Back ورام حبيبي ويغنري Arabic Granulomatose mit Polyangiitis German Αγγειίτιδα Βέγκενερ Greek Granulomatosis con poliangitis Spanish گرانولوماتوز با پلی‌آنژیت Persian Wegenerin granulomatoosi Finnish Granulomatose avec polyangéite French גרנולומטוזיס עם פוליאנגאיטיס HE Գրանուլեմատոզ պոլիանգիիտ Armenian Granulomatosis Wegener ID

Granulomatosis with polyangiitis

Not to be confused with eosinophilic granulomatosis with polyangiitis.
Granulomatosis with polyangiitis
Other namesWegener's granulomatosis (WG) (formerly)
Micrograph showing features characteristic of granulomatosis with polyangiitis – a vasculitis and granulomas with multi-nucleated giant cells. H&E stain.
SpecialtyImmunology, rheumatology Edit this on Wikidata
CausesAutoimmune disease

Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis (WG),[1][2][3][4][5] after the German physician Friedrich Wegener, is a rare long-term systemic disorder that involves the formation of granulomas and inflammation of blood vessels (vasculitis). It is an autoimmune disease and a form of vasculitis that affects small- and medium-size vessels in many organs but most commonly affects the upper respiratory tract, lungs and kidneys.[6] The signs and symptoms of GPA are highly varied and reflect which organs are supplied by the affected blood vessels. Typical signs and symptoms include nosebleeds, stuffy nose and crustiness of nasal secretions, and inflammation of the uveal layer of the eye.[3] Damage to the heart, lungs and kidneys can be fatal.

The cause of GPA is unknown. Genetics have a role in GPA though the risk of inheritance appears to be low.[7]

GPA treatment depends on the severity of the disease.[8] Severe disease is typically treated with a combination of immunosuppressive medications such as rituximab or cyclophosphamide and high-dose corticosteroids to control the symptoms of the disease and azathioprine, methotrexate, or rituximab to keep the disease under control.[1][7][8] Plasma exchange is also used in severe cases with damage to the lungs, kidneys, or intestines.[9]

The number of new cases of GPA each year is estimated to be 2.1–14.4 new cases per million people in Europe.[3] GPA is rare in Japanese and African-American populations but occurs more often in people of Northern European descent.[7] GPA is estimated to affect 3 cases per 100,000 people in the United States and equally affects men and women.[10] GPA has infrequently been reported in minors.[11]

  1. ^ a b Singer, O; McCune, WJ (May 2017). "Update on maintenance therapy for granulomatosis with polyangiitis and microscopic polyangiitis". Current Opinion in Rheumatology (Review). 29 (3): 248–53. doi:10.1097/BOR.0000000000000382. PMID 28306595. S2CID 35805200.
  2. ^ Cite error: The named reference Lopalco2016 was invoked but never defined (see the help page).
  3. ^ a b c Yates, M; Watts, R (February 2017). "ANCA-associated vasculitis". Clinical Medicine (Review). 17 (1): 60–64. doi:10.7861/clinmedicine.17-1-60. PMC 6297586. PMID 28148583.
  4. ^ Lally, L; Spiera, R (2015). "Current landscape of antineutrophil cytoplasmic antibody-associated vasculitis: classification, diagnosis, and treatment". Rheumatic Disease Clinics of North America (Review). 41 (1): 1–19. doi:10.1016/j.rdc.2014.09.003. PMID 25399936.
  5. ^ Keller, SF; Miloslavsky, EM (February 2016). "Corticosteroids in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis". Rheumatic Disease Clinics of North America. 42 (1): 91–101. doi:10.1016/j.rdc.2015.08.010. PMID 26611553.
  6. ^ Papaliodis, GN (November 2017). "Ophthalmologic manifestations of systemic vasculitis". Current Opinion in Ophthalmology (Review). 28 (6): 613–16. doi:10.1097/ICU.0000000000000422. PMID 28817388. S2CID 36254262.
  7. ^ a b c Millet, A; Pederzoli-Ribeil, M; Guillevin, L; Witko-Sarsat, V; Mouthon, L (August 2013). "Antineutrophil cytoplasmic antibody-associated vasculitides: is it time to split up the group?". Annals of the Rheumatic Diseases (Review). 72 (8): 1273–9. doi:10.1136/annrheumdis-2013-203255. PMID 23606701. S2CID 206849855.
  8. ^ a b Lally, L; Spiera, R (2015). "Current therapies for ANCA-associated vasculitis". Annual Review of Medicine (Review). 66: 227–40. doi:10.1146/annurev-med-011514-023051. PMID 25341007.
  9. ^ Walters, Giles D.; Willis, Narelle S.; Cooper, Tess E.; Craig, Jonathan C. (2020-01-13). "Interventions for renal vasculitis in adults". The Cochrane Database of Systematic Reviews. 1 (1): CD003232. doi:10.1002/14651858.CD003232.pub4. ISSN 1469-493X. PMC 6956643. PMID 31927782.
  10. ^ Pakalniskis, MG; Berg, AD; Policeni, BA; Gentry, LR; Sato, Y; Moritani, T; Smoker, WR (December 2015). "The Many Faces of Granulomatosis With Polyangiitis: A Review of the Head and Neck Imaging Manifestations". AJR. American Journal of Roentgenology (Review). 205 (6): W619–29. doi:10.2214/AJR.14.13864. PMID 26587951.
  11. ^ Treitman, P; Herskowitz, JL; Bass, HN (August 1991). "Churg-Strauss syndrome in a 12-year-old boy diagnosed by transbronchial biopsy". Clinical Pediatrics (Case Report). 30 (8): 502–505. doi:10.1177/clinpediatr.30.8.502. PMID 1914353.
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