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Primary systemic sclerosis heart involvement: A systematic literature review and preliminary data-driven,
Cosimo Bruni1, Maya H Buch2,3, Daniel E Furst1,4
1Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence, Florence, Italy.
Journal of Scleroderma and Related Disorders
|April 7, 2022
Summary
A new expert consensus definition for primary heart involvement in systemic sclerosis was developed and validated. This definition aims to improve clinical research understanding of cardiac abnormalities in systemic sclerosis patients.
Area of Science:
- Rheumatology and Cardiology
- Systemic Sclerosis Research
- Cardiac Manifestations
Background:
- Primary heart involvement in systemic sclerosis leads to significant cardiac abnormalities and mortality.
- Lack of a clear definition hinders clinical research and understanding of cardiac involvement in systemic sclerosis.
Purpose of the Study:
- To establish an expert consensus definition for primary heart involvement in systemic sclerosis.
- To facilitate focused clinical research on cardiac issues in systemic sclerosis.
Main Methods:
- Systematic literature review on cardiac involvement in systemic sclerosis.
- Nominal group technique employed by an international, multi-disciplinary task force.
- Validation using 16 clinical cases for face validity, feasibility, reliability, and criterion validity.
Main Results:
- A consensus definition was created with 100% agreement on face validity.
- Moderate inter-rater agreement (mKappa = 0.56) and good intra-rater agreement (mKappa = 0.77) were observed.
- Criterion validity demonstrated 78% correctness against a gold standard.
Conclusions:
- A preliminary, consensus-based definition for primary heart involvement in systemic sclerosis has been established.
- This definition has undergone partial validation and is intended for future clinical research applications.
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