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First-line diagnostic tests to intercept primary heart involvement in systemic sclerosis: Clinical associations from

Antonio Tonutti1,2, Francesca Motta1,2, Rossella De Angelis3,4

  • 1Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, Milan, Italy.

European Journal of Clinical Investigation
|July 4, 2025
PubMed

Insights

Primary heart involvement (PHI) affects a quarter of systemic sclerosis (SSc) patients, increasing heart failure risk. Early detection in SSc patients with diffuse skin fibrosis, muscle, or intestinal issues is vital.

Area of Science:

  • Cardiology
  • Rheumatology
  • Systemic Sclerosis Research

Background:

  • Primary heart involvement (PHI) is an underrecognized complication of systemic sclerosis (SSc).
  • PHI is linked to heart failure, arrhythmias, and mortality.
  • Diagnostic criteria and risk factors for SSc-PHI require further elucidation despite consensus definitions.

Purpose of the Study:

  • To identify factors associated with primary heart involvement in systemic sclerosis patients.
  • To characterize the prevalence and phenotypes of SSc-PHI.
  • To investigate the longitudinal development of SSc-PHI.

Main Methods:

  • Analysis of 600 patients from the Italian national SPRING registry with defined parameters for SSc-PHI.
  • Exclusion of patients with confounding conditions or incomplete cardiac assessments.
  • Cross-sectional and longitudinal analyses to determine risk factors and disease progression.

Main Results:

  • SSc-PHI was detected in 25% of patients at baseline, associated with older age, diffuse cutaneous SSc, and intestinal symptoms.
  • Diastolic dysfunction (62%) and conduction disturbances (34%) were the most common SSc-PHI phenotypes.
  • An additional 25% of patients developed new-onset SSc-PHI during follow-up, particularly those with skeletal muscle involvement.

Conclusions:

  • Primary heart involvement is a significant complication affecting approximately 25% of SSc patients.
  • Early detection of SSc-PHI is crucial for patient management.
  • Patients with diffuse skin fibrosis, muscular involvement, and intestinal manifestations require close cardiac monitoring.
Abstract

Keywords:
autoimmunityconnective tissue diseaseechocardiographyimmunologyrisk factorssystemic sclerosis

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