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• W2891606198 abstract "IntroductionEosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a rare, multi-system auto-immune vasculitic disorder characterized by allergic rhinitis, asthma, and prominent peripheral blood eosinophilia. Cardiac involvement is the primary contributor to mortality.Case ReportMr. W is a 53-year-old man with a history of asthma and environmental allergies presented to the emergency department with sudden onset of blurred vision in his left eye and evidence of optic neuritis. He endorsed a 2-week history of night sweats, arthralgias, anorexia, transient ankle edema, and a recent rash. His exam was notable for purpuric lesions in extremities and left visual field deficits. Laboratory data showed a WBC's of 36,000 cells/mm3 with 69% eosinophils, CRP of 63 mg/dl, ESR of 122 mm/min, IgE of 1,741 IU/ml, and troponin of 11 ng/ml. An Electrocardiogram showed low voltage and q waves in anteroseptal leads. An infectious work-up was negative.A brain MRI revealed acute punctate infarcts. A transthoracic echocardiogram (TTE) revealed a severely dilated left ventricle with an ejection fraction of 25%. Cardiac catheterization showed normal coronary arteries, elevated filling pressures, and normal cardiac output. Cardiac Magnetic Resonance (CMR) showed moderate to severe biventricular dysfunction, a moderate pericardial effusion, and patchy bi-ventricular subendocardial enhancement (Figure 1A). So he underwent endomyocardial biopsy, which showed increased perivascular eosinophils/eosinophilic degranulation and fibrin (Figure 1B). The differential diagnosis was EGPA, hypersensitivity myocarditis or a myeloproliferative variant of hypereosinophilic syndrome. A bone marrow biopsy was performed which showed hypereosinophilia (Figure 1C), without evidence of malignancy, confirming EGPA. He was treated with high dose steroids with a prolonged taper and cyclophosphamide, which rapidly resolved his eosinophilia. A repeat TTE 7 weeks later showed near normalization of cardiac function and resolution of pericardial fluid.ConclusionCardiac involvement in EGPA is up to 60% and early development of myocardial fibrosis. Prompt recognition via multi-modality imaging and endomyocardial biopsy and rapid treatment with high dose steroids or other immunotherapies, may prevent early progression to dilated or restrictive cardiomyopathy. Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a rare, multi-system auto-immune vasculitic disorder characterized by allergic rhinitis, asthma, and prominent peripheral blood eosinophilia. Cardiac involvement is the primary contributor to mortality. Mr. W is a 53-year-old man with a history of asthma and environmental allergies presented to the emergency department with sudden onset of blurred vision in his left eye and evidence of optic neuritis. He endorsed a 2-week history of night sweats, arthralgias, anorexia, transient ankle edema, and a recent rash. His exam was notable for purpuric lesions in extremities and left visual field deficits. Laboratory data showed a WBC's of 36,000 cells/mm3 with 69% eosinophils, CRP of 63 mg/dl, ESR of 122 mm/min, IgE of 1,741 IU/ml, and troponin of 11 ng/ml. An Electrocardiogram showed low voltage and q waves in anteroseptal leads. An infectious work-up was negative.A brain MRI revealed acute punctate infarcts. A transthoracic echocardiogram (TTE) revealed a severely dilated left ventricle with an ejection fraction of 25%. Cardiac catheterization showed normal coronary arteries, elevated filling pressures, and normal cardiac output. Cardiac Magnetic Resonance (CMR) showed moderate to severe biventricular dysfunction, a moderate pericardial effusion, and patchy bi-ventricular subendocardial enhancement (Figure 1A). So he underwent endomyocardial biopsy, which showed increased perivascular eosinophils/eosinophilic degranulation and fibrin (Figure 1B). The differential diagnosis was EGPA, hypersensitivity myocarditis or a myeloproliferative variant of hypereosinophilic syndrome. A bone marrow biopsy was performed which showed hypereosinophilia (Figure 1C), without evidence of malignancy, confirming EGPA. He was treated with high dose steroids with a prolonged taper and cyclophosphamide, which rapidly resolved his eosinophilia. A repeat TTE 7 weeks later showed near normalization of cardiac function and resolution of pericardial fluid. Cardiac involvement in EGPA is up to 60% and early development of myocardial fibrosis. Prompt recognition via multi-modality imaging and endomyocardial biopsy and rapid treatment with high dose steroids or other immunotherapies, may prevent early progression to dilated or restrictive cardiomyopathy." @default.
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• W2891606198 date "2018-08-01" @default.
• W2891606198 modified "2023-10-16" @default.
• W2891606198 title "Multi-modality Imaging in a Hypereosinophilic Syndrome with Cardiac Involvement" @default.
• W2891606198 doi "https://doi.org/10.1016/j.cardfail.2018.07.329" @default.
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