Focusing on Arterial and Cardiac Dysfunction in Systemic Sclerosis: Targeted Therapy is Still Pending

Abstract

Although the pathophysiology and therapy of microvascular damage was so far addressed by both rheumatologists and angiologists, cardiac disease related to Systemic sclerosis (SSc) remained obscure or misdiagnosed for long time. Although its pathogenetic chain is far to be completely understood, myocardial fibrosis and microvascular dysfunction play a prominent role in the clinical history of patients with SSc. Bearing in mind that it is one of the main causes of death and disabilities for SSc patients, an early screening has become mandatory at time of diagnosis, and then every year during the follow-up. Moreover, the prevalence of coronary heart disease in SSc resulted comparable, in older patients, to that of patients with type 2 diabetics. Global longitudinal strain (GLS), a well-known early echocardiographic marker of cardiac dysfunction, has been recently shown to be a useful tool predicting prognosis and effects of therapy in SSc. Target therapy for primary cardiac disease in SSc is so far not well established. Inhibitor of IL-1 and IL-6 are under investigation. The acute infusion of iloprost ameliorates the baseline depressed GLS of left ventricle in patients with SSc, but this beneficial effect remains to be confirmed during chronic treatment.

Keywords: cardiomyopathy; global longitudinal strain; iloprost; systemic sclerosis.