Granulomatosis With Polyangiitis

Description

The eponym ‘Wegener Granulomatosis’ has been replaced with the term ‘Granulomatosis with polyangiitis (GPA)‘. This transition was in part to achieve nomenclature symmetry with Microscopic Polyangiitis (MPA) and Eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss); and to remove any historical association with Friedrich Wegener following revelations of his association with the Nazi Party in World War II

  • GPA is necrotizing granulomatous inflammation usually involving the upper and lower respiratory tract, and necrotizing vasculitis affecting predominantly small to medium vessels
  • Necrotizing glomerulonephritis is common. Ocular vasculitis and pulmonary capillaritis with hemorrhage are frequent.

History

Retrospective review of the literature leads to multiple previous descriptions of GPA under terms including Giant-Cell Granuloma of the respiratory tract (Walton); Idiopathic lethal granuloma; Malignant granuloma; Respiratory-renal type of polyarteritis nodosa; Rhinogenous granuloma (Wegener); Necrotizing granulomatosis with angiitis; and Pathergic granulomatosis (Feldman).

1897 – McBride presented a photographic example of Idiopathic Midfacial Pleomorphic Granuloma (Stewart’s type) to the Laryngological Society Of London

1931 – Klinger first described GPA as a variant of polyarteritis nodosa. He reported the autopsy findings of two patients with ulcerative sinusitis, splenic granulomas, and nephritis in a paper entitled entitled ‘Borderline Forms of Periarteritis Nodosa’.

1934Wegener performed the autopsy on a 38 year old truck driver who had died from uraemia after prolonged febrile illness. He noted mouth and stomach ulceration and a saddle nose deformity. Histological examination revealed generalised angiitis and multiple necrotic granulomas affecting the nose, trachea, lung, kidney, and spleen. Wegener was sure that this was a new condition, clinically and pathologically distinct from periarteritis nodosa

1936  – Wegener observed two similar cases of angiitis with granulomas and described the condition (GPA) as ‘rhinogenous granuloma’ associated with diffuse vascular disease a distinct entity to PAN in two articles. Firstly, in 1936 ‘On Generalised Septic Vessel Diseases‘  and in more detail in 1939 ‘On a Peculiar Rhinogenic Granuloma with particular involvement of the arterial system and the kidney.

1947 – Ringertz, a Norwegian pathologist, described a special form of periarteritis nodosa, which he called ‘Wegener’s granulomatos‘.

1948 – Sven Johnsson, a Swedish pathologist first recognised Wegener’s granulomatosis as a distinct entity, separate from periarteritis nodosa.

1951Churg and Strauss described 12 cases with a striking resemblance to GPA. However the cases demonstrated a marked eosinophilia and most of the patients were atopic they concluded that they were dealing with a new entity, which they entitled ‘allergic granulomatosis.

1954 – Godman and Churg reviewed 22 cases from the literature; added 7 of their own; summarized the salient features; tabulated characteristic clinical and pathologic findings; and termed then condition ‘Wegener’s granulomatosis.‘ They proposed the diagnostic triad of

  • necrotising granuloma in the upper and lower respiratory tract,
  • generalised focal necrotising vasculitis affecting arteries and veins, and
  • glomerulonephritis with necrosis and granulomas.

2010 – 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 move towards a vasculitis terminology based on pathology, rather than historical reference, was triggered by evidence that Dr. Friedrich Wegener was an active member of the Nazi party before and during World War II. 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

2011

2012 – CHCC2012 adopted the recommendation of the American College of Rheumatology, the American Society of Nephrology, and the European League Against Rheumatism to replace “Wegener’s granulomatosis” with “granulomatosis with polyangiitis (Wegener’s)”


Associated Persons


Alternative names

  • Granulomatosis with polyangiitis (GPA)
  • Wegener’s Granulomatosis (Rhinogenous granuloma)

Controversies

2006 – Woywodt and Matteson publish on Wegener’s granulmatosis (Lancet, 2006) and record in detail the significant historical review undertaken to connect Wegener with potential war crimes (Rheumatology, 2006). They conclude: “The facts we have uncovered do not prove Dr Friedrich Wegener guilty of war crimes. However, the evidence suggests that Dr Wegener was, at least at some point of his career, a follower of the Nazi regime…his mentor Martin Staemmler, was an ardent supporter of the racial hygiene…he (Wegener) worked in close proximity to the genocide machinery in Lodz…our data raise serious concerns about Dr Wegener’s professional conduct

2012 – Chapel Hill Consensus Conference (CHCC 2012) provided a consensus for the use of descriptive wording to make nomenclature more consistent. ANCA-associated vasculitides include Microscopic Polyangiitis (MPA); Granulomatosis with Polyangiitis (GPA, Wegener); and Eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss). The polyangiitis is similar in all three conditions


References

  • McBride P. Photographs of a Case of Rapid Destruction of the nose and Face. In: Semon F. The Laryngological Society Of London. The Journal of Laryngology, Rhinology, and Otology, 1897; 12(02), 64-66
  • Friedmann I. McBride and the Midfacial Granuloma Syndrome. 1982;96(1) :1-23
  • Klinger H. Grenzformen der Periarteriitis Nodosa. Frankfurter Zeitschrift für Pathologie. 1931;42:455–480.
  • Wegener F. Über generalisierte septische Gefäßerkrankungen. Verhandlungen der Deutschen Pathologischen Gesellschaft. 1936;29:202–210. [Translation: On generalised septic vessel diseases. Thorax. 1987 Dec; 42(12): 918–919. [PMC461047]]
  • Wegener F. Über eine eigenartige rhinogene Granulomatose mit besonderer Beteiligung des Arterien systems und der Nieren. Beiträge zur Pathologie. 1939;102:30–68. [Re-print: Beiträge zur Pathologie. 1976;158(1):127-143]
  • Ringertz N. En egenartad form av periarteritis nodosa (Wegener’s granulomatos). Nordisk Medicin 1947;36:2252-2253.
  • Johnsson S. A case of Wegener’s granulomatosis. Acta Pathol Microbiol Scand 1948;25:573-84. [PMID 18102532]
  • Strauss L, Churg J, Zak FG. Cutaneous lesions of allergic granulomatosis: a histopathologic study. J Invest Dermatol. 1951;17(6):349-359. [PMID 14908172]
  • Godman GC, Churg J. Wegener’s granulomatosis: pathology and review of the literature. AMA Arch Pathol. 1954;58(6):533-553. [PMID 13217569]
  • De Oreo GA. Wegener’s Granulomatosis. AMA Arch Derm. 1960;81(2):169-174.
  • Falk RJ et al. Granulomatosis with polyangiitis (Wegener’s): an alternative name for Wegener’s granulomatosis. Arthritis Rheum. 2011 Apr;63(4):863-4. [PMID 21374588]
  • Jennette JC, Falk RJ et al 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013 Jan;65(1):1-11. [PMID 23045170]

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Emergency physician MA (Oxon) MBChB (Edin) FACEM FFSEM with a passion for rugby; medical history; medical education; and informatics. Asynchronous learning #FOAMed evangelist. Co-founder and CTO of Life in the Fast lane | Eponyms | Books | vocortex |

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