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ANCA negative eosinophilic granulomatosis with polyangiitis (EGPA) presenting with left ventricular thrombus: An appreciation of distinct phenotypes and eosinophilic driven pathogenesis

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multisystem disorder, included in the spectrum of the antineutrophil cytoplasmic antibodies (ANCA) associated vasculitides. There are heterogeneous clinical features and a lack of consensus in standardized diagnostic criteria, with an un...

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Autores principales: Chaivannacoopt, Kai, Flanagan, Eliza
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley & Sons, Ltd 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9437554/
https://www.ncbi.nlm.nih.gov/pubmed/36090017
http://dx.doi.org/10.1002/rcr2.1033
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author Chaivannacoopt, Kai
Flanagan, Eliza
author_facet Chaivannacoopt, Kai
Flanagan, Eliza
author_sort Chaivannacoopt, Kai
collection PubMed
description Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multisystem disorder, included in the spectrum of the antineutrophil cytoplasmic antibodies (ANCA) associated vasculitides. There are heterogeneous clinical features and a lack of consensus in standardized diagnostic criteria, with an underappreciation of eosinophilic manifestations. There are now reported phenotypical differences between ANCA‐positive and negative EGPA, with myocardial involvement, lung infiltrates and gastrointestinal symptoms predominating in ANCA‐negative cases. We report a rare presentation of ANCA‐negative EGPA in a woman with respiratory, neurological and cardiac involvement, manifesting as a large left ventricular thrombus without significant cardiac dysfunction.
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spelling pubmed-94375542022-09-09 ANCA negative eosinophilic granulomatosis with polyangiitis (EGPA) presenting with left ventricular thrombus: An appreciation of distinct phenotypes and eosinophilic driven pathogenesis Chaivannacoopt, Kai Flanagan, Eliza Respirol Case Rep Case Reports Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multisystem disorder, included in the spectrum of the antineutrophil cytoplasmic antibodies (ANCA) associated vasculitides. There are heterogeneous clinical features and a lack of consensus in standardized diagnostic criteria, with an underappreciation of eosinophilic manifestations. There are now reported phenotypical differences between ANCA‐positive and negative EGPA, with myocardial involvement, lung infiltrates and gastrointestinal symptoms predominating in ANCA‐negative cases. We report a rare presentation of ANCA‐negative EGPA in a woman with respiratory, neurological and cardiac involvement, manifesting as a large left ventricular thrombus without significant cardiac dysfunction. John Wiley & Sons, Ltd 2022-09-02 /pmc/articles/PMC9437554/ /pubmed/36090017 http://dx.doi.org/10.1002/rcr2.1033 Text en © 2022 The Authors. Respirology Case Reports published by John Wiley & Sons Australia, Ltd on behalf of The Asian Pacific Society of Respirology. https://creativecommons.org/licenses/by/4.0/This is an open access article under the terms of the http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Reports
Chaivannacoopt, Kai
Flanagan, Eliza
ANCA negative eosinophilic granulomatosis with polyangiitis (EGPA) presenting with left ventricular thrombus: An appreciation of distinct phenotypes and eosinophilic driven pathogenesis
title ANCA negative eosinophilic granulomatosis with polyangiitis (EGPA) presenting with left ventricular thrombus: An appreciation of distinct phenotypes and eosinophilic driven pathogenesis
title_full ANCA negative eosinophilic granulomatosis with polyangiitis (EGPA) presenting with left ventricular thrombus: An appreciation of distinct phenotypes and eosinophilic driven pathogenesis
title_fullStr ANCA negative eosinophilic granulomatosis with polyangiitis (EGPA) presenting with left ventricular thrombus: An appreciation of distinct phenotypes and eosinophilic driven pathogenesis
title_full_unstemmed ANCA negative eosinophilic granulomatosis with polyangiitis (EGPA) presenting with left ventricular thrombus: An appreciation of distinct phenotypes and eosinophilic driven pathogenesis
title_short ANCA negative eosinophilic granulomatosis with polyangiitis (EGPA) presenting with left ventricular thrombus: An appreciation of distinct phenotypes and eosinophilic driven pathogenesis
title_sort anca negative eosinophilic granulomatosis with polyangiitis (egpa) presenting with left ventricular thrombus: an appreciation of distinct phenotypes and eosinophilic driven pathogenesis
topic Case Reports
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9437554/
https://www.ncbi.nlm.nih.gov/pubmed/36090017
http://dx.doi.org/10.1002/rcr2.1033
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