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Granulomatosis with polyangiitis

granulomatosis with polyangiitis, granulomatosis with polyangiitis icd 10
Granulomatosis with polyangiitis GPA, previously known as Wegener's granulomatosis WG, is a systemic disorder that involves both granulomatosis and polyangiitis It is a form of vasculitis inflammation of blood vessels that affects small- and medium-size vessels in many organs Damage to the lungs and kidneys can be fatal Treatment requires long-term immunosuppression1

Granulomatosis with polyangiitis is part of a larger group of vasculitic syndromes called systemic vasculitides or necrotizing vasculopathies, all of which feature an autoimmune attack by an abnormal type of circulating antibody termed ANCAs antineutrophil cytoplasmic antibodies against small and medium-size blood vessels Apart from GPA, this category includes eosinophilic granulomatosis with polyangiitis EGPA and microscopic polyangiitis1 Although GPA affects small- and medium-size vessels,2 it is formally classified as one of the small vessel vasculitides in the Chapel Hill system34


  • 1 Signs and symptoms
  • 2 Causes
  • 3 Pathophysiology
  • 4 Diagnosis
    • 41 Criteria
  • 5 Treatment
  • 6 Prognosis
  • 7 Epidemiology
  • 8 History
  • 9 See also
  • 10 References
  • 11 External links

Signs and symptomsedit

Typical saddle nose damage due to granulomatosis with polyangiitis

Initial signs are extremely variable, and diagnosis can be severely delayed due to the nonspecific nature of the symptoms In general, rhinitis is the first sign in most people15

  • Kidney: rapidly progressive glomerulonephritis 75%, leading to chronic kidney failure
  • Upper airway, eye and ear disease:
    • Nose: pain, stuffiness, nosebleeds, rhinitis, crusting, saddle-nose deformity due to a perforated septum
    • Ears: conductive hearing loss due to auditory tube dysfunction, sensorineural hearing loss unclear mechanism
    • Oral cavity: strawberry gingivitis,6 underlying bone destruction with loosening of teeth, non-specific ulcerations throughout oral mucosa
    • Eyes: pseudotumours, scleritis, conjunctivitis, uveitis, episcleritis
  • Trachea: subglottal stenosis
  • Lungs: pulmonary nodules referred to as "coin lesions", infiltrates often interpreted as pneumonia, cavitary lesions, pulmonary haemorrhage causing haemoptysis, and rarely bronchial stenosis
  • Arthritis: Pain or swelling 60%, often initially diagnosed as rheumatoid arthritis
  • Skin: nodules on the elbow, purpura, various others see cutaneous vasculitis
  • Nervous system: occasionally sensory neuropathy 10% and rarely mononeuritis multiplex
  • Heart, gastrointestinal tract, brain, other organs: rarely affected


Its causes are unknown, although microbes, such as bacteria and viruses, as well as genetics have been implicated in its pathogenesis57


Inflammation with granuloma formation against a nonspecific inflammatory background is the classical tissue abnormality in all organs affected by GPA1

It is now widely presumed that the anti-neutrophil cytoplasmic antibodies ANCAs are responsible for the inflammation in GPA1 The typical ANCAs in GPA are those that react with proteinase 3, an enzyme prevalent in neutrophil granulocytes8

In vitro studies have found that ANCAs can activate neutrophils, increase their adherence to endothelium, and induce their degranulation that can damage endothelial cells In theory, this phenomenon could cause extensive damage to the vessel wall, in particular of arterioles1


Immunofluorescence pattern produced by binding of ANCA to ethanol-fixed neutrophils, from a person with GPA Photo showing the sclerokeratitis associated with GPA

Granulomatosis with polyangiitis is usually suspected only when a person has had unexplained symptoms for a long period of time Determination of Anti-neutrophil cytoplasmic antibodies ANCAs can aid in the diagnosis, but positivity is not conclusive and negative ANCAs are not sufficient to reject the diagnosis Cytoplasmic-staining ANCAs that react with the enzyme proteinase 3 cANCA in neutrophils a type of white blood cell are associated with GPA1

If the person has kidney failure or cutaneous vasculitis, a biopsy is obtained from the kidneys On rare occasions, thoracoscopic lung biopsy is required On histopathological examination, a biopsy will show leukocytoclastic vasculitis with necrotic changes and granulomatous inflammation clumps of typically arranged white blood cells on microscopy These granulomas are the main reason for the name granulomatosis with polyangiitis, although it is not an essential feature Nevertheless, necrotizing granulomas are a hallmark of this disease However, many biopsies can be nonspecific and 50% provide too little information for the diagnosis of GPA1


In 1990, the American College of Rheumatology accepted classification criteria for GPA These criteria were not intended for diagnosis, but for inclusion in randomized controlled trials Two or more positive criteria have a sensitivity of 882% and a specificity of 920% of describing GPA9

  • Nasal or oral inflammation:
    • painful or painless oral ulcers or
    • purulent or bloody nasal discharge
  • Lungs: abnormal chest X-ray with:
    • nodules,
    • infiltrates or
    • cavities
  • Kidneys: urinary sediment with:
    • microhematuria or
    • red cell casts
  • Biopsy: granulomatous inflammation
    • within the arterial wall or
    • in the perivascular area

