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Updated: Jun 17, 2025
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Heart involvement in systemic sclerosis: emerging concepts
Jessica L Fairley1,2, Laura Ross1,2, Mandana Nikpour1,2,3,4,5
1The University of Melbourne.
Insights
Systemic sclerosis-associated heart involvement (SHI) is common and increases sudden cardiac death risk in SSc patients. Routine screening for SHI in all patients requires caution due to limited evidence-based treatments.
Area of Science:
- Cardiology
- Rheumatology
- Immunology
Background:
- Systemic sclerosis (SSc) significantly increases morbidity and mortality.
- Cardiac involvement in SSc (SHI) presents diversely, including arrhythmias and dysfunction.
- Sudden cardiac death risk is substantially elevated in SSc patients.
Purpose of the Study:
- To review the pathogenesis, prevention, and management of SHI.
- To discuss screening strategies for SHI based on new recommendations.
- To highlight the under-recognized burden of SHI in SSc.
Main Methods:
- Literature review of SHI.
- Analysis of clinical features and pathogenesis.
- Evaluation of new screening recommendations for SHI.
Main Results:
- SHI encompasses a spectrum of fibroinflammatory cardiac disease.
- New recommendations exist for routine SHI screening in SSc patients.
- The burden of SHI is substantial but often under-recognized.
Conclusions:
- SHI contributes significantly to increased sudden cardiac death in SSc.
- Broad screening, including low-risk patients, warrants caution due to limited interventions.
- Further research is needed to guide evidence-based management of SHI.
Purpose Of Review:
Systemic sclerosis (SSc)-associated heart involvement (SHI) is a significant cause of both morbidity and mortality in individuals with SSc. SHI can take many different forms, and likely is a spectrum of fibroinflammatory cardiac disease. Presenting features include arrhythmia, ventricular systolic or diastolic dysfunction, pericardial disease, and exercise intolerance. Risk of sudden cardiac death in SSc is likely 10-30-fold greater than general population estimates. In this review, we explore what is known about the pathogenesis of SHI, its prevention and management, and discuss available strategies for screening for SHI in light of new recommendations for the routine screening of SHI in all SSc patients.
Recent Findings:
We describe the spectrum, clinical features, and pathogenesis of SHI. Furthermore, we review the new recommendations for screening for SHI in individuals with SSc.
Summary:
There is a large, under-recognized burden of SHI in people living with SSc, which likely contributes to the significant increase in sudden cardiac death observed in SSc. However, a broad-based screening approach, including asymptomatic, low-risk patients should be viewed with caution given the lack of evidence-based treatments and interventions for SHI particularly in this group.
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