Red flags for clinical suspicion of eosinophilic granulomatosis with polyangiitis (EGPA)

May 13, 2025

Publications

>

Article

No

Publicated to: European Journal of Internal Medicine. 128 45-52 - 2024-10-01 128(), DOI: 10.1016/j.ejim.2024.06.008

Authors:

Solans-Laque, R; Rua-Figueroa, I; Aparicio, M Blanco; Moguel, I Garcia; Blanco, R; Grimaldi, F Perez; Mozo, A Noblejas; Horrillo, M Labrador; Alvaro-Gracia, J M; Ribas, C Domingo; Espigol-Frigole, G; Lopez, F Sanchez-Toril; Sanjuan, F M Ortiz; Arismendi, E; Cid, M C

[+]

Affiliations

CIBERES, Barcelona, Spain - Author

H Univ Jerez, Pneumol Dept, Cadiz, Spain - Author

Hosp Arnau Vilanova, Pneumol Dept, Valencia, Spain - Author

Hosp Univ & Politecn La Fe, Rheumatol Dept, Valencia, Spain - Author

Hosp Univ 12 Octubre, Allergy Dept, Madrid, Spain - Author

Hosp Univ A Coruna, Pneumol Dept, La Coruna, Spain - Author

Hosp Univ Gran Canaria Dr Negrin, Rheumatol Dept, Las Palmas Gran Canaria, Spain - Author

Hosp Univ La Paz, Internal Med Dept, Madrid, Spain - Author

Hosp Univ Marques Valdecilla, Rheumatol Dept, Immunopathol Grp, IDIVAL, Santander, Spain - Author

Inst Invest Sanitaria Hosp 12 Octubre imas12, Madrid, Spain - Author

Univ Autonoma Barcelona UAB, Pneumol Dept, Corporacio Sanitaria Parc Tauli, Barcelona, Spain - Author

Univ Autonoma Barcelona, H Univ Vall Hebron, Internal Med Dept, Barcelona, Spain - Author

Univ Barcelona, Hosp Clin Barcelona, Pneumol Dept, Barcelona, Spain - Author

Univ Barcelona, Hosp Clin, Inst Invest Biomed August Pi i Sunyer FRCB IDIBAPS, Autoimmune Dis Dept, Barcelona, Spain - Author

Abstract

Background: Eosinophilic granulomatosis with polyangiitis (EGPA), is a rare ANCA-associated systemic vasculitis. Its overlapping features with other vasculitic or eosinophilic diseases, and the wide and heterogeneous range of clinical manifestations, often result in a delay to diagnosis. Objective: To identify red flags that raise a suspicion of EGPA to prompt diagnostic testing and to present an evidence-based clinical checklist tool for use in routine clinical practice. Methods: Systematic literature review and expert consensus to identify a list of red flags based on clinical judgement. GRADE applied to generate a strength of recommendation for each red flag and to develop a checklist tool. Results: 86 studies were included. 40 red flags were identified as relevant to raise a suspicion of EGPA and assessed by the experts as being clinically significant. Experts agreed that a diagnosis of EGPA should be considered in a patient aged >= 6 years with a blood eosinophil level >1000 cells/mu L if untreated and >500 cells/ mu L if previously treated with any medication likely to have altered the blood eosinophil count. The presence of asthma and/or nasal polyposis should reinforce a suspicion of EGPA. Red flags of asthma, lung infiltrates, pericarditis, cardiomyopathy, polyneuropathy, biopsy with inflammatory eosinophilic infiltrates, palpable purpura, digital ischaemia and ANCA positivity, usually anti-myeloperoxidase, among others, were identified. Conclusion: The identification of a comprehensive set of red flags could be used to raise a suspicion of EGPA in patients with eosinophilia, providing clinicians with an evidence-based checklist tool that can be integrated into their practice.

[+]

Keywords

2022 american-collegeAllianceAntibodies, antineutrophil cytoplasmicAntineutrophil cytoplasmic antibodiesArticleAssociationsAsthmaBenralizumabBiological markerBloodCardiomyopathyChecklistChurg strauss syndromeChurg-straussChurg-strauss syndromeChurg-strauss-syndromeClinical assessmentClinical suspicionDiagnosisDigital ischemiaEgpaEosinophilEosinophil countEosinophiliaEosinophilic granulomatosis with polyangiitisEosinophilsEye diseaseGastrointestinal diseaseGranulomatosis with polyangiitisHead and neck diseaseHeart diseaseHistopathologyHumanHumansIschemiaKidney diseaseLeukocyte countLung biopsyLung infiltrateMusculoskeletal diseaseMyeloperoxidaseNasal polypsNeurologic diseaseNeutrophil cytoplasmic antibodyPericarditisPolyneuropathyPulmonary eosinophiliaPurpuraQualityRecommendationsRespiratory tract diseaseRheumatology classification criteriaSafetSinonasal polypSkin diseaseSparing treatment optionVasculitiVasculitisWegener granulomatosis

Quality index

Bibliometric impact. Analysis of the contribution and dissemination channel

The work has been published in the journal European Journal of Internal Medicine due to its progression and the good impact it has achieved in recent years, according to the agency WoS (JCR), it has become a reference in its field. In the year of publication of the work, 2024 there are still no calculated indicators, but in 2023, it was in position 29/332, thus managing to position itself as a Q1 (Primer Cuartil), in the category Medicine, General & Internal. Notably, the journal is positioned above the 90th percentile.

Independientemente del impacto esperado determinado por el canal de difusión, es importante destacar el impacto real observado de la propia aportación.

Según las diferentes agencias de indexación, el número de citas acumuladas por esta publicación hasta la fecha 2026-04-05:

WoS: 11
Scopus: 9
Europe PMC: 7

[+]

Leadership analysis of institutional authors

There is a significant leadership presence as some of the institution’s authors appear as the first or last signer, detailed as follows: Last Author (Cid Xutgla, Maria Cinta).

the author responsible for correspondence tasks has been Cid Xutgla, Maria Cinta.

[+]

Awards linked to the item

The present work was funded by GSK. Authors were in full editorial control of the content and conclusions and accepted full responsibility for the final approval of the manuscript. The decision to submit for publication was taken solely by the authors and the sponsor did not place any restrictions on access to the data or on the statements made in the manuscript. GSK funded Adelphi Targis who undertook the literature research and provided medical writing support under the direction and guidance of the expert authors. The authors were solely responsible for critical assessment and interpretation of results of the literature review and approved the final content.

[+]

Indexed in

Citations

Altmetrics

Analysis of institutional authors

Share