| Summary: | Abstract 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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