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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Cardiovascular disease in systemic sclerosis
Francesca Cannarile1, Valentina Valentini1, Giulia Mirabelli1
1Department of Medicine, Rheumatology Unit, University of Perugia, Via dal Pozzo 06132, Perugia, Italy.
Insights
Systemic sclerosis (SSc) patients show increased macrovascular disease and premature atherosclerosis, linked to inflammation and endothelial damage. This review examines the causes and clinical impact of these cardiovascular complications in SSc.
Area of Science:
- Rheumatology
- Cardiology
- Immunology
Background:
- Cardiovascular system involvement is common in autoimmune diseases like systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA).
- While microvascular disease is characteristic of systemic sclerosis (SSc), recent studies indicate a higher prevalence of macrovascular disease in SSc patients compared to controls.
- This macrovascular disease in SSc is associated with a poorer prognosis.
Purpose of the Study:
- To analyze the causes of macrovascular involvement and premature atherosclerosis (ATS) in systemic sclerosis (SSc).
- To describe the clinical manifestations of macrovascular disease and ATS in SSc patients.
- To review the current understanding of mechanisms underlying ATS in SSc.
Main Methods:
- Literature review of studies investigating cardiovascular complications in systemic sclerosis (SSc).
- Analysis of research on the prevalence and mechanisms of macrovascular disease and atherosclerosis in SSc.
- Synthesis of data on endothelial dysfunction, inflammation, and vascular repair in SSc-related ATS.
Main Results:
- Systemic sclerosis (SSc) is associated with a higher prevalence of macrovascular disease and premature atherosclerosis (ATS) compared to the general population.
- Mechanisms contributing to ATS in SSc include multi-system organ inflammation, endothelial damage, vasculopathy, and impaired vascular repair.
- Endothelial cell injury, ischemia/reperfusion, immune-mediated cytotoxicity, and defective vasculogenesis play key roles in vascular injury in SSc.
Conclusions:
- Macrovascular disease and premature atherosclerosis are significant concerns in systemic sclerosis (SSc) patients.
- Understanding the complex interplay of inflammation, endothelial dysfunction, and immune mechanisms is crucial for managing cardiovascular risk in SSc.
- Further research is needed to elucidate the precise pathways and develop targeted therapies for ATS in SSc.
Abstract:
Cardiovascular (CV) system involvement is a frequent complication of autoimmune diseases such as systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA). It still remains unclear if a premature atherosclerosis (ATS) occurs even in systemic sclerosis (SSc). Although microvascular disease is a hallmark of SSc, in the last few years a number of studies highlighted a higher prevalence of macrovascular disease in SSc patients in comparison to healthy individuals and these data have been correlated with a poorer prognosis. The mechanisms promoting ATS in SSc are not fully understood, but it is believed to be secondary to multi-system organ inflammation, endothelial wall damage and vasculopathy. Both traditional risk factors and endothelial dysfunction have been proposed to participate to the onset and progression of ATS in such patients. In particular, endothelial cell injury induced by anti-endothelial antibodies, ischemia/reperfusion damage, immune-mediated cytotoxicity represent the main causes of vascular injury together with an impaired vascular repair mechanism that determine a defective vasculogenesis. Aim of this review is to analyse both causes and clinical manifestations of macrovascular involvement and ATS in SSc.
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