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PRACA PRZEGLĄDOWA
Eosinophilic granulomatosis with polyangiitis across the eosinophilic spectrum: from molecular mechanisms to practical differential diagnosis and targeted therapy
1
Department of Rheumatology, National Institute of Geriatric, Rheumatology and Rehabilitation, Warsaw, Poland
Data nadesłania: 03-10-2025
Data ostatniej rewizji: 14-11-2025
Data akceptacji: 18-11-2025
Data publikacji online: 22-12-2025
Autor do korespondencji
Małgorzata Wisłowska
National Institute Geriatric, Rheumatology and Rehabilitation, 1 Spartanska St., 02-637 Warsaw, Poland
National Institute Geriatric, Rheumatology and Rehabilitation, 1 Spartanska St., 02-637 Warsaw, Poland
SŁOWA KLUCZOWE
DZIEDZINY
STRESZCZENIE
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 – anti- neutrophil cytoplasmic antibody (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 glucocorticoid exposure.
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 – anti- neutrophil cytoplasmic antibody (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 glucocorticoid exposure.
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Copyright: © Narodowy Instytut Geriatrii, Reumatologii i Rehabilitacji w Warszawie. This is an Open Access journal, all articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International (CC BY-NC-SA 4.0) License (https://creativecommons.org/licenses/by-nc-sa/4.0/), allowing third parties to copy and redistribute the material in any medium or format and to remix, transform, and build upon the material, provided the original work is properly cited and states its license.
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| eISSN: | 2084-9834 |
| ISSN: | 0034-6233 |
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