Article type
Abstract
"Background: Pulmonary arterial hypertension (PAH) is a rare, progressive disease with a poor prognosis, affecting around five cases per population of 1 million per year. Its management should be grounded on well-developed clinical practice guidelines (CPG). Considering the increased number and recently updated guidelines addressing PAH treatment, a more comprehensive evaluation of their quality and generalizability is needed to identify potential weaknesses and strengths that may guide the development and improvement of future documents and practical frameworks for managing this rare disease.
Objectives: We critically assess the methodological quality of available CPG on 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 on recommendations' quality and certainty, AGREE-REX. Both instruments were applied following the original user manual, and descriptive statistics were used to summarize data (SPSS v.28).
Results: Overall, 31 guidelines, mainly authored by professional societies (90%) and targeting only physicians as primary users (84%) were identified. Only 8 (26%) guidelines reported following GRADE or AGREE systems. Guidelines presented a moderate overall quality (scores of 63% - AGREE II and 51% - AGREE-REX, respectively), with a few domains showing slight improvements over the years. The highest scores were identified to AGREE II ' Scope and Purpose' (94%) and 'Presentation Clarity' (99%) domains. The items related to 'Stakeholder involvement’,' 'Editorial independence’,' and 'Clinical applicability' (AGREE-REX) were fairly reported. Conversely, CPG lacks rigor of development (32% score on AGREE II), scarcely discusses the role of stakeholders, and provides deficient data on recommendations implementability process (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), as well as no relationship between the quality of guidelines and PAH target therapy recommendations.
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."
Objectives: We critically assess the methodological quality of available CPG on 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 on recommendations' quality and certainty, AGREE-REX. Both instruments were applied following the original user manual, and descriptive statistics were used to summarize data (SPSS v.28).
Results: Overall, 31 guidelines, mainly authored by professional societies (90%) and targeting only physicians as primary users (84%) were identified. Only 8 (26%) guidelines reported following GRADE or AGREE systems. Guidelines presented a moderate overall quality (scores of 63% - AGREE II and 51% - AGREE-REX, respectively), with a few domains showing slight improvements over the years. The highest scores were identified to AGREE II ' Scope and Purpose' (94%) and 'Presentation Clarity' (99%) domains. The items related to 'Stakeholder involvement’,' 'Editorial independence’,' and 'Clinical applicability' (AGREE-REX) were fairly reported. Conversely, CPG lacks rigor of development (32% score on AGREE II), scarcely discusses the role of stakeholders, and provides deficient data on recommendations implementability process (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), as well as no relationship between the quality of guidelines and PAH target therapy recommendations.
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."