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Eosinophilic Granulomatosis With Polyangiitis: Dissecting the Pathophysiology

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 distinc...

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Autores principales: Fagni, Filippo, Bello, Federica, Emmi, Giacomo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7943470/
https://www.ncbi.nlm.nih.gov/pubmed/33718405
http://dx.doi.org/10.3389/fmed.2021.627776
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author Fagni, Filippo
Bello, Federica
Emmi, Giacomo
author_facet Fagni, Filippo
Bello, Federica
Emmi, Giacomo
author_sort Fagni, Filippo
collection PubMed
description 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.
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spelling pubmed-79434702021-03-11 Eosinophilic Granulomatosis With Polyangiitis: Dissecting the Pathophysiology Fagni, Filippo Bello, Federica Emmi, Giacomo Front Med (Lausanne) Medicine 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. Frontiers Media S.A. 2021-02-24 /pmc/articles/PMC7943470/ /pubmed/33718405 http://dx.doi.org/10.3389/fmed.2021.627776 Text en Copyright © 2021 Fagni, Bello and Emmi. http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Medicine
Fagni, Filippo
Bello, Federica
Emmi, Giacomo
Eosinophilic Granulomatosis With Polyangiitis: Dissecting the Pathophysiology
title Eosinophilic Granulomatosis With Polyangiitis: Dissecting the Pathophysiology
title_full Eosinophilic Granulomatosis With Polyangiitis: Dissecting the Pathophysiology
title_fullStr Eosinophilic Granulomatosis With Polyangiitis: Dissecting the Pathophysiology
title_full_unstemmed Eosinophilic Granulomatosis With Polyangiitis: Dissecting the Pathophysiology
title_short Eosinophilic Granulomatosis With Polyangiitis: Dissecting the Pathophysiology
title_sort eosinophilic granulomatosis with polyangiitis: dissecting the pathophysiology
topic Medicine
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7943470/
https://www.ncbi.nlm.nih.gov/pubmed/33718405
http://dx.doi.org/10.3389/fmed.2021.627776
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