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

•Systematic literature review & expert consensus to identify red flags for raising EGPA suspicion.•EGPA diagnosis is tricky due to its heterogeneity and its overlap with eosinophilic disorders.•Creating an evidence-based checklist with red flags to initiate EGPA diagnosis process. Eosinophilic g...

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Bibliographic Details
Published in:European journal of internal medicine 2024-06
Main Authors: Solans-Laqué, R., Rúa-Figueroa, I., Blanco Aparicio, M., García Moguel, I., Blanco, R., Pérez Grimaldi, F., Noblejas Mozo, A., Labrador Horrillo, M., Álvaro-Gracia, J.M., Domingo Ribas, C., Espigol-Frigolé, G., Sánchez-Toril López, F., Ortiz Sanjuán, F.M., Arismendi, E., Cid, M.C.
Format: Article
Language:English
Subjects:
Asthma
Checklist
Churg-Strauss
Clinical suspicion
EGPA
Eosinophilia
Eosinophilic granulomatosis with polyangiitis
Recommendations
Vasculitis
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Summary:•Systematic literature review & expert consensus to identify red flags for raising EGPA suspicion.•EGPA diagnosis is tricky due to its heterogeneity and its overlap with eosinophilic disorders.•Creating an evidence-based checklist with red flags to initiate EGPA diagnosis process. 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. 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. 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. 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/µL if untreated and >500 cells/µL 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. 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.
ISSN:0953-6205
1879-0828
1879-0828
DOI:10.1016/j.ejim.2024.06.008