 1936 and 1939.

In 2006, Dr. Alexander Woywodt (Preston, United Kingdom) and Dr. Eric Matteson (Mayo Clinic, USA) investigated Dr. Wegener's past, and discovered that he was, at least at some point of his career, a follower of the Nazi regime. In addition, their data indicated that Dr. Wegener was wanted by Polish authorities and that his files were forwarded to the United Nations War Crimes Commission. Finally, Dr. Wegener worked in close proximity to the genocide machinery in Lodz. Their data raised serious concerns about Dr. Wegener's professional conduct. They suggested that the eponym be abandoned and proposed "ANCA-associated granulomatous vasculitis."[6] The authors have since campaigned for other medical eponyms to be abandoned, too.[7]

As of November 7, 2010, Wegener's granulomatosis has been changed to Granulomatosis with polyangiitis by the American College of Rheumatology, American Society of Nephrology, and the European League Against Rheumatism. The name has been change to characterize the pathology of the disease rather than having it remain as a historical reference, as it has become evident that Dr. Wegener was associated with the Nazi party.[4][8]

Discovery

  • Granulomatosis with polyangitis was first discovered by Peter McBride, a Scottish otolaryngologist, in 1897.[1]
  • It was called as Wegener's granulomatosis, named after a German pathologist, Friedrich Wegener who described in detail.
  • The association between [important risk factor/cause] and [disease name] was made in/during [year/event].
  • In [year], [scientist] was the first to discover the association between [risk factor] and the development of [disease name].
  • In [year], [gene] mutations were first implicated in the pathogenesis of [disease name].

Landmark Events in the Development of Treatment Strategies

  • In [year], [diagnostic test/therapy] was developed by [scientist] to treat/diagnose [disease name].
  • The use of cyclophosphamide as a treatment for Wegener's granulomatosis was first administered to patients in 1973 at 2mg/kg/day with prednisone.[9]

Famous Cases

  • The following are a few famous cases of disease name:
    • Heinrich Hertz, famous German physicist who worked on electromagnetic waves[10]


References

  1. 1.0 1.1 Friedmann I (1982). "McBride and the midfacial granuloma syndrome. (The second 'McBride Lecture', Edinburgh, 1980)". The Journal of laryngology and otology. 96 (1): 1–23. PMID 7057076.
  2. Fienberg R (1955). "Pathergic granulomatosis". Am. J. Med. 19 (6): 829–31. doi:10.1016/0002-9343(55)90150-9. PMID 13275478.
  3. Mendenhall WM, Olivier KR, Lynch JW Jr, Mendenhall NP (2006). "Lethal midline granuloma-nasal natural killer/T-cell lymphoma". Am J Clin Oncol. 29 (2): 202–6. doi:10.1097/01.coc.0000198738.61238.eb. PMID 16601443.
  4. 4.0 4.1 Falk RJ, Gross WL, Guillevin L, Hoffman GS, Jayne DR, Jennette JC; et al. (2011). "Granulomatosis with polyangiitis (Wegener's): an alternative name for Wegener's granulomatosis". Arthritis Rheum. 63 (4): 863–4. doi:10.1002/art.30286. PMID 21374588.
  5. Woywodt A, Haubitz M, Haller H, Matteson EL (2006). "Wegener's granulomatosis". Lancet. 367 (9519): 1362–6. doi:10.1016/S0140-6736(06)68583-8. PMID 16631915.
  6. Woywodt A, Matteson EL (2006). "Wegener's granulomatosis--probing the untold past of the man behind the eponym". Rheumatology (Oxford). 45 (10): 1303–6. doi:10.1093/rheumatology/kel258. PMID 16887845.
  7. Woywodt A, Matteson E (2007). "Should eponyms be abandoned? Yes". BMJ. 335 (7617): 424. doi:10.1136/bmj.39308.342639.AD. PMID 17762033.
  8. Woywodt A, Haubitz M, Haller H, Matteson EL (2006). "Wegener's granulomatosis". Lancet. 367 (9519): 1362–6. doi:10.1016/S0140-6736(06)68583-8. PMID 16631915.
  9. Langford CA (2011). "Cyclophosphamide as induction therapy for Wegener's granulomatosis and microscopic polyangiitis". Clin Exp Immunol. 164 Suppl 1: 31–4. doi:10.1111/j.1365-2249.2011.04364.x. PMC 3095863. PMID 21447129.
  10. Feldmann H (June 2005). "[A historic case of Wegener's granulomatosis: the physicist who discovered the electromagnetic waves: Heinrich Hertz]". Laryngorhinootologie (in German). 84 (6): 426–31. doi:10.1055/s-2004-826062. PMID 15940574.

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