According to the Chapel Hill Consensus Conference CHCC on the nomenclature of systemic vasculitis 1992, establishing the diagnosis of GPA demands:10

  • a granulomatous inflammation involving the respiratory tract, and
  • a vasculitis of small to medium-size vessels

Several investigators have compared the ACR and Chapel Hill criteria11


The standard treatment for GPA is cyclophosphamide and high dose corticosteroids for remission induction and less toxic immunosuppressants like azathioprine, leflunomide, methotrexate or mycophenolate mofetil12 Trimethoprim/sulfamethoxazole may also help prevent relapse12 Rituximab may be substituted for cyclophosphamide in inducing remission1213 A systematic review of 84 trials examined the evidence for various treatments in GPA Many trials include data on pooled groups of people with GPA and microscopic polyangiitis In this review, cases are divided between localised disease, non-organ threatening, generalized organ-threatening disease and severe kidney vasculitis and immediately life-threatening disease14

  • In generalised non-organ-threatening disease, remission can be induced with methotrexate and steroids, where the steroid dose is reduced after a remission has been achieved and methotrexate used as maintenance
  • In case of organ-threatening disease, pulsed intravenous cyclophosphamide with steroids is recommended Once remission has been achieved, azathioprine and steroids can be used to maintain remission
  • In severe kidney vasculitis, the same regimen is used but with the addition of plasma exchange
  • In pulmonary haemorrhage, high doses of cyclophosphamide with pulsed methylprednisolone may be used, or alternatively CYC, steroids, and plasma exchange

Therapy for GPA and MPA has two main components: induction of remission with initial immunosuppressive therapy, and maintenance of remission with immunosuppressive therapy for a variable period to prevent relapse The mainstay of treatment for granulomatosis with polyangiitis GPA is a combination of corticosteroids and cytotoxic agents

  • Medications
  • Side effect treatments
  • Plasma exchange
  • Kidney transplant


Before modern treatments, the 2-year mortality was over 90% and average survival five months515 Death usually resulted from uremia or respiratory failure5

With corticosteroids and cyclophosphamide, 5-year survival is over 80%5 Long-term complications are common 86%, mainly chronic kidney failure, hearing loss and deafness1

Today, drug toxicity is managed more carefully and long-term remissions are possible Some patients are able to lead relatively normal lives and remain in remission for 20+ years after treatment16


The incidence is 10–20 cases per million per year1417 It is exceedingly rare in Japan and with African Americans17


Scottish otolaryngologist Peter McBride 1854–1946 first described the condition in 1897 in a BMJ article entitled "Photographs of a case of rapid destruction of the nose and face"18 Heinz Karl Ernst Klinger born 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 1939,19 leading to the name Wegener's granulomatosis or Wegener granulomatosis English: /ˈvɛɡənər/

An earlier name for the disease was pathergic granulomatosis20 The disease is still sometimes confused with lethal midline granuloma and lymphomatoid granulomatosis, both malignant lymphomas21

In 2006, Alexander Woywodt Preston, United Kingdom and Eric Matteson Mayo Clinic, USA investigated 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 indicate that Wegener was wanted by Polish authorities and that his files were forwarded to the United Nations War Crimes Commission Furthermore, Wegener worked in close proximity to the genocide machinery in Łódź Their data raised serious concerns about Wegener's professional conduct They suggest that the eponym should be abandoned and propose "ANCA-associated granulomatous vasculitis"22 The authors have since campaigned for other medical eponyms to be abandoned, too23 In 2011, the American College of Rheumatology ACR, the American Society of Nephrology ASN and the European League Against Rheumatism EULAR resolved to change the name to granulomatosis with polyangiitis24

