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

A Case of Churg-Strauss Syndrome with Endomyocardial Fibrosis

• Kim HW
• Song YW
• Chang SH
• Ryu HH
• Song R
• Lee EB
• Son DW
• Kim YY

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.

Keyword

Churg-Strauss syndrome; Endomyocardial fibrosis

MeSH Terms

Cardiomyopathies
Churg-Strauss Syndrome
Dyspnea
Endomyocardial Fibrosis
Eosinophils
Humans
Magnetic Resonance Imaging
Myocarditis
Pericarditis

Figure

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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.

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  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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