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Abstract
Eosinophilic granulomatosis with polyangiitis (EGPA, also known as the Churg-Strauss syndrome) is a disorder characterized by asthma, peripheral eosinophilia and systemic vasculitis. It rarely occurs in children, so that physicians may frequently mistake it for a simple uncontrolled asthma. Since a subsequent cardiac involvement is critical for the prognosis, it is important to suspect EGPA in children with severe, uncontrolled asthma. The cardiac manifestations in EGPA are variable from asymptomatic electrocardiogram abnormalities to pericarditis with pericardial effusion, myocarditis with cardiomyopathy, heart failure, and sudden cardiac death. Although delayed treatment may lead to fatal cardiac complications in EGPA, adequate immune suppression can reverse cardiac impairment. We report a 14-year-old girl with persistent asthma refractory to steroids who was eventually diagnosed with an anti-neu-trophil cytoplasmic antibody-negative EGPA.
References
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Fig. 1.
Chest computed tomography reveals interlobular septal thickening and large amount of bilateral pleural effusion with passive atelectasis.
Fig. 2.
A) Pretreatment echocardiography shows severe left ventricular dysfunction with ejection fraction of 19.1%. (B) Pretreatment cardiac magnetic resonance imaging reveals that late gadolinium enhancement at midinferior and inferolateral wall of left ventricle. (C) Duodenal biopsy shows mild eosinophilic infiltration (H&E, ×200). (D) Posttreatment echocardiography shows improved left ventricular ejection fraction of 45% without mitral regurgitation.
Fig. 3.
Time changes in the blood eosinophilia, ejection fraction, B-type natriuretic peptide and degree of dyspnea during treatment. Green box indicates the blood eosinophil percent, solid line with square stands for the ejection fraction, and the dotted line with empty circle represents the B-type natriuretic peptide. Dyspnea scale is described using a modified Medical Research Council dyspnea scale.
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