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Eosinophilic granulomatosis with polyangiitis

MedGen UID:
3088
•Concept ID:
C0008728
•
Disease or Syndrome
Synonyms: Allergic Angiitides; Allergic Angiitides, Granulomatous; Allergic Angiitis; Allergic Angiitis and Granulomatosis; Allergic Angiitis, Granulomatous; Allergic Granulomatoses; Allergic Granulomatosis; Allergic Granulomatous and Angiitis; Allergic Granulomatous Angiitides; Allergic Granulomatous Angiitis; Angiitides, Allergic; Angiitides, Allergic Granulomatous; Angiitides, Granulomatous Allergic; Angiitis, Allergic; Angiitis, Allergic Granulomatous; Angiitis, Granulomatous Allergic; Churg Strauss Syndrome; Churg-Strauss Syndrome; Churg-Strauss Vasculitis; Eosinophilic Granulomatous Vasculitides; Eosinophilic Granulomatous Vasculitis; Granulomatoses, Allergic; Granulomatosis, Allergic; Granulomatous Allergic Angiitides; Granulomatous Allergic Angiitis; Granulomatous Angiitides, Allergic; Granulomatous Angiitis, Allergic; Granulomatous Vasculitides, Eosinophilic; Granulomatous Vasculitis, Eosinophilic; Syndrome, Churg-Strauss; Vasculitides, Eosinophilic Granulomatous; Vasculitis, Churg Strauss; Vasculitis, Churg-Strauss; Vasculitis, Eosinophilic Granulomatous
SNOMED CT: Allergic granulomatous angiitis (82275008); CSS - Churg-Strauss syndrome (82275008); Allergic granulomatosis angiitis (82275008); Churg-Strauss syndrome (82275008); Churg Strauss syndrome (82275008); Eosinophilic granulomatosis with polyangiitis (82275008); EGPA - eosinophilic granulomatosis with polyangiitis (82275008)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0015943
Orphanet: ORPHA183

Definition

A rare systemic vasculitis of small vessels characterized by asthma, blood and tissue eosinophilia and vasculitis manifestations. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVEosinophilic granulomatosis with polyangiitis
Follow this link to review classifications for Eosinophilic granulomatosis with polyangiitis in Orphanet.

Professional guidelines

PubMed

Treatment Guidelines in Vasculitis.
Kermani TA, Warrington KJ, Dua AB
Rheum Dis Clin North Am 2022 Aug;48(3):705-724. Epub 2022 Jul 5 doi: 10.1016/j.rdc.2022.03.006. PMID: 35953232
2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.
Chung SA, Langford CA, Maz M, Abril A, Gorelik M, Guyatt G, Archer AM, Conn DL, Full KA, Grayson PC, Ibarra MF, Imundo LF, Kim S, Merkel PA, Rhee RL, Seo P, Stone JH, Sule S, Sundel RP, Vitobaldi OI, Warner A, Byram K, Dua AB, Husainat N, James KE, Kalot MA, Lin YC, Springer JM, Turgunbaev M, Villa-Forte A, Turner AS, Mustafa RA
Arthritis Rheumatol 2021 Aug;73(8):1366-1383. Epub 2021 Jul 8 doi: 10.1002/art.41773. PMID: 34235894
Updates for the treatment of EGPA.
Raffray L, Guillevin L
Presse Med 2020 Oct;49(3):104036. Epub 2020 Jul 8 doi: 10.1016/j.lpm.2020.104036. PMID: 32652104

Recent clinical studies

Etiology

Eosinophilic granulomatosis with polyangiitis: A review.
White J, Dubey S
Autoimmun Rev 2023 Jan;22(1):103219. Epub 2022 Oct 22 doi: 10.1016/j.autrev.2022.103219. PMID: 36283646
Treatment Guidelines in Vasculitis.
Kermani TA, Warrington KJ, Dua AB
Rheum Dis Clin North Am 2022 Aug;48(3):705-724. Epub 2022 Jul 5 doi: 10.1016/j.rdc.2022.03.006. PMID: 35953232
2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis.
Grayson PC, Ponte C, Suppiah R, Robson JC, Craven A, Judge A, Khalid S, Hutchings A, Luqmani RA, Watts RA, Merkel PA; DCVAS Study Group
Ann Rheum Dis 2022 Mar;81(3):309-314. Epub 2022 Feb 2 doi: 10.1136/annrheumdis-2021-221794. PMID: 35110334
ANCA-Associated Vasculitis: Core Curriculum 2020.
Geetha D, Jefferson JA
Am J Kidney Dis 2020 Jan;75(1):124-137. Epub 2019 Jul 26 doi: 10.1053/j.ajkd.2019.04.031. PMID: 31358311
Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis.
Wechsler ME, Akuthota P, Jayne D, Khoury P, Klion A, Langford CA, Merkel PA, Moosig F, Specks U, Cid MC, Luqmani R, Brown J, Mallett S, Philipson R, Yancey SW, Steinfeld J, Weller PF, Gleich GJ; EGPA Mepolizumab Study Team
N Engl J Med 2017 May 18;376(20):1921-1932. doi: 10.1056/NEJMoa1702079. PMID: 28514601Free PMC Article

