PDF(Pubmed)
Abstract：
UNASSIGNED: Pulmonary arterial hypertension (PAH), defined as the presence of a mean pulmonary artery pressure > 20 mmHg, pulmonary artery wedge pressure ≤ 15 mmHg, and pulmonary vascular resistance (PVR) > 2 Wood units based on expert consensus, is characterized by a progressive and sustained increase in PVR, which may lead to right heart failure and death. PAH is a well-known complication of connective tissue diseases (CTDs), such as systemic sclerosis, systemic lupus erythematosus, Sjogren\'s syndrome, and other autoimmune conditions. In the past few years, tremendous progress in the understanding of PAH pathogenesis has been made, with various novel diagnostic and screening methods for the early detection of PAH proposed worldwide.
UNASSIGNED: This study aimed to obtain a comprehensive understanding and provide recommendations for the management of CTD-PAH in Taiwan, focusing on its clinical importance, prognosis, risk stratification, diagnostic and screening algorithm, and pharmacological treatment.
UNASSIGNED: The members of the Taiwan Society of Cardiology (TSOC) and Taiwan College of Rheumatology (TCR) reviewed the related literature thoroughly and integrated clinical trial evidence and real-world clinical experience for the development of this consensus.
UNASSIGNED: Early detection by regularly screening at-risk patients with incorporations of relevant autoantibodies and biomarkers may lead to better outcomes of CTD-PAH. This consensus proposed specific screening flowcharts for different types of CTDs, the risk assessment tools applicable to the clinical scenario in Taiwan, and a recommendation of medications in the management of CTD-PAH.
UNASSIGNED: This study aimed to obtain a comprehensive understanding and provide recommendations for the management of CTD-PAH in Taiwan, focusing on its clinical importance, prognosis, risk stratification, diagnostic and screening algorithm, and pharmacological treatment.
UNASSIGNED: The members of the Taiwan Society of Cardiology (TSOC) and Taiwan College of Rheumatology (TCR) reviewed the related literature thoroughly and integrated clinical trial evidence and real-world clinical experience for the development of this consensus.
UNASSIGNED: Early detection by regularly screening at-risk patients with incorporations of relevant autoantibodies and biomarkers may lead to better outcomes of CTD-PAH. This consensus proposed specific screening flowcharts for different types of CTDs, the risk assessment tools applicable to the clinical scenario in Taiwan, and a recommendation of medications in the management of CTD-PAH.
摘要：
未经批准：肺动脉高压（PAH），定义为存在平均肺动脉压>20mmHg，肺动脉楔压≤15mmHg，根据专家共识，肺血管阻力(PVR)>2木质单位，其特点是PVR逐渐持续增加，这可能会导致右心衰竭和死亡。PAH是一种众所周知的结缔组织疾病（CTDs）的并发症，比如系统性硬化症，系统性红斑狼疮,干燥综合征,和其他自身免疫性疾病。在过去的几年里,在理解PAH发病机制方面取得了巨大进展，在全球范围内提出了各种新的诊断和筛查方法，用于早期检测PAH。
UNASSIGNED：本研究旨在获得全面的了解，并为台湾CTD-PAH的管理提供建议，专注于它的临床重要性，预后,风险分层，诊断和筛选算法，和药物治疗。
UNASSIGNED：台湾心脏病学会（TSOC）和台湾风湿病学院（TCR）的成员全面回顾了相关文献，并整合了临床试验证据和实际临床经验，以发展这一共识。
UNASSIGNED：通过定期筛查合并有相关自身抗体和生物标志物的高危患者进行早期检测可能会导致CTD-PAH的更好结局。该共识为不同类型的CTD提出了具体的筛选流程图，适用于台湾临床情景的风险评估工具，以及CTD-PAH管理中的药物建议。
UNASSIGNED：本研究旨在获得全面的了解，并为台湾CTD-PAH的管理提供建议，专注于它的临床重要性，预后,风险分层，诊断和筛选算法，和药物治疗。
UNASSIGNED：台湾心脏病学会（TSOC）和台湾风湿病学院（TCR）的成员全面回顾了相关文献，并整合了临床试验证据和实际临床经验，以发展这一共识。
UNASSIGNED：通过定期筛查合并有相关自身抗体和生物标志物的高危患者进行早期检测可能会导致CTD-PAH的更好结局。该共识为不同类型的CTD提出了具体的筛选流程图，适用于台湾临床情景的风险评估工具，以及CTD-PAH管理中的药物建议。
