Expert review and recommendations on the diagnosis and therapeutic management of eosinophilic granulomatosis with polyangiitis

专家对嗜酸性肉芽肿性血管炎的诊断和治疗管理进行审查和建议

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Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare and heterogeneous immune-mediated disorder in which eosinophilic inflammation, vasculitis, and airway disease coexist in highly variable combinations. In clinical practice, diagnosis remains challenging because no single biomarker reliably captures disease complexity, and therapeutic decisions must still be based on careful integration of clinical, laboratory, imaging, and histopathological findings. In recent years, EGPA management has evolved from a largely glucocorticoid-based approach toward a more phenotype-oriented strategy that distinguishes eosinophilic and vasculitic manifestations and incorporates targeted biologic therapies. This Perspective discusses current challenges and emerging opportunities in EGPA diagnosis and treatment, with emphasis on early recognition, multidisciplinary assessment, glucocorticoid-sparing strategies, and individualized long-term follow-up. Specific recommendations are provided on diagnostic orientation, phenotype-driven therapeutic selection, and the practical use of conventional immunosuppressants and anti-IL-5/IL-5R biologics according to disease severity and organ involvement. In addition, we propose a new treatment algorithm intended to support real-world decision-making and to facilitate a more consistent and structured approach to care. By combining expert recommendations with a forward-looking perspective on evolving therapeutic strategies, this article aims to contribute to safer, more effective, and more personalized management of patients with EGPA.

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