A Case of Churg-Strauss Syndrome with Endomyocardial Fibrosis

Hye Won Kim; Yeong Wook Song; Sung Hae Chang; Han Hee Ryu; Ran Song; Eun Bong Lee; Dae Won Son; You Young Kim

doi:10.4078/jkra.2010.17.3.306

Journal List > J Korean Rheum Assoc > v.17(3) > 1003743

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Kim, Song, Chang, Ryu, Song, Lee, Son, and Kim: A Case of Churg-Strauss Syndrome with Endomyocardial Fibrosis

Original Article

J Korean Rheum Assoc 2010;17(3):306-310.

Published online: 23 September 2010

DOI: https://doi.org/10.4078/jkra.2010.17.3.306

A Case of Churg-Strauss Syndrome with Endomyocardial Fibrosis

Hye Won Kim, Yeong Wook Song, Sung Hae Chang, Han Hee Ryu, Ran Song, Eun Bong Lee, Dae Won Son, You Young Kim

¹Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea

Received 3 October 20109 May 2010 Accepted 20 May 2010

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Cardiac involvement is an important cause of mortality in patients with Churg-Strauss syndrome. The typical cardiac presentation of Churg-Strauss syndrome includes pericarditis, myocarditis, and cardiomyopathy. Endomyocardial fibrosis has rarely been described in patients with Churg-Strauss syndrome. We experienced a patient with Churg-Strauss syndrome who exhibited exertional dyspnea and endomyocardial fibrosis visualized as delayed enhancement on cardiac magnetic resonance imaging (MRI). After glucocorticoid treatment, the patient's symptom resolved, and the eosinophil count decreased to normal. Nine months later, the delayed-enhanced lesion on the cardiac MRI nearly disappeared. Here, we report a case of endomyocardial fibrosis in a patient with Churg-Strauss syndrome with a literature review.

Keywords: Churg-Strauss syndrome, Endomyocardial fibrosis

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Fig. 1.

Transthoracic echocardiography shows echogenic mass in the right ventricular apex (arrow) with right ventricularcavity obliteration and a moderate amount of pericardial effusion (arrowhead). The left ventricular cavity size and systolic function were normal (ejection fraction: 64%). (A) Parasternal long axis view (B) Four chambered view.

Fig. 2.

Magnetic resonance imaging (MRI). (A) Diffuse wall thickening and endomyocardial linear high signal intensity of the right ventricle (white arrows) with constricted ventricular cavity on T2-weighted MRI. (B) Delayed enhancement MRI shows endomyocardial enhancement of the right ventricle (arrowhead) and tiny low signal intensities (thrombus) (arrow) in the right ventricular cavity. (C) A 1-month follow-up image shows no significant interval change in the right ventricular apical obliteration and small right ventricular cavity on T2-weighted MRI. (D) A 9-month follow-up image shows a nearly disappeared delayed enhancement of the right ventricle (arrowhead) and absent thrombi in the right ventricular cavity (arrow).

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