C. Hammann et al., Myocarditis of mixed connective tissue disease: Favourable outcome after intravenous pulsed cyclophosphamide, CLIN RHEUMA, 18(1), 1999, pp. 85-87
A 30-year-old woman with mixed connective tissue disease was admitted with
Wernicke's aphasia and progressive dyspnoea with chest pain. Multiple brain
infarcts on a computed tomographic scan were compatible with a thromboembo
lic aetiology. Echocardiography showed marked hypokinesia of the posterior
wall, biventricular dilatation and a decreased left-ventricle ejection frac
tion (40%). A diagnosis of myocarditis was made on myocardial biopsies disc
losing interstitial lymphocytic infiltrates and myocardial fibre necrosis.
A treatment with steroids and monthly pulsed cyclophosphamide was introduce
d. The heart function rapidly improved as assessed by a left-ventricle ejec
tion fraction of 55% and remained stable 17 months thereafter.