Eosinophilic Granulomatosis With Polyangiitis: Dissecting the Pathophysiology.
ANCA-associated vasculitis
Churg-Strauss syndrome
EGPA classification
Eosinophilic Granulomatosis with Polyangiitis
eosinophils
hypereosinophilic syndromes
myeloperoxidase
neutrophils
Journal
Frontiers in medicine
ISSN: 2296-858X
Titre abrégé: Front Med (Lausanne)
Pays: Switzerland
ID NLM: 101648047
Informations de publication
Date de publication:
2021
2021
Historique:
received:
10
11
2020
accepted:
18
01
2021
entrez:
15
3
2021
pubmed:
16
3
2021
medline:
16
3
2021
Statut:
epublish
Résumé
Eosinophilic Granulomatosis with Polyangiitis (EGPA) is a rare multisystemic disease classified both amongst hypereosinophilic disorders and ANCA-associated vasculitis. Vessel inflammation and eosinophilic proliferation are the hallmarks of the disease and main effectors of organ damage. Two distinct disease phenotypes have classically been described according to ANCA-status: the ANCA-negative subset with eosinophil-driven manifestation and the ANCA-positive one with vasculitic manifestations. An analogous dichotomization has also been backed by histological findings and a distinct genetic background. EGPA is typically consider a Th2-mediated disease and blood and tissue eosinophilia represent the cornerstone of diagnosis. Besides, ANCA are known for inducing endothelial injury and vascular inflammation by activating the circulating neutrophils. Thus, the pathogenesis of EGPA seems to be mediated by two coexisting mechanisms. However, the verbatim application of this strict dualism cannot always be translated into routine clinical practice. In the present review we describe the current knowledge on the eosinophilic and ANCA-mediated aspects of EGPA pathogenesis. Finally, we review the rationale of the currently proposed EGPA dichotomy and future research perspectives.
Identifiants
pubmed: 33718405
doi: 10.3389/fmed.2021.627776
pmc: PMC7943470
doi:
Types de publication
Journal Article
Review
Langues
eng
Pagination
627776Informations de copyright
Copyright © 2021 Fagni, Bello and Emmi.
Déclaration de conflit d'intérêts
The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
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