Cardiac involvement in systemic sclerosis: mechanisms, manifestations and management

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Updated: Feb 6, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
04:44

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Published on: June 16, 2020

20.9K

Chin Yit Soo1, Nicholas Jex1, Lesley-Anne Bissell2

  • 1Biomedical Imaging Science Department, Leeds Institute of Cardiovascular and Metabolic Medicine, University of Leeds, United Kingdom.

Rheumatology (Oxford, England)
|February 4, 2026
PubMed

Insights

Systemic sclerosis can cause primary heart involvement (SSc-PHI) through vascular and immune pathways, leading to myocardial fibrosis. Early detection via screening and advanced imaging like CMR is crucial for better risk stratification and patient outcomes.

Area of Science:

  • Cardiology
  • Rheumatology
  • Immunology

Background:

  • Cardiovascular disease is a major cause of mortality in Systemic Sclerosis (SSc).
  • Primary heart involvement in SSc (SSc-PHI) significantly impacts patient outcomes.
  • Understanding SSc-PHI pathophysiology is vital for effective management.

Purpose of the Study:

  • To review the pathophysiology of SSc-PHI.
  • To outline clinical manifestations and management strategies.
  • To discuss unmet needs and potential solutions for SSc-PHI.

Main Methods:

  • Literature review focusing on SSc-PHI mechanisms, clinical features, and diagnostics.
  • Analysis of vascular and immune-mediated pathways.
  • Evaluation of screening tools including biomarkers and imaging (echocardiography, CMR).

Main Results:

  • SSc-PHI involves vascular and immune pathways, NLRP3 inflammasome, and pro-fibrotic cytokines, causing myocardial fibrosis.
  • Clinical manifestations require regular screening with biomarkers and echocardiography.
  • Cardiac MRI (CMR) detects subclinical disease with prognostic value.

Conclusions:

  • Early identification of subclinical SSc-PHI is a critical unmet need for improved risk stratification.
  • Enhanced interdisciplinary care through cardiology-rheumatology collaboration is essential.
  • Developing novel diagnostic and therapeutic strategies is imperative for managing SSc-PHI.

Keywords:
cardiac energeticscardiac fibrosiscardiac functioncardiac magnetic resonancecoronary microvascular circulationprimary heart involvementprognosissystemic sclerosis

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