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.

Heart and Vessels
|January 1, 1997
PubMed

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.

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