PubMed2000
HISTORY: A 50-year-old woman was admitted because of marked dyspnoea at rest and signs of left heart failure with pulmonary oedema. 9 years ago, the diagnostic constellation of bronchial asthma, polyneuropathy, pericardial effusion and eosinophilia had indicated Churg-Strauss syndrome. Since then she had remained symptom-free under maintenance doses of azathioprine (for 2 years) and gradually reduced doses of steroids. INVESTIGATIONS: Chest X-ray showed signs of pulmonary congestion and cardiomegaly, echocardiography demonstrating enlargement of the left heart with marked impairment of ventricular function, and both revealed pericardial effusion. The electrocardiogram showed complete absence of R waves and ST elevation in leads V1-V5. Coronary angiography excluded coronary artery disease. Myocardial biopsy contained signs of active but no longer acute myocarditis with eosinophilic tissue infiltration and microgranulomas. White blood cell count was normal, but there was marked eosinophilia (39%). IgE was elevated (601 kIU/l). DIAGNOSIS, TREATMENT AND COURSE: In view of the good therapeutic effects 9 years ago, this relapse of Churg-Strauss syndrome with eosinophilic myocarditis was again treated with azathioprine and steroids. In addition, diuretics, digitalis and ACE-inhibitors successfully treated the heart failure. In the course of treatment the signs of inflammation, including the eosinophilia, regressed or became normal. CONCLUSION: After a 10-year remission without complication of a Churg-Strauss syndrome the onset of cardiac signs is the decisive long-term prognostic factor.