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Cardiovascular Risk in Systemic Sclerosis

  • Scleroderma (S Bhattacharyya, Section Editor)
  • Published:
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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 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 implicated pathogenetic mechanisms.

Summary

SSc conveys a higher risk for CVD associated with both vascular and fibrotic complications during the course of the disease. Increasing attention is given on the use of vasodilators, immunosuppressants, and more recently antifibrotic drugs that potentially improve myocardial function and reduce atherosclerotic disease burden.

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Abbreviations

CVD:

cardiovascular disease

SSc:

systemic sclerosis

ESC/ERS:

European Society of Cardiology/European Respiratory Society

PAH:

pulmonary arterial hypertension

SHI:

scleroderma heart involvement

FMD:

flow-mediated dilatation

cIMT:

carotid intima-media thickness

CAD:

coronary artery disease

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Authors and Affiliations

  1. Second Department of Internal Medicine, Hippokration General Hospital, Medical School,, Aristotle University of Thessaloniki, Konstantinoupoleos Str. 49, Thessaloniki, Greece

    Eleni Angeloudi MD

  2. Fourth Department of Internal Medicine, Hippokration General Hospital, Medical School,, Aristotle University of Thessaloniki, Konstantinoupoleos Str. 49, Thessaloniki, Greece

    Eleni Pagkopoulou MD, Alexandra Arvanitaki MD, Alexandros Garyfallos MD, PhD & Theodoros Dimitroulas MD, PhD

  3. First Department of Cardiology, AHEPA University Hospital, Medical School,, Aristotle University of Thessaloniki, Athens, Greece

    Alexandra Arvanitaki MD

  4. First Department of Cardiology, Medical School of Athens University, Hippokration Hospital, Athens, Greece

    Stergios Soulaidopoulos

  5. Department of Rheumatology, Russells Hall Hospital, Dudley Group NHS FT, Pensnett Road,, Dudley, DY1 2HQ, UK

    George Kitas MD, PhD

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  1. Eleni Angeloudi MD
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Correspondence to Theodoros Dimitroulas MD, PhD.

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Eleni Angeloudi declares that she has no conflict of interest. Eleni Pagkopoulou declares that she has no conflict of interest. Alexandra Arvanitaki declares that she has no conflict of interest. Stergios Soulaidopoulos declares that he has no conflict of interest. Alexandros Garyfallos declares that he has no conflict of interest. George Kitas declares that he has no conflict of interest. Theodoros Dimitroulas declares that he has no conflict of interest.

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Angeloudi, E., Pagkopoulou, E., Arvanitaki, A. et al. Cardiovascular Risk in Systemic Sclerosis. Curr Treat Options in Rheum 6, 282–298 (2020). https://doi.org/10.1007/s40674-020-00152-z

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