Browsing by Author "Buch, Maya H (7003995450)"

Introduction: Heart involvement is a common problem in systemic sclerosis. Recently, a definition of systemic sclerosis primary heart involvement had been proposed. Our aim was to establish consensus guidance on the screening, diagnosis and follow-up of systemic sclerosis primary heart involvement patients. Methods: A systematic literature review was performed to investigate the tests used to evaluate heart involvement in systemic sclerosis. The extracted data were categorized into relevant domains (conventional radiology, electrocardiography, echocardiography, cardiac magnetic resonance imaging, laboratory, and others) and presented to experts and one patient research partner, who discussed the data and added their opinion. This led to the formulation of overarching principles and guidance statements, then reviewed and voted on for agreement. Consensus was attained when the mean agreement was ⩾7/10 and of ⩾70% of voters. Results: Among 2650 publications, 168 met eligibility criteria; the data extracted were discussed over three meetings. Seven overarching principles and 10 guidance points were created, revised and voted on. The consensus highlighted the importance of patient counseling, differential diagnosis and multidisciplinary team management, as well as defining screening and diagnostic approaches. The initial core evaluation should integrate history, physical examination, rest electrocardiography, trans-thoracic echocardiography and standard serum cardiac biomarkers. Further investigations should be individually tailored and decided through a multidisciplinary management. The overall mean agreement was 9.1/10, with mean 93% of experts voting above 7/10. Conclusion: This consensus-based guidance on screening, diagnosis and follow-up of systemic sclerosis primary heart involvement provides a foundation for standard of care and future feasibility studies that are ongoing to support its application in clinical practice. © The Author(s) 2023.

Introduction: Heart involvement is a common problem in systemic sclerosis. Recently, a definition of systemic sclerosis primary heart involvement had been proposed. Our aim was to establish consensus guidance on the screening, diagnosis and follow-up of systemic sclerosis primary heart involvement patients. Methods: A systematic literature review was performed to investigate the tests used to evaluate heart involvement in systemic sclerosis. The extracted data were categorized into relevant domains (conventional radiology, electrocardiography, echocardiography, cardiac magnetic resonance imaging, laboratory, and others) and presented to experts and one patient research partner, who discussed the data and added their opinion. This led to the formulation of overarching principles and guidance statements, then reviewed and voted on for agreement. Consensus was attained when the mean agreement was ⩾7/10 and of ⩾70% of voters. Results: Among 2650 publications, 168 met eligibility criteria; the data extracted were discussed over three meetings. Seven overarching principles and 10 guidance points were created, revised and voted on. The consensus highlighted the importance of patient counseling, differential diagnosis and multidisciplinary team management, as well as defining screening and diagnostic approaches. The initial core evaluation should integrate history, physical examination, rest electrocardiography, trans-thoracic echocardiography and standard serum cardiac biomarkers. Further investigations should be individually tailored and decided through a multidisciplinary management. The overall mean agreement was 9.1/10, with mean 93% of experts voting above 7/10. Conclusion: This consensus-based guidance on screening, diagnosis and follow-up of systemic sclerosis primary heart involvement provides a foundation for standard of care and future feasibility studies that are ongoing to support its application in clinical practice. © The Author(s) 2023.

Objective. The aim of this study was to evaluate the level of agreement among international experts on the World Scleroderma Foundation/Heart Failure Association consensus-based definition of systemic sclerosis–associated primary heart involvement (SSc-pHI) and on the guidance for its screening, diagnosis, and follow-up assessment, including feasibility and applicability assessments. Methods. An online survey was conducted to assess the level of agreement and feasibility/applicability using a 10-point scale (0 = not at all, 10 = completely agree or completely feasible/applicable). The weblink to the survey was shared with 1199 participants worldwide. A high level of agreement was defined by an average score over 7/10. Results. In total, 161 external experts completed the survey. Most of them were rheumatologists (80.7%), working in Europe (81.4%), and had > 10 years of clinical experience in managing SSc patients (59%). Overall agreement among external experts was high (mean 8.27, SD 1.86). The highest scores regarded items emphasizing the involvement of a multidisciplinary team, personalization of patient management, and initial evaluation techniques. A lower level of agreement was obtained in questions related to cardiac magnetic resonance imaging and endomyocardial biopsy. No factors associated with low level of agreement and feasibility/applicability were identified. Conclusion. The consensus-based definition and guidance on screening, diagnosis, and follow-up assessment of SSc-pHI provides a basis for application by the wider community. A lower level of agreement on the use of advanced or more invasive diagnostic techniques likely reflects regional differences in access and the need for more evidence on its use. This emphasizes the importance of involving a multidisciplinary team. © The Author(s) 2025.

