Abstract：
OBJECTIVE: Pulmonary arterial hypertension (PAH) is a progressive disease with a poor prognosis, and its management should be grounded in well-developed clinical practice guidelines (CPG). Thus, we critically assess the methodological quality of the available CPG for pharmacological treatments for PAH.
METHODS: A systematic review (CRD42023387168) was performed in PubMed, Cochrane, Embase, and Tripdatabase (Jan-2023). Eligible records were appraised by four reviewers using the Appraisal of Guidelines, Research, and Evaluation Collaboration tool (AGREE II) and the complementary tool for assessing recommendations\' quality and certainty, AGREE REX. Descriptive statistics were used to summarize the data.
RESULTS: Overall, 31 guidelines, mainly authored by professional societies (90%), targeting only physicians as primary users (84%), were identified. Guidelines presented a moderate overall quality (scores of 63% and 51% in AGREE II and AGREE REX, respectively), with a few domains showing slight improvements over the years. AGREE II \"Scope and Purpose\" (94%) and \"Presentation Clarity\" (99%) domains obtained the highest scores. The items related to \"Stakeholder involvement,\" \"Editorial independence,\" and \"Clinical applicability\" (AGREE REX) were fairly reported. Conversely, CPG lacks rigor in development (32% score, AGREE II), scarcely discusses the role of stakeholders, and provides deficient data on the implementation of recommendations (scores of 35% and 46% in AGREE II and AGREE REX, respectively). No differences in the quality of guidelines published by different developers or countries were observed (p > 0.05).
CONCLUSIONS: Methodological weaknesses are common among guidelines addressing PAH treatment, especially regarding scientific rigor, stakeholders\' values and preferences, and facilitators and barriers to implementability. Particular attention should be given to developing future guidelines.
METHODS: A systematic review (CRD42023387168) was performed in PubMed, Cochrane, Embase, and Tripdatabase (Jan-2023). Eligible records were appraised by four reviewers using the Appraisal of Guidelines, Research, and Evaluation Collaboration tool (AGREE II) and the complementary tool for assessing recommendations\' quality and certainty, AGREE REX. Descriptive statistics were used to summarize the data.
RESULTS: Overall, 31 guidelines, mainly authored by professional societies (90%), targeting only physicians as primary users (84%), were identified. Guidelines presented a moderate overall quality (scores of 63% and 51% in AGREE II and AGREE REX, respectively), with a few domains showing slight improvements over the years. AGREE II \"Scope and Purpose\" (94%) and \"Presentation Clarity\" (99%) domains obtained the highest scores. The items related to \"Stakeholder involvement,\" \"Editorial independence,\" and \"Clinical applicability\" (AGREE REX) were fairly reported. Conversely, CPG lacks rigor in development (32% score, AGREE II), scarcely discusses the role of stakeholders, and provides deficient data on the implementation of recommendations (scores of 35% and 46% in AGREE II and AGREE REX, respectively). No differences in the quality of guidelines published by different developers or countries were observed (p > 0.05).
CONCLUSIONS: Methodological weaknesses are common among guidelines addressing PAH treatment, especially regarding scientific rigor, stakeholders\' values and preferences, and facilitators and barriers to implementability. Particular attention should be given to developing future guidelines.
摘要：
目的：肺动脉高压（PAH）是一种预后不良的进行性疾病，其管理应基于完善的临床实践指南（CPG）。因此，我们严格评估现有CPG用于PAH药物治疗的方法学质量.
方法：在PubMed，科克伦,Embase,和Tripdatabase（2023年1月）。合格记录由四名审查人员使用《评估指南》进行评估，Research,和评估协作工具(AGREEII)以及评估建议质量和确定性的补充工具，同意雷克斯。使用描述性统计来总结数据。
结果：总体而言，31条准则，主要由专业协会撰写(90%)，仅将医生作为主要用户（84%），已确定。指南提出了中等的总体质量(AGREEII和AGREEREX得分分别为63%和51%，分别)，几年来，一些领域显示出轻微的改进。AGREEII“范围和目的”（94％）和“演示文稿清晰度”（99％）领域得分最高。与“利益相关者参与”相关的项目，\"\"编辑独立,“”和“临床适用性”(AGREEREX)报告相当。相反，CPG在发展中缺乏严谨性(32%得分，AGREEII),很少讨论利益相关者的作用，并提供了有关建议执行情况的不足数据（AGREEII和AGREEREX的得分分别为35％和46％，分别)。没有观察到不同开发者或国家发布的指南的质量差异(p>0.05)。
结论：在处理PAH治疗的指南中，方法学上的弱点很常见，特别是关于科学的严谨性，利益相关者的价值观和偏好，和促进者和可实施性的障碍。应特别注意制定未来的准则。
方法：在PubMed，科克伦,Embase,和Tripdatabase（2023年1月）。合格记录由四名审查人员使用《评估指南》进行评估，Research,和评估协作工具(AGREEII)以及评估建议质量和确定性的补充工具，同意雷克斯。使用描述性统计来总结数据。
结果：总体而言，31条准则，主要由专业协会撰写(90%)，仅将医生作为主要用户（84%），已确定。指南提出了中等的总体质量(AGREEII和AGREEREX得分分别为63%和51%，分别)，几年来，一些领域显示出轻微的改进。AGREEII“范围和目的”（94％）和“演示文稿清晰度”（99％）领域得分最高。与“利益相关者参与”相关的项目，\"\"编辑独立,“”和“临床适用性”(AGREEREX)报告相当。相反，CPG在发展中缺乏严谨性(32%得分，AGREEII),很少讨论利益相关者的作用，并提供了有关建议执行情况的不足数据（AGREEII和AGREEREX的得分分别为35％和46％，分别)。没有观察到不同开发者或国家发布的指南的质量差异(p>0.05)。
结论：在处理PAH治疗的指南中，方法学上的弱点很常见，特别是关于科学的严谨性，利益相关者的价值观和偏好，和促进者和可实施性的障碍。应特别注意制定未来的准则。
