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Cardiovascular disease in systemic sclerosis - an emerging association?

Gene-Siew Ngian12*, Joanne Sahhar3, Ian P Wicks12 and Sharon Van Doornum12

Author Affiliations

1 The University of Melbourne, Department of Medicine (Royal Melbourne Hospital/Western Hospital), 4th Floor, Clinical Sciences Building, Royal Melbourne Hospital, Royal Parade, Parkville, Victoria 3050, Australia

2 Royal Melbourne Hospital, Grattan Street, Parkville, Victoria 3050, Australia

3 Monash Medical Centre, 246 Clayton Road, Clayton, Victoria 3168, Australia

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Arthritis Research & Therapy 2011, 13:237  doi:10.1186/ar3445

Published: 26 August 2011


Microvascular disease is a prominent feature of systemic sclerosis (SSc) and leads to Raynaud's phenomenon, pulmonary arterial hypertension, and scleroderma renal crisis. The presence of macrovascular disease is less well established, and, in particular, it is not known whether the prevalence of coronary heart disease in SSc is increased. Furthermore, in terms of cardiac involvement in SSc, there remains conjecture about the relative contributions of atherosclerotic macrovascular disease and myocardial microvascular disease. In this review, we summarize the literature describing cardiovascular disease in SSc, discuss the pathophysiological mechanisms common to SSc and atherosclerosis, and review the surrogate markers of cardiovascular disease which have been examined in SSc. Proposed mediators of the vasculopathy of SSc which have also been implicated in atherosclerosis include endothelial dysfunction, a reduced number of circulating endothelial progenitor cells, and an increased number of microparticles. Excess cardiovascular risk in SSc is suggested by increased arterial stiffness and carotid intima thickening and reduced flow-mediated dilatation. Cohort studies of adequate size are required to resolve whether this translates into an increased incidence of cardiovascular events in patients with SSc.