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Updated: Jul 11, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Heart and systemic sclerosis-findings from a national cohort study
Alexis F Guédon1,2, Fabrice Carrat2,3, Luc Mouthon4
1Sorbonne Université, APHP, Service de Médecine Interne, Département Hospitalo-Universitaire Inflammation-Immunopathologie-Biotherapie (DMU i3), Paris, France.
Insights
Cardiac involvement in Systemic Sclerosis (SSc) is a major concern. This study reveals its prevalence, risk factors like diffuse SSc, and its association with increased mortality in SSc patients.
Area of Science:
- Cardiology
- Rheumatology
- Epidemiology
Background:
- Cardiac involvement is a leading cause of death in Systemic Sclerosis (SSc).
- The prevalence and prognostic impact of SSc-related heart disease are not well-established.
- Understanding these factors is crucial for patient management and outcomes.
Purpose of the Study:
- To investigate the prevalence and prognosis of SSc-related cardiac involvement.
- To determine the incidence and risk factors of heart diseases in a nationwide SSc cohort.
- To analyze the survival impact of various cardiac conditions in SSc.
Main Methods:
- Utilized data from a multicentric prospective study of the French SSc national database.
- Focused on identifying SSc-related cardiac involvement, its incidence, and associated risk factors.
- Conducted survival analyses on a cohort of SSc patients with and without cardiac involvement.
Main Results:
- 10.9% of 3528 SSc patients had cardiac involvement at baseline, associated with diffuse subtype and cardiovascular risk factors.
- SSc-related cardiac involvement increased the risk of death; no significant survival difference was noted between SSc-related cardiac involvement, ischemic heart disease, or pulmonary arterial hypertension.
- 5-year event rates: 11.15% for SSc-related cardiac involvement, 2.49% for reduced LVEF, 5.84% for diastolic dysfunction, and 3% for pericarditis. Diffuse SSc subtype was a risk factor for SSc-related cardiac involvement and pericarditis; female sex was linked to less diastolic dysfunction.
Conclusions:
- This large-scale study clarifies the incidence and prognostic burden of SSc-related cardiac involvement.
- Diffuse SSc subtype and female sex are identified as significant risk factors for specific cardiac outcomes.
- Further research is needed to evaluate the impact of treatments on cardiac outcomes in SSc.
Objectives:
Heart involvement is one of the leading causes of death in SSc. The prevalence of SSc-related cardiac involvement is poorly known. Our objective was to investigate the prevalence and prognosis burden of different heart diseases in a nationwide cohort of patients with SSc.
Methods:
We used data from a multicentric prospective study using the French SSc national database. Focusing on SSc-related cardiac involvement, we aimed to determine its incidence and risk factors.
Results:
Of the 3528 patients with SSc, 312 (10.9%) had SSc-related cardiac involvement at baseline. They tended to have a diffuse SSc subtype more frequently and to have more severe clinical features, and presented more cardiovascular risk factors. From the 1646 patients available for follow-up analysis, SSc-related cardiac involvement was associated with an increased risk of death. There was no significant difference in overall survival between SSc-related cardiac involvement, ischaemic heart disease or pulmonary arterial hypertension. Regarding survival analysis, 98 patients developed SSc-related cardiac involvement at 5 years (5-year event rate 11.15%). Regarding reduced left ventricular ejection fraction <50% and left ventricular diastolic dysfunction, the 5-year event rate was 2.49% and 5.84%, respectively. Pericarditis cumulative incidence at 5 years was 3%. Diffuse SSc subtype was a risk factor for SSc-related cardiac involvement and pericarditis. Female sex was associated with less left ventricular diastolic dysfunction incidence.
Conclusions:
Our results describe the incidence and prognostic burden of SSc-related cardiac involvement at a large scale, with gender and diffuse SSc subtype as risk factors. Further analyses should assess the potential impact of treatment on these various cardiac outcomes.
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