Atteinte myocardique primitive de la sclérodermie systémique

Abstract

Systemic sclerosis (SSc) is a connective tissue disease characterised by diffuse vascular lesions and fibrosis. Primary myocardial involvement is common in SSc and, when clinically evident, appears as a poor prognostic factor. An increasing body of evidence suggests that myocardial involvement is due, at least in part, to microcirculation impairment with abnormal vasoreactivity, with or without associated structural abnormalities of the small coronary arteries or arterioles. Using conventional methods, myocardial peffusion impairment, systolic and diastolic left ventricular dysfunction and right ventricular dysfunction have been shown. Recently, tissue Doppler echocardiography and magnetic resonance imaging have confirmed these results. Vasodilators, such as calcium channel blockers and angiotensin converting enzyme inhibitors, improve both myocardial perfusion and function abnormalities. Using these latter recent techniques, bosentan also showed its ability to improve myocardial parameters in SSc patients without pulmonary arterial hypertension.