Micro- and Macrovascular Treatment Targets in Scleroderma Heart Disease
George D. Kitas.
Cardiac involvement in systemic sclerosis (SSc) is a frequent visceral complication that considerably affects the prognosis of
the disease. The pathophysiologic hallmark is myocardial fibrosis which can progress leading to arrhythmia, right and/or left heart dysfunction
and failure. Symptoms range from unusual to prominent and from mild to dramatic, but clinically overt disease is a poor prognostic
factor. Primary myocardial involvement is related to focal ischemia due to transient coronary spasm, and the available data support
that microvascular functional and structural abnormalities rather than macrovascular coronary involvement represent the main underlying
mechanism of the disease. However, the existence and prevalence of atherosclerotic coronary artery disease in SSc remain to be determined,
as several studies have generated conflicting reports. Despite the lack of effective targeted therapy for SSc itself, sensitive and
quantitative techniques have demonstrated the ability of vasodilators to improve myocardial function and perfusion and to prevent the
evolution of subclinical heart involvement to decompensated heart failure. Further research will provide a better understanding of the disease
by detecting the potent contribution of coronary artery involvement, explaining differences in accelerated atherosclerosis between
SSc and other autoimmune disorders, and opening directions for the development of novel treatment strategies for this life-threatening
complication of SSc.
Keywords: Systemic sclerosis, cardiac involvement, myocardium, small coronary artery disease, pulmonary arterial hypertension, tissue
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