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Updated: Jul 1, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Macrovascular disease in systemic sclerosis: the tip of an iceberg?
J J F Belch1, S McSwiggan, C Lau
1Vascular and Inflammatory Diseases Research Unit, Ninewells Hospital, Tayside, Scotland, UK. J.J.F.Belch@dundee.ac.uk
Insights
Premature death occurs in systemic sclerosis (SSc). Interstitial lung disease is now the leading cause of SSc-related mortality, with cardiovascular disease also significantly contributing.
Area of Science:
- Rheumatology
- Cardiology
- Pulmonology
Background:
- Systemic sclerosis (SSc) is a multisystem autoimmune disease.
- Mortality in SSc patients has been a significant concern.
- Understanding the evolving causes of death is crucial for patient management.
Purpose of the Study:
- To review and evaluate the causes of mortality in patients with systemic sclerosis (SSc).
- To analyze the changing spectrum of causes of death over the past three decades in SSc.
- To assess the contribution of cardiovascular disease to premature mortality in SSc.
Main Methods:
- Literature review of studies evaluating mortality in SSc.
- Analysis of epidemiological data on SSc-related deaths.
- Examination of cardiovascular disease biomarkers in SSc patients.
Main Results:
- Premature death is confirmed in the SSc population.
- Interstitial lung disease has emerged as the most common cause of SSc-related mortality.
- Cardiovascular disease and events are significant contributors to premature mortality, supported by biomarker evidence.
Conclusions:
- The leading cause of death in systemic sclerosis has shifted towards interstitial lung disease.
- Cardiovascular disease plays a substantial role in the premature mortality observed in SSc patients.
- Further research is needed to determine if managing conventional risk factors can reduce cardiovascular disease impact in SSc.
Abstract:
This mini review evaluates mortality in SSc and provides a literature review concluding that premature death does occur in this population. However, there has been a changing spectrum of cause of death over the past three decades, with interstitial lung disease now being the commonest cause of SSc-related mortality. Cardiovascular (CV) mortality and events also contribute to the premature mortality seen in these patients, and this contention is supported by epidemiological studies, and further underpinned by a plethora of increased biomarkers for CV disease and events. Thus, macrovascular disease does occur in these patients, and is likely to contribute to mortality. It remains to be seen whether addressing conventional risk factors will attenuate CV disease in this population.
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