2025/12/22 by Aleksandra Hus, Małgorzata Wisłowska · 1 voice
Medicine · #Asthma and respiratory diseases #Eosinophilic Disorders and Syndromes #Vasculitis and related conditions
paper · pdf · doi:10.5114/reum/214426
openalex created_date 2025/12/22 · openalex publication_date 2025/12/22 · openalex updated_date 2026/08/01
Introduction: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis defined by asthma, hypereosinophilia, and multiorgan involvement. Differentiating EGPA from other eosinophilic disorders is crucial because management differs substantially. The aim of the study is to summarize the pathogenesis, epidemiology, genetics, clinical manifestation, and treatment of EGPA and to provide a comparative differential diagnosis of eosinophilic disorders. Material and methods: Narrative review using the 2022 American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) classification criteria, 2024 EULAR recommendations, pivotal randomized trials, and major consensus statements; search strategy and selection criteria are detailed in the Introduction. Results: Eosinophilic granulomatosis with polyangiitis comprises 2 immunologic endotypes - ANCA-positive and ANCA-negative - with distinct organ tropism and therapeutic implications. The interleukin-5 (IL-5)-eosinophil axis is central, supporting anti-IL-5/IL-5R biologics in relapsing or refractory disease. A structured differential first excludes secondary hypereosinophilia (parasites, drugs, malignancies) and then addresses pulmonary "mimics". Conclusions: An algorithm combining exclusion of secondary causes with organ and endotype profiling enables targeted therapy and reduced glucocorticosteroids exposure.