Primary myocardial disease in scleroderma – a comprehensive review of the literature to inform the UK Systemic Sclerosis Study Group cardiac working group

Cardiac disease is prevalent in Systemic Sclerosis (SSc) and associated with a poor prognosis. Differentiating primary myocardial disease (SSc-cardiomyopathy) from ischaemic heart disease (IHD) is difficult, and the disease phenotype most at risk is unclear. A comprehensive literature review was performed to inform the UK Systemic Sclerosis Study Group for cardiac disease tasked with producing a best practice pathway for the management of cardiac disease in SSc. This review describes the prevalence of SSc-cardiomyopathy, its associated greater mortality, and various manifestations, for example, heart failure, arrhythmias and diastolic dysfunction. The limited evidence suggests SSc-cardiomyopathy is associated with other poor prognostic indicators such as diffuse cutaneous disease, positive SSc-specific serology, black ethnicity, older age at disease onset, tendon friction rubs, abnormal nail-fold capillaroscopy and worse quality of life scores. Differentiating SSc-cardiomyopathy from IHD requires well planned studies. Non-invasive investigative techniques are improving the understanding of its pathophysiological basis.