U. Koehler et al., Eosinophilic myocarditis as part of Churg-Strauss syndrome, a rare cause of left heart failure with pulmonary oedema, DEUT MED WO, 125(44), 2000, pp. 1323-1327
Citations number
17
Categorie Soggetti
General & Internal Medicine","Medical Research General Topics
History: A 50-year-old woman was admitted because of marked dyspnoea at res
t and signs of left heart failure with pulmonary oedema. 9 years ago, the d
iagnostic constellation of bronchial asthma, polyneuropathy, pericardial ef
fusion and eosinophilia had indicated Churg-Strauss syndrome. Since then sh
e 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 cardio
megaly, echocardiography demonstrating enlargement of the left heart with m
arked impairment of ventricular function, and both revealed pericardial eff
usion. The electrocardiogram showed complete absence of R waves and ST elev
ation in leads V1-V5. Coronary angiography excluded coronary artery disease
. Myocardial biopsy contained signs of active but no longer acute myocardit
is with eosinophilic tissue infiltration and microgranulomas. White blood c
ell count was normal, but there was marked eosinophilia (39%). IgE was elev
ated (601 klU/l).
Diagnosis, treatment and course: In view of the good therapeutic effects 9
years ago, this relapse of Churg-Strauss syndrome with eosinophilic myocard
itis was again treated with azathioprine and steroids. In addition, diureti
cs, digitalis and ACE-inhibitors successfully treated the heart failure. In
the course of treatment the signs of inflammation, including the eosinophi
lia, regressed or became normal.
Conclusion: After a 10-year remission without complication of a Churg-Strau
ss syndrome the onset of cardiac signs is the decisive long-term prognostic
factor.