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Updated: Jun 24, 2026
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Heart involvements in systemic sclerosis beyond pulmonary hypertension: From conduction, rhythm and function defects
Raffaele Barile1, Cinzia Rotondo1, Giulio Giancaspro1
1Rheumatology Unit, Department of Medical and Surgical Sciences, University of Foggia, 71122 Foggia, Italy.
Insights
Systemic sclerosis (SSc) significantly impacts cardiac health, causing fibrosis and dysfunction in many patients. Early screening and multidisciplinary care are crucial for managing this underrecognized cause of morbidity and mortality.
Area of Science:
- Cardiology
- Rheumatology
- Systemic Sclerosis Research
Background:
- Systemic sclerosis (SSc) is a rare autoimmune disease characterized by fibrosis, vasculopathy, and immune dysregulation, leading to multiorgan damage.
- Cardiac involvement in SSc, including myocardial fibrosis and coronary microvascular dysfunction, is a major, often underestimated, cause of morbidity and mortality.
- Subclinical cardiac dysfunction affects up to 70% of SSc patients, with a tenfold higher risk of sudden cardiac death compared to the general population.
Purpose of the Study:
- To review the current evidence on the epidemiology, pathogenesis, diagnostic evaluation, and therapeutic strategies for cardiac involvement in Systemic Sclerosis.
- To emphasize the importance of early, systematic, and risk-stratified screening for cardiac complications in SSc patients.
- To advocate for dedicated multidisciplinary cardiac teams in managing SSc-related heart disease.
Main Methods:
- This narrative review synthesizes existing literature on cardiac manifestations in Systemic Sclerosis.
- Key diagnostic tools discussed include electrocardiography and cardiac magnetic resonance imaging (CMR) with late gadolinium enhancement.
- Pathophysiological mechanisms explored involve endothelial injury, fibrosis, and autonomic nervous system imbalance.
Main Results:
- Clinically apparent cardiac disease occurs in 15-35% of SSc patients, while advanced screening reveals dysfunction in ~70%.
- Electrocardiographic abnormalities (25-85%) and diastolic dysfunction (18-62%) are significant predictors of mortality.
- CMR imaging frequently detects myocardial fibrosis in SSc patients, even those without prior cardiovascular diagnoses.
Conclusions:
- Cardiac involvement is a prevalent and serious complication of Systemic Sclerosis, significantly contributing to patient mortality.
- Early detection of cardiac dysfunction through systematic screening is essential for improved outcomes.
- A multidisciplinary management approach, integrating SSc-specific therapies and cardiovascular risk reduction, is critical for addressing cardiac complications in SSc.
Abstract:
Systemic sclerosis (SSc) is a rare systemic disease characterised by progressive fibrosis, vasculopathy, and immune dysregulation, resulting in multiorgan damage. Independently of pulmonary arterial hypertension, cardiac involvement, encompassing myocardial fibrosis, coronary microvascular dysfunction, arrhythmias, conduction disorders, pericardial and valvular disease, and heart failure, represents a frequently underestimated cause of morbidity and mortality. Clinically manifest cardiac disease is observed in 15 to 35% of patients, while subclinical dysfunction is detectable in approximately 70% when advanced screening tools are employed. The annual incidence of sudden cardiac death is estimated at between 1.0% and 3.3%, exceeding the general-population risk by more than tenfold. The pathophysiological framework rests on coronary microvascular dysfunction driven by endothelial injury, ischaemia-reperfusion cycles, and TGF-β-mediated replacement fibrosis, potentiated by autonomic nervous system imbalance and activation of the renin-angiotensin-aldosterone system. Electrocardiographic abnormalities, detectable in 25 to 85% of patients, are independent predictors of mortality, while diastolic dysfunction, present in 18 to 62% of cases, constitutes a robust prognostic marker. Cardiac magnetic resonance imaging has transformed subclinical detection, revealing late gadolinium enhancement fibrosis in the majority of screened patients without a prior cardiovascular diagnosis. Management requires a multidisciplinary approach, integrating SSc-specific adaptation of guideline-directed heart failure therapies, immunosuppression targeting inflammatory and fibrotic pathways, arrhythmia management with implantable devices, and EULAR-recommended cardiovascular risk reduction. This narrative review synthesises current evidence on the epidemiology, pathogenesis, diagnostic evaluation, and therapeutic strategies of cardiac involvement in SSc, highlighting the need for early, systematic, risk-stratified screening and the establishment of dedicated multidisciplinary cardiac teams.
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