Eosinophilic granulomatosis with polyangiitis (EGPA) is an exceptionally rare systemic necrotizing vasculitis. The disease is clinically characterized by asthma with concomitant blood and tissue eosinophilia, often progressing to eosinophilic vasculitis. From the onset of asthma, there is usually a three to nine year delay of EGPA diagnosis. We report a case of this highly uncommon disease identified in an early stage.
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Background: Hypereosinophilic syndromes (HES) are a heterogenous group of eosinophilic disorders. To date, only retrospective studies of limited sample-size and/or follow-up duration are available.
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Pediatric eosinophilic granulomatosis with polyangiitis and intracardiac thrombus: A case report.
SAGE Open Med Case Rep
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Department of Pediatrics, Faculty of Medicine & Dentistry, University of Alberta, Edmonton, AB, Canada.
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic necrotizing vasculitis marked by eosinophilia and extravascular granulomas, predominantly affecting the respiratory tract. This report details a unique EGPA case in a 6-year-old girl with extensive cardiac involvement, featuring an atypical intracardiac mass suggestive of endomyocardial fibrosis and a concomitant thrombus. The clinical course unfolded in three phases: an initial prodrome with asthma; subsequent peripheral hypereosinophilia; and ultimately systemic vasculitis.
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