Diagnosis

Eosinophilic granulomatosis with polyangiitis: A review.
White J, Dubey S
Autoimmun Rev 2023 Jan;22(1):103219. Epub 2022 Oct 22 doi: 10.1016/j.autrev.2022.103219. PMID: 36283646
Treatment Guidelines in Vasculitis.
Kermani TA, Warrington KJ, Dua AB
Rheum Dis Clin North Am 2022 Aug;48(3):705-724. Epub 2022 Jul 5 doi: 10.1016/j.rdc.2022.03.006. PMID: 35953232
2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis.
Grayson PC, Ponte C, Suppiah R, Robson JC, Craven A, Judge A, Khalid S, Hutchings A, Luqmani RA, Watts RA, Merkel PA; DCVAS Study Group
Ann Rheum Dis 2022 Mar;81(3):309-314. Epub 2022 Feb 2 doi: 10.1136/annrheumdis-2021-221794. PMID: 35110334
Update on eosinophilic granulomatosis with polyangiitis.
Furuta S, Iwamoto T, Nakajima H
Allergol Int 2019 Oct;68(4):430-436. Epub 2019 Jun 29 doi: 10.1016/j.alit.2019.06.004. PMID: 31266709
ANCA-associated vasculitis.
Yates M, Watts R
Clin Med (Lond) 2017 Feb;17(1):60-64. doi: 10.7861/clinmedicine.17-1-60. PMID: 28148583Free PMC Article

Therapy

Eosinophilic granulomatosis with polyangiitis: A review.
White J, Dubey S
Autoimmun Rev 2023 Jan;22(1):103219. Epub 2022 Oct 22 doi: 10.1016/j.autrev.2022.103219. PMID: 36283646
2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.
Chung SA, Langford CA, Maz M, Abril A, Gorelik M, Guyatt G, Archer AM, Conn DL, Full KA, Grayson PC, Ibarra MF, Imundo LF, Kim S, Merkel PA, Rhee RL, Seo P, Stone JH, Sule S, Sundel RP, Vitobaldi OI, Warner A, Byram K, Dua AB, Husainat N, James KE, Kalot MA, Lin YC, Springer JM, Turgunbaev M, Villa-Forte A, Turner AS, Mustafa RA
Arthritis Rheumatol 2021 Aug;73(8):1366-1383. Epub 2021 Jul 8 doi: 10.1002/art.41773. PMID: 34235894
ANCA-Associated Vasculitis: Core Curriculum 2020.
Geetha D, Jefferson JA
Am J Kidney Dis 2020 Jan;75(1):124-137. Epub 2019 Jul 26 doi: 10.1053/j.ajkd.2019.04.031. PMID: 31358311
Update on eosinophilic granulomatosis with polyangiitis.
Furuta S, Iwamoto T, Nakajima H
Allergol Int 2019 Oct;68(4):430-436. Epub 2019 Jun 29 doi: 10.1016/j.alit.2019.06.004. PMID: 31266709
Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis.
Wechsler ME, Akuthota P, Jayne D, Khoury P, Klion A, Langford CA, Merkel PA, Moosig F, Specks U, Cid MC, Luqmani R, Brown J, Mallett S, Philipson R, Yancey SW, Steinfeld J, Weller PF, Gleich GJ; EGPA Mepolizumab Study Team
N Engl J Med 2017 May 18;376(20):1921-1932. doi: 10.1056/NEJMoa1702079. PMID: 28514601Free PMC Article

Prognosis

Eosinophilic granulomatosis with polyangiitis: A review.
White J, Dubey S
Autoimmun Rev 2023 Jan;22(1):103219. Epub 2022 Oct 22 doi: 10.1016/j.autrev.2022.103219. PMID: 36283646
Immunopathogenesis of ANCA-Associated Vasculitis.
Kronbichler A, Lee KH, Denicolò S, Choi D, Lee H, Ahn D, Kim KH, Lee JH, Kim H, Hwang M, Jung SW, Lee C, Lee H, Sung H, Lee D, Hwang J, Kim S, Hwang I, Kim DY, Kim HJ, Cho G, Cho Y, Kim D, Choi M, Park J, Park J, Tizaoui K, Li H, Smith L, Koyanagi A, Jacob L, Gauckler P, Shin JI
Int J Mol Sci 2020 Oct 3;21(19) doi: 10.3390/ijms21197319. PMID: 33023023Free PMC Article
ANCA-Associated Vasculitis: Core Curriculum 2020.
Geetha D, Jefferson JA
Am J Kidney Dis 2020 Jan;75(1):124-137. Epub 2019 Jul 26 doi: 10.1053/j.ajkd.2019.04.031. PMID: 31358311
Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss).
Nguyen Y, Guillevin L
Semin Respir Crit Care Med 2018 Aug;39(4):471-481. Epub 2018 Nov 7 doi: 10.1055/s-0038-1669454. PMID: 30404114
Churg-Strauss syndrome.
Greco A, Rizzo MI, De Virgilio A, Gallo A, Fusconi M, Ruoppolo G, Altissimi G, De Vincentiis M
Autoimmun Rev 2015 Apr;14(4):341-8. Epub 2014 Dec 11 doi: 10.1016/j.autrev.2014.12.004. PMID: 25500434

