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Editorial
Getting to the heart of the matter: detecting and managing cardiac complications in systemic sclerosis
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  1. John Varga1,
  2. Daniel C Lee2
  1. 1 Northwestern Scleroderma Program, Chicago, Illinois, USA
  2. 2 Division of Cardiology, Northwestern University - Chicago, Chicago, Illinois, USA
  1. Correspondence to Dr John Varga, Medicine, Northwestern University, Chicago, IL 60611, USA; j-varga{at}northwestern.edu

https://doi.org/10.1136/annrheumdis-2019-216115

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    Cardiac complications of systemic sclerosis (SSc) are well-deservedly gaining the attention of the medical community. This increased interest is driven by technological advances in non-invasive imaging technologies such as tissue Doppler echocardiography and cardiac MR (CMR) that reveal frequent involvement of the heart and its clinical importance in SSc. Historically, autopsy studies in SSc indicate a high prevalence (>50%) of cardiac fibrosis,1 and clinically evident cardiac involvement has long been considered a poor prognostic marker in SSc.2 3 Indeed, the European Clinical Trials and Research Group reported that 26% of SSc disease-related mortality could be attributed to cardiac causes.4 However, the overall prevalence of cardiac disease in SSc is unknown, in part due to the lack of a consensus definition, classification of ‘subclinical’ cardiac involvement, and the plethora of diverse approaches employed for its detection.5

    The cardiac complications of SSc encompass multiple distinct entities including primary cardiac involvement manifested by diastolic dysfunction (an early manifestation of cardiac fibrosis), heart failure with preserved ejection faction, conduction blocks and arrhythmias, myocarditis, as well as pericardial disease; and cardiac involvement secondary to systemic or pulmonary arterial hypertension, renal failure, amyloidosis and other SSc complications. The pathogenesis of SSc-associated primary cardiac involvement is not well understood, and likely encompasses small vessel damage, vasoconstriction and chronic ischaemia-reperfusion injury, cardiac inflammation and fibrosis.

    Although systolic dysfunction in SSc is uncommon, diastolic dysfunction is common and predicts poor outcomes. In an observational study of 153 consecutive SSc patients, we showed that 23% had echocardiographically defined left ventricular diastolic dysfunction (often asymptomatic), and its presence was predictive of mortality.6 A recent study in a large and unselected cohort of SSc patients similarly found that the incidence of diastolic dysfunction was 17% at baseline, and increased to 29% during follow-up of 3.4 years.7 …

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