Heart involvement in systemic sclerosis

Abstract

Systemic sclerosis (SSc) is coccenctive tissue disease characterized by progressive fibrosis of the skin and internal organs leading to their failure, autoimmune dysfunction and the morphological and functional changes in the blood vessels. Primary cardiac involvement is common for patients with SSc, and when it becomes clinically evident appears as a bad prognostic factor. There is increasing evidence that heart failure occurs as a result of repeated episodes of ischemia leading to focal irreversible myocardial fibrosis. The reasons for this phenomenon are impaired microcirculation and abnormal vasoreactivity with or without associated structural abnormalities. Myocardial fibrosis and vascular microangiopathy lead to arrhythmias and impaired contractility with secondary left and right heart failure, as evidenced by the latest quantitative methods, such as echocardiography and MRI.