Recent advances in the heart and soul of primary cardiac involvement and cardiovascular disease in systemic sclerosis.

Systemic sclerosis (SSc) has direct effects on the heart. Primary SSc-associated heart involvement (pSHI) is a spectrum of disease, with myriad presentations that range from acute heart failure and myocarditis, breathlessness due to diastolic dysfunction to asymptomatic ventricular ectopy. There have been recent significant advances towards the standardized identification of pSHI with the development of both a consensus definition and classification criteria for pSHI. Such instruments are required to improve the quality of research in this area by enabling the recruitment of homogenous patient populations and permitting comparison of results between studies. Prospective studies are required to test the efficacy of screening strategies to identify pSHI at an early stage, as well as the effectiveness of immunosuppressive, anti-fibrotic and heart failure management strategies to reverse the disease related effects of SSc on the heart.