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Article Abstract

Objectives: To examine real-world clinical practices in the treatment of eosinophilic granulomatosis with polyangiitis (EGPA) across Japan and evaluate concordance with national treatment guidelines.

Methods: A cross-sectional, web-based survey was conducted between February 14 and 19, 2024. Physicians from rheumatology, pulmonology, and neurology departments who had treated EGPA within the past 24 months were invited to complete a 17-item questionnaire. The survey assessed actual prescribing behavior, future treatment preferences, responses to two clinical scenarios, and the frequency of guideline use.

Results: Among 220 respondents (110 rheumatologists, 55 neurologists, 55 pulmonologists), cyclophosphamide (77.3%) and mepolizumab (73.2%) were frequently used with glucocorticoids (GCs) for remission induction. GC monotherapy was the most commonly selected option overall (31.4%), but its use decreased to 14.8% for severe cases. GC + mepolizumab was the most frequently selected regimen for both maintenance and relapse. Responses to clinical scenarios revealed partial divergence from guideline-based recommendations, particularly regarding the limited use of immunosuppressants and the preferential use of mepolizumab.

Conclusion: This study identified a modest but clear evidence-practice gap in EGPA management in Japan. Broader dissemination of updated guidelines and enhanced interdisciplinary collaboration may help align clinical practice with evidence-based standards and improve patient care.

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http://dx.doi.org/10.1093/mr/roaf064DOI Listing

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