See alsoedit

  • List of medical eponyms with Nazi associations


  1. ^ a b c d e f g h i Seo P, Stone JH July 2004 "The antineutrophil cytoplasmic antibody-associated vasculitides" Am J Med 117 1: 39–50 PMID 15210387 doi:101016/jamjmed200402030 
  2. ^ Gota, CE May 2013 "Granulomatosis with Polyangiitis GPA: Vasculitis" Merck Manual Professional Merck Sharp & Dohme Corp Retrieved 16 March 2014 
  3. ^ Silva, Fred; Jennette, J Charles; Heptinstall, Robert H; Olson, Jean T; Schwartz, Melvin 2007 Hepinstall's pathology of the kidney Hagerstwon, MD: Lippincott Williams & Wilkins p 677 ISBN 0-7817-4750-3 
  4. ^ Ottoman BAE: Strawberry gingivitis of Wegener’s granulomatosis: A clinicopathological and immunohistochemical study and review of literatureContemporary Journal immunology: Vol 2 No 1 pp 59-67
  5. ^ a b c d e Berden, A; Göçeroglu, A; Jayne, D; Luqmani, R; Rasmussen, N; Bruijn, JA; Bajema, I January 2012 "Diagnosis and management of ANCA associated vasculitis" BMJ 344: e26 PMID 22250224 doi:101136/bmje26 
  6. ^ Ottoman, Bacem 2015 "Strawberry Gingivitis of Wegener’s Granulomatosis: A Clinico-pathological and Immunohistochemical Case Study with Review of Literature" Journal of Contemporary Immunology doi:107726/jci20151004 
  7. ^ Tracy, CL; Papadopoulos, PJ; Bye, MR; Connolly, H; Goldberg, E; O'Brian, RJ; Sharma, GD; Talavera, F; Toder, DS; Valentini, RP; Windle, ML; Wolf, RE 10 February 2014 Diamond, HS, ed "Granulomatosis with Polyangiitis" Medscape Reference WebMD Retrieved 16 March 2014 
  8. ^ van der Woude FJ, Rasmussen N, Lobatto S, et al February 1985 "Autoantibodies against neutrophils and monocytes: tool for diagnosis and marker of disease activity in Wegener's granulomatosis" Lancet 1 8426: 425–9 PMID 2857806 doi:101016/S0140-67368591147-X 
  9. ^ Leavitt RY, Fauci AS, Bloch DA, et al August 1990 "The American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis" Arthritis Rheum 33 8: 1101–7 PMID 2202308 doi:101002/art1780330807 
  10. ^ Jennette JC, Falk RJ, Andrassy K, et al February 1994 "Nomenclature of systemic vasculitides Proposal of an international consensus conference" Arthritis Rheum 37 2: 187–92 PMID 8129773 doi:101002/art1780370206 
  11. ^ Bruce IN, Bell AL April 1997 "A comparison of two nomenclature systems for primary systemic vasculitis" Br J Rheumatol 36 4: 453–8 PMID 9159539 doi:101093/rheumatology/364453 
  12. ^ a b c Tracy, CL; Papadopoulos, PJ; Bye, MR; Connolly, H; Goldberg, E; O'Brian, RJ; Sharma, GD; Talavera, F; Toder, DS; Valentini, RP; Windle, ML; Wolf, RE 10 February 2014 Diamond, HS, ed "Granulomatosis with Polyangiitis Treatment & Management" Medscape Reference WebMD Retrieved 16 March 2014 
  13. ^ Tarabishy, AB; Schulte, M; Papaliodis, GN; Hoffman, GS September–October 2010 "Wegener's granulomatosis: clinical manifestations, differential diagnosis, and management of ocular and systemic disease" Survey of Ophthalmology 55 5: 429–44 PMID 20638092 doi:101016/jsurvophthal200912003 
  14. ^ a b Bosch X, Guilabert A, Espinosa G, Mirapeix E 2007 "Treatment of antineutrophil cytoplasmic antibody associated vasculitis: a systematic review" JAMA 298 6: 655–69 PMID 17684188 doi:101001/jama2986655 
  15. ^ Smith, RM; Jones, RB; Jayne, DR April 2012 "Progress in treatment of ANCA-associated vasculitis" PDF Arthritis Research & Therapy 14 2: 210 PMC 3446448  PMID 22569190 doi:101186/ar3797 
  16. ^ "Vasculitis Foundation » Granulomatosis with Polyangiitis GPA/Wegener’s" wwwvasculitisfoundationorg Retrieved 2016-03-16 
  17. ^ a b Cartin-Ceba, R; Peikert, T; Specks, U December 2012 "Pathogenesis of ANCA-associated vasculitis" Current Rheumatology Reports 14 6: 481–93 PMID 22927039 doi:101007/s11926-012-0286-y 
  18. ^ 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 doi:101017/s0022215100092197 
  19. ^ synd/2823 at Who Named It
  20. ^ Fienberg R 1955 "Pathergic granulomatosis" Am J Med 19 6: 829–31 PMID 13275478 doi:101016/0002-93435590150-9 
  21. ^ 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 PMID 16601443 doi:101097/01coc000019873861238eb 
  22. ^ Woywodt A, Matteson EL 2006 "Wegener's granulomatosis—probing the untold past of the man behind the eponym" Rheumatology Oxford 45 10: 1303–6 PMID 16887845 doi:101093/rheumatology/kel258 
  23. ^ Woywodt A, Matteson E 2007 "Should eponyms be abandoned Yes" BMJ 335 7617: 424 PMC 1962844  PMID 17762033 doi:101136/bmj39308342639AD 
  24. ^ Falk RJ, Gross WL, Guillevin L, et al 2011 "Granulomatosis with polyangiitis Wegener's: An alternative name for Wegener's granulomatosis" Ann Rheum Dis 70: 74 PMID 21372195 doi:101136/ard2011150714 

External linksedit

granulomatosis with polyangiitis, granulomatosis with polyangiitis (wegener's disease), granulomatosis with polyangiitis (wegener's), granulomatosis with polyangiitis icd 10, granulomatosis with polyangiitis life span, granulomatosis with polyangiitis nose, granulomatosis with polyangiitis pulmonary involvement, granulomatosis with polyangiitis skin involvement, granulomatosis with polyangiitis symptoms, granulomatosis with polyangiitis treatment

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