Cited by 175Vasculitis and related conditionsOtitis Media and Relapsing PolychondritisEosinophilic Disorders and Syndromes

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Revisiting the systemic vasculitis in eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

Abstract

Objective To guide nosology and classification of patients with eosinophilic granulomatosis with polyangiitis (EGPA) based on phenotype and presence or absence of ANCA. Methods Organ manifestations and ANCA status were retrospectively analyzed based on the presence or not of predefined definite vasculitis features or surrogates of vasculitis in patients asthma, eosinophilia, and at least one systemic organ manifestation attributable to systemic disease. Results The study population included 157 patients (mean age 49.4±14.1), with a follow-up of 7.4±6.4years. Patients with ANCA (31%) more frequently had weight loss, myalgias, arthralgias, biopsy-proven vasculitis, glomerulonephritis on biopsy, hematuria, leukocytoclastic capillaritis and/or eosinophilic infiltration of arterial wall on biopsy, and other renal disease. A total of 41% of patients had definite vasculitis manifestations (37%) or strong surrogates of vasculitis (4%), of whom only 53% had ANCA. Mononeuritis multiplex was associated with systemic vasculitis (p=0.005) and with the presence of ANCA (p Conclusion We suggest a revised nomenclature and definition for EGPA and a new proposed entity referred to as hypereosinophilic asthma with systemic (non vasculitic) manifestations.

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Crossref · 10.1016/j.autrev.2016.09.018

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  • Approximately 20% to 50% of patients with hypereosinophilic syndrome exhibit inflammation or fibrosis in the heart.

    “Approximately 20% to 50% of patients with hypereosinophilic syndrome exhibit inflammation or fibrosis in the heart”

    From Autoimmune Myocarditis, Old Dogs and New Tricks. (2024) · cites this paper as [203]

  • Eosinophil-related autoimmune disorders are strongly linked to cardiovascular manifestations including myocarditis.

    “eosinophil-related autoimmune disorders are strongly linked to cardiovascular manifestations including myocarditis.”

    From Autoimmune Myocarditis, Old Dogs and New Tricks. (2024) · cites this paper as [203]

  • Over 40% of patients with eosinophilic granulomatosis with polyangiitis experience cardiovascular complications.

    “over 40% of patients with eosinophilic granulomatosis with polyangiitis, also known as ChurgStrauss, experience cardiovascular complications.”

    From Autoimmune Myocarditis, Old Dogs and New Tricks. (2024) · cites this paper as [203]

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Research works printed but not linked: 20

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  2. Churg, 1951, Allergic granulomatosis, allergic angiitis, and periarteritis nodosa, Am J Pathol, 27, 277
  3. Churg, 1995, Formes frustes of Churg-Strauss syndrome, Chest, 108, 320, 10.1378/chest.108.2.320 · DOI
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  16. Watts, 2007, Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies, Ann Rheum Dis, 66, 222, 10.1136/ard.2006.054593 · DOI
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Metadata

Authors
Cottin, V., Bel, E., Bottero, P., Dalhoff, K., Humbert, M., Lazor, R., Sinico, R. A., Sivasothy, P., Wechsler, M. E., Groh, M., Marchand-Adam, S., Khouatra, C., Wallaert, B., Taillé, C., Delaval, P., Cadranel, J., Bonniaud, P., Prévot, G., Hirschi, S., Gondouin, A., Dunogué, B., Chatté, G., Briault, C., Pagnoux, C., Jayne, D., Guillevin, L., & Cordier, J.-F.
Affiliations
  • Lyon 1 Université (FR)
  • Amsterdam UMC Location University of Amsterdam (NL)
  • Azienda Ospedaliera Ospedale Civile di Legnano (IT)
  • University Hospital Schleswig-Holstein (DE)
  • Bicêtre Hospital (FR)
  • Centre Hospitalier Universitaire Vaudois (CH)
  • University of Milano-Bicocca (IT)
  • Cambridge University Hospitals NHS Foundation Trust (GB)
  • National Jewish Health (US)
  • Hôpital Cochin (FR)
  • Université de Tours (FR)
  • Inserm (FR)
  • Centre Hospitalier Universitaire de Rennes (FR)
  • Assistance Publique – Hôpitaux de Paris (FR)
  • Hôpitaux Universitaires de Strasbourg (FR)
  • Centre Hospitalier Universitaire de Besançon (FR)
  • Centre Hospitalier Universitaire de Grenoble (FR)
  • University of Cambridge (GB)
Corresponding author
Cottin, Vincent
Venue
Autoimmunity Reviews
Publisher
Elsevier BV
Published
2017-01
Volume
16
Issue
1
Pages
1-9
ISSN
1568-9972 (ISSN-L)
OpenAlex
W2526725029
Cited by
175
References
0
Citations by year
2026: 13 · 2025: 11 · 2024: 12 · 2023: 18 · 2022: 17 · 2021: 17 · 2020: 25 · 2019: 23 · 2018: 19 · 2017: 18
FWCI
18.18
Type
journal article
Language
en
Open access
closed
Locations
  • Autoimmunity Reviews (publishedVersion)
  • PubMed (publishedVersion)
  • Pure Amsterdam UMC (publishedVersion)
  • Data Archiving and Networked Services (DANS) (submittedVersion)
  • HAL (submittedVersion)
  • BOA (University of Milano-Bicocca) (submittedVersion)
  • IRIS (submittedVersion)
  • SERVAL (Université de Lausanne) (submittedVersion)
Topics
  • Health Sciences › Medicine › Rheumatology › Vasculitis and related conditions
  • Health Sciences › Medicine › Rheumatology › Otitis Media and Relapsing Polychondritis
  • Health Sciences › Medicine › Rheumatology › Eosinophilic Disorders and Syndromes
Keywords
eosinophilic granulomatosis with polyangiitis, ANCA, systemic vasculitis, mononeuritis multiplex, eosinophilia, asthma, disease classification, classification criteria, kidney disease, glomerulonephritis, myocarditis
MeSH terms
Adult, Asthma, Female, Humans, Immunosuppressive Agents, Male, Middle Aged, Prognosis, Retrospective Studies, Churg-Strauss Syndrome, Treatment Outcome

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