Abstract
Introduction: primary heart involvement in systemic sclerosis (SSc-pHI) may cause morpho-functional and electrical cardiac abnormalities and is a common cause of death. The absence of a clear definition of SSc-pHI limits our understanding and ability to focus clinical research. We aimed to create an expert consensus definition for SSc-pHI.
Methods: A systematic literature review of cardiac involvement and manifestations in SSc was conducted to inform an international and multi-disciplinary task-force. In addition, the nominal group technique (NGT) was used to derive a definition that was then subject to voting. Sixteen clinical cases were evaluated to test face validity, feasibility, reliability and criterion validity of the newly created definition.
Results: 171 publications met eligibility criteria. Using the NGT, experts added their opinion, provided statements to consider and ranked them to create the consensus definition, which received 100% agreement on face validity. A median 60(5-300) seconds was taken for the feasibility on a single case. Inter-rater agreement was moderate [mKappa(95%CI) 0.56(0.46-1.00) for first and 0.55(0.44-1.00) for second round] and intra-rater agreement was good [mKappa(95%CI) 0.77(0.47-1,00)]. Criterion validity showed a 78(73-84) % correctness versus gold standard.
Conclusion: A preliminary SSc-pHI consensus-based definition was created and partially validated, for use in future clinical research.
Methods: A systematic literature review of cardiac involvement and manifestations in SSc was conducted to inform an international and multi-disciplinary task-force. In addition, the nominal group technique (NGT) was used to derive a definition that was then subject to voting. Sixteen clinical cases were evaluated to test face validity, feasibility, reliability and criterion validity of the newly created definition.
Results: 171 publications met eligibility criteria. Using the NGT, experts added their opinion, provided statements to consider and ranked them to create the consensus definition, which received 100% agreement on face validity. A median 60(5-300) seconds was taken for the feasibility on a single case. Inter-rater agreement was moderate [mKappa(95%CI) 0.56(0.46-1.00) for first and 0.55(0.44-1.00) for second round] and intra-rater agreement was good [mKappa(95%CI) 0.77(0.47-1,00)]. Criterion validity showed a 78(73-84) % correctness versus gold standard.
Conclusion: A preliminary SSc-pHI consensus-based definition was created and partially validated, for use in future clinical research.
| Original language | English |
|---|---|
| Journal | Journal of Scleroderma and Related Disorders |
| Publication status | Accepted/In press - 23 Sept 2021 |