Cardiovascular Risk in Systemic Sclerosis

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Scleroderma (S Bhattacharyya, Section Editor)

Cardiovascular Risk in Systemic Sclerosis Eleni Angeloudi, MD1 Eleni Pagkopoulou, MD2 Alexandra Arvanitaki, MD2,3 Stergios Soulaidopoulos4 Alexandros Garyfallos, MD, PhD2 George Kitas, MD, PhD5 Theodoros Dimitroulas, MD, PhD2,* Address 1 Second Department of Internal Medicine, Hippokration General Hospital, Medical School,, Aristotle University of Thessaloniki, Konstantinoupoleos Str. 49, Thessaloniki, Greece *,2 Fourth Department of Internal Medicine, Hippokration General Hospital, Medical School,, Aristotle University of Thessaloniki, Konstantinoupoleos Str. 49, Thessaloniki, Greece Email: [email protected] 3 First Department of Cardiology, AHEPA University Hospital, Medical School,, Aristotle University of Thessaloniki, Athens, Greece 4 First Department of Cardiology, Medical School of Athens University, Hippokration Hospital, Athens, Greece 5 Department of Rheumatology, Russells Hall Hospital, Dudley Group NHS FT, Pensnett Road,, Dudley, DY1 2HQ, UK

* Springer Nature Switzerland AG 2020

This article is part of the Topical Collection on Scleroderma Keywords Systemic sclerosis I Scleroderma I Cardiovascular disease I Atherosclerosis I Macrovascular disease

Abstract Purpose of review Systemic sclerosis (SSc) is a systemic inflammatory, autoimmune disorder characterized by diffuse fibrosis of the skin and visceral organ involvement. Endothelial dysfunction and microvascular injury dominate the pathophysiology and clinical manifestations of the disease, while the impact of macrovascular atherosclerotic disease on cardiovascular (CVD) morbidity and mortality is yet to be established. In this article, we aim to review current knowledge about CVD as well as cardiac complications in SSc and discuss the potentially implicated pathogenetic mechanisms. Recent findings Systemic inflammation has been identified as an important trigger and contributor for the development and progression of atherosclerosis, closely associated with high cardiovascular mortality in patients with autoimmune disorders, such as rheumatoid arthritis. A close interplay between traditional risk factors and factors related to

Scleroderma (S Bhattacharyya, Section Editor) the disease, including inflammation, endothelial injury, and immune-mediated cytotoxicity, sharing common pathogenetic features with microvasculopathy, may be responsible for large-vessel involvement and promotion of atherosclerosis in SSc. Cardiac complications, including heart failure due to impairment of coronary microcirculation and myocardial fibrosis, are listed among the primary cause of death in SSc. Evaluation of indirect surrogate markers of CVD, namely, arterial stiffness, carotid media thickness, and flow-mediated dilation, in small studies has provided inconsistent results regarding the association between SSc and atherosclerosis, highlighting the need for further research on this field. In this article, we aim to review current knowledge about large-vessel involvement and CVD in SSc and discuss the potentially implic