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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
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Definition, diagnosis, treatment for scleroderma primary heart involvement

DeAnna Baker Frost1, Ryan J Tedford2, Richard Silver1

  • 1Department of Medicine, Division of Rheumatology, Medical University of South Carolina, 96 Jonathan Lucas Street, Suite 833, MSC 637, Charleston, SC, 29425, USA.

Best Practice & Research. Clinical Rheumatology
|March 21, 2026
PubMed

Insights

Systemic sclerosis (SSc) is an autoimmune disease that can cause primary heart involvement (pHI), leading to significant mortality. Early screening and multidisciplinary management are crucial for patients with SSc-pHI.

Area of Science:

  • Cardiology
  • Rheumatology
  • Immunology

Background:

  • Systemic sclerosis (SSc) is an autoimmune disease causing inflammation and fibrosis.
  • Primary heart involvement (pHI) in SSc includes various cardiac dysfunctions.
  • SSc-pHI is associated with high mortality and sudden cardiac death risk.

Purpose of the Study:

  • To summarize the characteristics, diagnosis, and management of SSc-primary heart involvement.
  • To highlight the importance of early detection and multidisciplinary care for SSc-pHI.

Main Methods:

  • Review of SSc-pHI manifestations, diagnostic tools, and treatment strategies.
  • Emphasis on serum biomarkers (troponin, NT-proBNP), ECG, echocardiography, Holter monitoring, and cardiac MRI.
  • Discussion of multidisciplinary team approach involving rheumatologists and cardiologists.

Main Results:

  • SSc-pHI encompasses conduction abnormalities, arrhythmias, myocarditis, heart failure, and valvular dysfunction.
  • Biomarkers and imaging techniques aid in screening and diagnosis, with cMRI as the gold standard.
  • Treatment is multifaceted, often requiring immunosuppression.

Conclusions:

  • SSc-pHI is a serious complication of systemic sclerosis requiring prompt diagnosis and management.
  • A collaborative approach integrating rheumatology and cardiology is essential for optimal patient outcomes.
  • Continued research into biomarkers and targeted therapies is needed.

Keywords:
Cardiac magnetic resonance imagingSystemic sclerosisSystemic sclerosis primary heart involvement

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