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(Circulation. 1995;91:2851.)
© 1995 American Heart Association, Inc.

Articles

Idiopathic Hypereosinophilic Vasculitis

M. Jakob, MD; M. Ritter, MD; J. Peter, MD; E. Walter, MD; W. Kiowski, MD; A. Fontana, MD; R. Jenni, MD

From the Division of Cardiology, Department of Internal Medicine, University Hospital, Zurich.

Correspondence to Markus Jakob, MD, Department of Internal Medicine, University Hospital, CH-8091, Zurich, Switzerland.

Key Words: Cardiovascular Images • vasculitis • coronary aneurysm

	Introduction

 
This 36-year-old male patient, at the age of 22 years, had had painless scalp swelling due to multiple aneurysms of the left temporal artery. Biopsy revealed an eosinophilic infiltration of all three wall layers with fresh fibrinoid necrosis beside old fibrous scars. An idiopathic blood eosinophilia was noted; no treatment was initiated.

In the present case, the 36-year-old patient presented with dyspnea at rest. The ECG showed evidence of prior inferior infarction. Transesophageal echocardiography found left ventricular (LV) enlargement with biplanar ejection fraction of 20% as well as dilatation of the right ventricle and the atria. LV muscle mass was increased (245 g/m²; normal, 134 g/m²). The epicardial coronary arteries showed grotesque aneurysmal dilatation. Intravascular thrombosis of the left and right main stem with reticular revascularization could be clearly identified (Fig 1). Ultrasonic myocardial texture was normal, without signs of focal granulomatosis, whereas endocardial echo density was enhanced, indicating endocardial fibrosis. Right ventricular endomyocardial biopsy yielded interstitial scars in the myocardium. However, there was no infiltration with eosinophilic leukocytes. Angiography visualized proximal obliteration of the left anterior descending and right coronary arteries. The left circumflex artery was subtotally obliterated (Figs 2 and 3). Aortography revealed a normal abdominal aorta and intestinal arteries but a stenosis of the arteria hepatica propria. The small vessels of the inferior spleen were not patent. Laboratory tests found hypereosinophilia (48%) and elevated IgE. There was no evidence of parasitosis, atopic disease (eg, asthma), or neoplastic or connective tissue disease.

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