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Updated: Jun 17, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
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Heart involvement in systemic sclerosis: emerging concepts.

Jessica L Fairley1,2, Laura Ross1,2, Mandana Nikpour1,2,3,4,5

  • 1The University of Melbourne.

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|August 9, 2024
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Summary
This summary is machine-generated.

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.

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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.