Objective. The aim of this study was to evaluate the level of agreement among international experts on the World Scleroderma Foundation/Heart Failure Association consensus-based definition of systemic sclerosis–associated primary heart involvement (SSc-pHI) and on the guidance for its screening, diagnosis, and follow-up assessment, including feasibility and applicability assessments. Methods. An online survey was conducted to assess the level of agreement and feasibility/applicability using a 10-point scale (0 = not at all, 10 = completely agree or completely feasible/applicable). The weblink to the survey was shared with 1199 participants worldwide. A high level of agreement was defined by an average score over 7/10. Results. In total, 161 external experts completed the survey. Most of them were rheumatologists (80.7%), working in Europe (81.4%), and had > 10 years of clinical experience in managing SSc patients (59%). Overall agreement among external experts was high (mean 8.27, SD 1.86). The highest scores regarded items emphasizing the involvement of a multidisciplinary team, personalization of patient management, and initial evaluation techniques. A lower level of agreement was obtained in questions related to cardiac magnetic resonance imaging and endomyocardial biopsy. No factors associated with low level of agreement and feasibility/applicability were identified. Conclusion. The consensus-based definition and guidance on screening, diagnosis, and follow-up assessment of SSc-pHI provides a basis for application by the wider community. A lower level of agreement on the use of advanced or more invasive diagnostic techniques likely reflects regional differences in access and the need for more evidence on its use. This emphasizes the importance of involving a multidisciplinary team. © The Author(s) 2025.

Introduction: Primary heart involvement in systemic sclerosis may cause morpho-functional and electrical cardiac abnormalities and is a common cause of death. The absence of a clear definition of primary heart involvement in systemic sclerosis limits our understanding and ability to focus on clinical research. We aimed to create an expert consensus definition for primary heart involvement in systemic sclerosis. Methods: A systematic literature review of cardiac involvement and manifestations in systemic sclerosis was conducted to inform an international and multi-disciplinary task force. In addition, the nominal group technique was used to derive a definition that was then subject to voting. A total of 16 clinical cases were evaluated to test face validity, feasibility, reliability and criterion validity of the newly created definition. Results: In total, 171 publications met eligibility criteria. Using the nominal group technique, 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 the first round and 0.55 (0.44–1.00) for the 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 primary heart involvement in systemic sclerosis consensus-based definition was created and partially validated, for use in future clinical research. © The Author(s) 2021.

Introduction: Primary heart involvement in systemic sclerosis may cause morpho-functional and electrical cardiac abnormalities and is a common cause of death. The absence of a clear definition of primary heart involvement in systemic sclerosis limits our understanding and ability to focus on clinical research. We aimed to create an expert consensus definition for primary heart involvement in systemic sclerosis. Methods: A systematic literature review of cardiac involvement and manifestations in systemic sclerosis was conducted to inform an international and multi-disciplinary task force. In addition, the nominal group technique was used to derive a definition that was then subject to voting. A total of 16 clinical cases were evaluated to test face validity, feasibility, reliability and criterion validity of the newly created definition. Results: In total, 171 publications met eligibility criteria. Using the nominal group technique, 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 the first round and 0.55 (0.44–1.00) for the 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 primary heart involvement in systemic sclerosis consensus-based definition was created and partially validated, for use in future clinical research. © The Author(s) 2021.