Bruni Cosimo, Buch Maya H, Djokovic Aleksandra, De Luca Giacomo, Dumitru Raluca B, Giollo Alessandro, Galetti Ilaria, Steelandt Alexia, Bratis Konstantinos, Suliman Yossra Atef, Milinkovic Ivan, Baritussio Anna, Hasan Ghadeer, Xintarakou Anastasia, Isomura Yohei, Markousis-Mavrogenis George, Mavrogeni Sophie, Gargani Luna, Caforio Alida Lp, Tschöpe Carsten, Ristic Arsen, Plein Sven, Behr Elijah, Allanore Yannick, Kuwana Masataka, Denton Christopher P, Furst Daniel E, Khanna Dinesh, Krieg Thomas, Marcolongo Renzo, Pepe Alessia, Distler Oliver, Sfikakis Petros, Seferovic Petar, Matucci-Cerinic Marco
Division of Rheumatology, Department of Experimental and Clinical Medicine, Careggi University Hospital (AOUC), University of Florence, Florence, Italy.
Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland.
J Scleroderma Relat Disord. 2023 Oct;8(3):169-182. doi: 10.1177/23971983231163413. Epub 2023 Apr 3.
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.
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.
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.
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.
心脏受累是系统性硬化症中的常见问题。最近，有人提出了系统性硬化症原发性心脏受累的定义。我们的目的是就系统性硬化症原发性心脏受累患者的筛查、诊断和随访建立共识性指南。
进行了一项系统的文献综述，以研究用于评估系统性硬化症心脏受累情况的检查。提取的数据被分类到相关领域（传统放射学、心电图、超声心动图、心脏磁共振成像、实验室检查及其他），并提交给专家和一名患者研究伙伴，他们对数据进行了讨论并发表了意见。这促成了总体原则和指导声明的制定，然后进行了审查并投票表决是否达成一致。当平均同意率⩾7/10且⩾70%的投票者同意时，即达成共识。
在2650篇出版物中，168篇符合纳入标准；提取的数据在三次会议上进行了讨论。创建、修订并投票表决了七条总体原则和十条指导要点。共识强调了患者咨询、鉴别诊断和多学科团队管理的重要性，以及明确筛查和诊断方法。初始核心评估应综合病史、体格检查、静息心电图、经胸超声心动图和标准血清心脏生物标志物。进一步的检查应根据个体情况进行定制，并通过多学科管理来决定。总体平均同意率为9.1/10，平均93%的专家投票高于7/10。
这份关于系统性硬化症原发性心脏受累筛查、诊断和随访的基于共识的指南为护理标准以及正在进行的支持其在临床实践中应用的未来可行性研究奠定了基础。
