U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

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; Disorder, EGPA; Disorders, EGPA; EGPA Disorder; EGPA Disorders; Eosinophilic Granulomatosis with Polyangiitis; 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.
 
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

Emmi G, Bettiol A, Gelain E, Bajema IM, Berti A, Burns S, Cid MC, Cohen Tervaert JW, Cottin V, Durante E, Holle JU, Mahr AD, Del Pero MM, Marvisi C, Mills J, Moiseev S, Moosig F, Mukhtyar C, Neumann T, Olivotto I, Salvarani C, Seeliger B, Sinico RA, Taillé C, Terrier B, Venhoff N, Bertsias G, Guillevin L, Jayne DRW, Vaglio A
Nat Rev Rheumatol 2023 Jun;19(6):378-393. Epub 2023 May 9 doi: 10.1038/s41584-023-00958-w. PMID: 37161084
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
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

Recent clinical studies

Etiology

White J, Dubey S
Autoimmun Rev 2023 Jan;22(1):103219. Epub 2022 Oct 22 doi: 10.1016/j.autrev.2022.103219. PMID: 36283646
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
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
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
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

Romero Gómez C, Hernández Negrín H, Ayala Gutiérrez MDM
Med Clin (Barc) 2023 Apr 6;160(7):310-317. Epub 2023 Feb 9 doi: 10.1016/j.medcli.2023.01.003. PMID: 36774291
White J, Dubey S
Autoimmun Rev 2023 Jan;22(1):103219. Epub 2022 Oct 22 doi: 10.1016/j.autrev.2022.103219. PMID: 36283646
Villa-Forte A
Postgrad Med 2023 Jan;135(sup1):52-60. Epub 2022 Dec 5 doi: 10.1080/00325481.2022.2134624. PMID: 36259957
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
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

Therapy

White J, Dubey S
Autoimmun Rev 2023 Jan;22(1):103219. Epub 2022 Oct 22 doi: 10.1016/j.autrev.2022.103219. PMID: 36283646
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
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
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
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

White J, Dubey S
Autoimmun Rev 2023 Jan;22(1):103219. Epub 2022 Oct 22 doi: 10.1016/j.autrev.2022.103219. PMID: 36283646
Villa-Forte A
Postgrad Med 2023 Jan;135(sup1):52-60. Epub 2022 Dec 5 doi: 10.1080/00325481.2022.2134624. PMID: 36259957
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
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
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

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
Bettiol A, Urban ML, Dagna L, Cottin V, Franceschini F, Del Giacco S, Schiavon F, Neumann T, Lopalco G, Novikov P, Baldini C, Lombardi C, Berti A, Alberici F, Folci M, Negrini S, Sinico RA, Quartuccio L, Lunardi C, Parronchi P, Moosig F, Espígol-Frigolé G, Schroeder J, Kernder AL, Monti S, Silvagni E, Crimi C, Cinetto F, Fraticelli P, Roccatello D, Vacca A, Mohammad AJ, Hellmich B, Samson M, Bargagli E, Cohen Tervaert JW, Ribi C, Fiori D, Bello F, Fagni F, Moroni L, Ramirez GA, Nasser M, Marvisi C, Toniati P, Firinu D, Padoan R, Egan A, Seeliger B, Iannone F, Salvarani C, Jayne D, Prisco D, Vaglio A, Emmi G; European EGPA Study Group
Arthritis Rheumatol 2022 Feb;74(2):295-306. Epub 2021 Dec 30 doi: 10.1002/art.41943. PMID: 34347947Free PMC Article
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
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
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

Sanchez-Alamo B, Schirmer JH, Hellmich B, Jayne D, Monti S, Tomasson G, Luqmani RA
RMD Open 2023 Jun;9(2) doi: 10.1136/rmdopen-2023-003083. PMID: 37349121Free PMC Article
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
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
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 Care Res (Hoboken) 2021 Aug;73(8):1088-1105. Epub 2021 Jul 8 doi: 10.1002/acr.24634. PMID: 34235880
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

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...