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.

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