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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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Detalles Bibliográficos
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
Descripción
Sumario: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.