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Necrotizing Myocardial Vasculitis in Churg-Strauss Syndrome : Clinicohistologic Evaluation of Steroids and Immunosuppressive Therapy

Andrea Frustaci; Nicola Gentiloni; Cristina Chimenti; Luigi Natale; Giovanni Gasbarrini; Attilio Maseri
Author and Funding Information

Affiliations: From the Department of Cardiology, Università Cattolica del Sacro Cuore, Rome, Italy,  From the Department of Internal Medicine, Università Cattolica del Sacro Cuore, Rome, Italy,  From the Department of Radiology, Università Cattolica del Sacro Cuore, Rome, Italy

Andrea Frustaci, MD, FCCP, Istituto di Cardiologia Università Cattolica del Sacro Cuore, Largo Gemelli 8, 00168 Rome, Italy


1998 by the American College of Chest Physicians


Chest. 1998;114(5):1484-1489. doi:10.1378/chest.114.5.1484
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Abstract

Treatment of cardiac dysfunction associated with Churg-Strauss syndrome (CSS) is empiric since the histologic findings provided by endomyocardial biopsy are rare and often nondiagnostic. Myocardial necrotizing vasculitis presenting as restrictive cardiomyopathy has not been reported before. A case of CSS, presenting with fever and progressive heart failure due to pericarditis, eosinophilic endomyocarditis, and myocardial necrotizing vasculitis, is reported. Cardiac involvement assessed by noninvasive (cardiac two-dimensional echocardiogram and nuclear magnetic resonance [NMR] imaging) and invasive (cardiac catheterization, angiography, and biopsy) studies showed a moderate degree of pericardial effusion and left ventricular (LV) dysfunction (ejection fraction 0.40), severe diastolic dysfunction (increased right and LV filling pressure with a dip and plateau pattern) and a severe reduction of cardiac index (1.6 L/min/m2). Histologic characteristics showed marked eosinophilic infiltration of the endocardium and myocardium with myocitolysis and fibrinoid necrosis of arterioles, venules, and capillaries. Combination therapy of steroids and cyclophosphamide resulted in both a clinical (regression of pericardial effusion, normalization of systolic and diastolic dysfunction, and increase of cardiac index to 2.8 L/min/m2) and histologic (sequential endomyocardial biopsies at 1, 3, and 6 months of follow-up) resolution of cardiac involvement. No recurrences were registered at 12-month follow-up with the patient receiving a maintenance drug regimen.


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