Clinical prediction guides

2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis.
Grayson PC, Ponte C, Suppiah R, Robson JC, Craven A, Judge A, Khalid S, Hutchings A, Luqmani RA, Watts RA, Merkel PA; DCVAS Study Group
Ann Rheum Dis 2022 Mar;81(3):309-314. Epub 2022 Feb 2 doi: 10.1136/annrheumdis-2021-221794. PMID: 35110334
Immunopathogenesis of ANCA-Associated Vasculitis.
Kronbichler A, Lee KH, Denicolò S, Choi D, Lee H, Ahn D, Kim KH, Lee JH, Kim H, Hwang M, Jung SW, Lee C, Lee H, Sung H, Lee D, Hwang J, Kim S, Hwang I, Kim DY, Kim HJ, Cho G, Cho Y, Kim D, Choi M, Park J, Park J, Tizaoui K, Li H, Smith L, Koyanagi A, Jacob L, Gauckler P, Shin JI
Int J Mol Sci 2020 Oct 3;21(19) doi: 10.3390/ijms21197319. PMID: 33023023Free PMC Article
Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss).
Nguyen Y, Guillevin L
Semin Respir Crit Care Med 2018 Aug;39(4):471-481. Epub 2018 Nov 7 doi: 10.1055/s-0038-1669454. PMID: 30404114
Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis.
Wechsler ME, Akuthota P, Jayne D, Khoury P, Klion A, Langford CA, Merkel PA, Moosig F, Specks U, Cid MC, Luqmani R, Brown J, Mallett S, Philipson R, Yancey SW, Steinfeld J, Weller PF, Gleich GJ; EGPA Mepolizumab Study Team
N Engl J Med 2017 May 18;376(20):1921-1932. doi: 10.1056/NEJMoa1702079. PMID: 28514601Free PMC Article
Churg-Strauss syndrome.
Greco A, Rizzo MI, De Virgilio A, Gallo A, Fusconi M, Ruoppolo G, Altissimi G, De Vincentiis M
Autoimmun Rev 2015 Apr;14(4):341-8. Epub 2014 Dec 11 doi: 10.1016/j.autrev.2014.12.004. PMID: 25500434

Recent systematic reviews

Performance of MPO-ANCA and PR3-ANCA immunoassays for the stratification of specific ANCA-associated vasculitis: A systematic review and meta-analysis.
Walker BS, Peterson LK, Koening C, White SK, Schmidt RL, Tebo AE
Autoimmun Rev 2022 Jun;21(6):103100. Epub 2022 Apr 19 doi: 10.1016/j.autrev.2022.103100. PMID: 35452854
Myocarditis in systemic immune-mediated diseases: Prevalence, characteristics and prognosis. A systematic review.
Cheng CY, Baritussio A, Giordani AS, Iliceto S, Marcolongo R, Caforio ALP
Autoimmun Rev 2022 Apr;21(4):103037. Epub 2022 Jan 5 doi: 10.1016/j.autrev.2022.103037. PMID: 34995763
2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.
Chung SA, Langford CA, Maz M, Abril A, Gorelik M, Guyatt G, Archer AM, Conn DL, Full KA, Grayson PC, Ibarra MF, Imundo LF, Kim S, Merkel PA, Rhee RL, Seo P, Stone JH, Sule S, Sundel RP, Vitobaldi OI, Warner A, Byram K, Dua AB, Husainat N, James KE, Kalot MA, Lin YC, Springer JM, Turgunbaev M, Villa-Forte A, Turner AS, Mustafa RA
Arthritis Rheumatol 2021 Aug;73(8):1366-1383. Epub 2021 Jul 8 doi: 10.1002/art.41773. PMID: 34235894
Vasculitis therapy refines vasculitis mechanistic classification.
Torp CK, Brüner M, Keller KK, Brouwer E, Hauge EM, McGonagle D, Kragstrup TW
Autoimmun Rev 2021 Jun;20(6):102829. Epub 2021 Apr 16 doi: 10.1016/j.autrev.2021.102829. PMID: 33872767
Central nervous system involvement in eosinophilic granulomatosis with polyangiitis (Churg-Strauss): Report of 26 patients and review of the literature.
André R, Cottin V, Saraux JL, Blaison G, Bienvenu B, Cathebras P, Dhote R, Foucher A, Gil H, Lapoirie J, Launay D, Loustau V, Maurier F, Pertuiset E, Zénone T, Seebach J, Costedoat-Chalumeau N, Puéchal X, Mouthon L, Guillevin L, Terrier B; French Vasculitis Study Group (FVSG)
Autoimmun Rev 2017 Sep;16(9):963-969. Epub 2017 Jul 12 doi: 10.1016/j.autrev.2017.07.007. PMID: 28709761

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