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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Main Authors: Filippo Fagni, Federica Bello, Giacomo Emmi
Format: Article
Language:English
Published: Frontiers Media S.A. 2021-02-01
Series:Frontiers in Medicine
Subjects:
Online Access:https://www.frontiersin.org/articles/10.3389/fmed.2021.627776/full
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spelling doaj-9f3d3e85d10c4c23b94ab3b532adcd982021-02-24T04:37:10ZengFrontiers Media S.A.Frontiers in Medicine2296-858X2021-02-01810.3389/fmed.2021.627776627776Eosinophilic Granulomatosis With Polyangiitis: Dissecting the PathophysiologyFilippo FagniFederica BelloGiacomo EmmiEosinophilic 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.https://www.frontiersin.org/articles/10.3389/fmed.2021.627776/fullEosinophilic Granulomatosis with PolyangiitisChurg-Strauss syndromeeosinophilshypereosinophilic syndromesANCA-associated vasculitisneutrophils
collection DOAJ
language English
format Article
sources DOAJ
author Filippo Fagni
Federica Bello
Giacomo Emmi
spellingShingle Filippo Fagni
Federica Bello
Giacomo Emmi
Eosinophilic Granulomatosis With Polyangiitis: Dissecting the Pathophysiology
Frontiers in Medicine
Eosinophilic Granulomatosis with Polyangiitis
Churg-Strauss syndrome
eosinophils
hypereosinophilic syndromes
ANCA-associated vasculitis
neutrophils
author_facet Filippo Fagni
Federica Bello
Giacomo Emmi
author_sort Filippo Fagni
title Eosinophilic Granulomatosis With Polyangiitis: Dissecting the Pathophysiology
title_short 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_sort eosinophilic granulomatosis with polyangiitis: dissecting the pathophysiology
publisher Frontiers Media S.A.
series Frontiers in Medicine
issn 2296-858X
publishDate 2021-02-01
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.
topic Eosinophilic Granulomatosis with Polyangiitis
Churg-Strauss syndrome
eosinophils
hypereosinophilic syndromes
ANCA-associated vasculitis
neutrophils
url https://www.frontiersin.org/articles/10.3389/fmed.2021.627776/full
work_keys_str_mv AT filippofagni eosinophilicgranulomatosiswithpolyangiitisdissectingthepathophysiology
AT federicabello eosinophilicgranulomatosiswithpolyangiitisdissectingthepathophysiology
AT giacomoemmi eosinophilicgranulomatosiswithpolyangiitisdissectingthepathophysiology
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