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Evolution to dilated cardiomyopathy from acute eosinophilic pancarditis in Churg-Strauss syndrome
F Terasaki1, T Hayashi, Y Hirota
1Department of Internal Medicine, Osaka Medical College, Japan.
Insights
Churg-Strauss syndrome can cause severe heart damage, progressing to dilated cardiomyopathy (DCM). Histopathology reveals eosinophilic endomyocarditis, fibrosis, and thrombi, suggesting DCM may result from hypereosinophilic heart disease.
Area of Science:
- Cardiology
- Pathology
- Rheumatology
Background:
- Churg-Strauss syndrome, also known as eosinophilic granulomatosis with polyangiitis, is a rare systemic vasculitis characterized by asthma, eosinophilia, and multi-system involvement.
- Cardiac involvement is a serious complication of Churg-Strauss syndrome, often leading to poor prognosis.
Observation:
- This case study details a patient with Churg-Strauss syndrome who experienced severe acute cardiac manifestations.
- The patient's condition evolved over 10 months, showing progression from acute eosinophilic endomyocarditis to dilated cardiomyopathy (DCM).
Findings:
- Sequential endomyocardial biopsies demonstrated progressive histopathological changes: acute eosinophilic endomyocarditis, followed by interstitial fibrosis, and finally endocardial thickening with mural thrombi.
- Despite steroid therapy reducing inflammation and eosinophil markers, left ventricular dilatation and reduced contractility persisted, indicating disease progression.
Implications:
- These findings suggest that a subset of dilated cardiomyopathy cases may not be idiopathic but rather a consequence of hypereosinophilic heart disease.
- This highlights the importance of considering Churg-Strauss syndrome in the differential diagnosis of DCM, especially in patients with a history of asthma and eosinophilia.
Abstract:
We describe the clinical manifestations of a patient with Churg-Strauss syndrome who presented with severe acute cardiac involvement and whose disease evolved to dilated cardiomyopathy (DCM), with special reference to the histopathological findings. Endomyocardial biopsies, conducted sequentially, three times within 10 months, revealed severe eosinophilic endomyocarditis in the acute phase, interstitial fibrosis in the subacute phase, and endocardial thickening with mural thrombi, at 10 months. Although acute inflammation associated with elevation of eosinophil granule proteins subsided with steroid therapy, left ventricular dilatation with reduced contractility progressed. A subgroup of DCM is not considered to be idiopathic but, rather, an aftereffect of hypereosinophilic heart disease.
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