Dataset Information

PAH-specific therapy for pulmonary hypertension and interstitial lung disease: A systemic review and meta-analysis.

ABSTRACT:

Objective

Pulmonary hypertension (PH) in context with interstitial lung disease (ILD) portends serious clinical consequences and a high rate of mortality. Recently published randomized controlled trials (RCTs) which assessed the pulmonary arterial hypertension (PAH)-specific drugs for pulmonary hypertension and interstitial lung disease (PH-ILD) revealed inconsistent clinical outcomes with previous studies. We conducted a systemic review and meta-analysis to further investigate the effect of PAH-specific therapies for PH-ILD.

Methods

Clinical trials were searched from the EMBASE, PUBMED, and CENTRAL databases. The duration from the establishment of the database to June 2022 for RCTs evaluates the effect of PAH-specific therapy in patients with PH-ILD. RevMan 5.4 was used for the meta-analysis.

Results

A total of six articles (with a total of 791 patients) were included, including 412 patients in the treated group and 379 patients in the control group. As compared to placebo, the change of 6MWD was a significant improvement with PAH-specific therapy in the six RCTs (23.09; 95% CI, 12.07-34.12 P < 0.0001); but when the study with inhaled treprostinil was excluded, the significant improvement in the change of 6MWD from baseline was not present anymore (MD 11.01, 95%CI-6.43-28.46 P = 0.22). There was no significant improvement in the change in lung function, hemodynamic parameters, clinical worsening, all-cause death, and serious adverse effects in the treated group compared to placebo.

Conclusion

PAH-specific therapy significantly improved exercise capacity in the patients with PH-ILD, but this is due to the greater contribution of the study with inhaled treprostinil. Therefore, our findings still did not support the routine use of the whole PAH-specific drugs for PH-ILD.

SUBMITTER: Zhao N

PROVIDER: S-EPMC9713234 | biostudies-literature | 2022

REPOSITORIES: biostudies-literature

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Publications

PAH-specific therapy for pulmonary hypertension and interstitial lung disease: A systemic review and meta-analysis.

Zhao Ning N Chen Jun J Zhang Mingming M Zhou Lihui L Liu Lisong L Yuan Jie J Pang Xingxue X Hu Dayi D Ren Xiaoxia X Jin Zhongyi Z

Frontiers in cardiovascular medicine 20221117

<h4>Objective</h4>Pulmonary hypertension (PH) in context with interstitial lung disease (ILD) portends serious clinical consequences and a high rate of mortality. Recently published randomized controlled trials (RCTs) which assessed the pulmonary arterial hypertension (PAH)-specific drugs for pulmonary hypertension and interstitial lung disease (PH-ILD) revealed inconsistent clinical outcomes with previous studies. We conducted a systemic review and meta-analysis to further investigate the effec ...[more]

PMID: 36465444

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Project description:Pulmonary hypertension (PH) complicating chronic obstructive pulmonary disease (COPD-PH) and interstitial lung disease (ILD-PH) (World Health Organization [WHO] Group III PH) increases medical costs and reduces survival. Despite limited data, many clinicians are using pulmonary arterial hypertension (PAH)-specific therapy to treat WHO Group III PH patients. To further investigate the utility of PAH-specific therapy in WHO Group III PH, we performed a systematic review and meta-analysis. Relevant studies from January 2000 through May 2016 were identified in the MEDLINE, EMBASE, and COCHRANE electronic databases and www.clinicaltrials.gov. Change in six-minute walk distance (6MWD) was estimated using random effects meta-analysis techniques. Five randomized controlled trials (RCTs) in COPD-PH (128 placebo or standard treatment and 129 PAH-medication treated patients), two RCTs in ILD-PH (23 placebo and 46 treated patients), and four single-arm clinical trials (50 patients) in ILD-PH were identified. Treatment in both COPD-PH and ILD-PH did not worsen hypoxemia. Symptomatic burden was not consistently reduced but there were trends for reduced pulmonary artery pressures and pulmonary vascular resistance with PAH-specific therapy. As compared to placebo, 6MWD was not significantly improved with PAH-specific therapy in the five COPD-PH RCTs (42.7 m; 95% confidence interval [CI], -1.0 - 86.3). In the four single-arm studies in ILD-PH patients, there was a significant improvement in 6MWD after PAH-specific treatment (46.2 m; 95% CI, 27.9-64.4), but in the two ILD-PH RCTs there was not an improvement (21.6 m; 95% CI, -17.8 - 61.0) in exercise capacity when compared to placebo. Due to the small numbers of patients evaluated and inconsistent beneficial effects, the utility of PAH-specific therapy in WHO Group III PH remains unproven. A future clinical trial that is appropriately powered is needed to definitively determine the efficacy of this widely implemented treatment approach.

Project description:BackgroundThe aim of the current systematic review was to summarize and evaluate the overall advantages of lung ultrasonography (LUS) examination using high-resolution computed tomography (HRCT) as a reference standard in assessing the presence of interstitial lung disease (ILD) in systemic sclerosis (SSc) patients.MethodsDatabases PubMed, Scopus, and Web of Science were searched for studies evaluating LUSs in ILD assessments including SSc patients on 1 February 2023. In assessing risk of bias and applicability, the Revised Tool for the Quality Assessment of Diagnostic Accuracy Studies (QUADAS-2) was used. A meta-analysis was performed and the mean specificity, sensitivity, and diagnostic odds ratio (DOR) with a 95% confidence interval (CI) were obtained. In addition, in a bivariate meta-analysis, the summary receiver operating characteristic (SROC) curve area was additionally calculated.ResultsNine studies with a total of 888 participants entered the meta-analysis. A meta-analysis was also performed without one study that used pleural irregularity to assess the diagnostic accuracy of LUSs using B-lines (with a total of 868 participants). Overall sensitivity and specificity did not differ significantly, with only the analysis of the B-lines having a specificity of 0.61 (95% CI 0.44-0.85) and a sensitivity of 0.93 (95% CI 0.89-0.98). The diagnostic odds ratio of univariate analysis of the eight studies using the B-lines as a criterion for ILD diagnosis was 45.32 (95% CI 17.88-114.89). The AUC value of the SROC curve was 0.912 (and 0.917 in consideration of all nine studies), which indicates high sensitivity and a low false-positive rate for the majority of the included studies.ConclusionsLUS examination proved to be a valuable tool in discerning which SSc patients should receive additional HRCT scans to detect ILD and therefore reduces the doses of ionizing radiation exposure in SSc patients. However, further studies are needed to achieve consensus in scoring and the evaluation methodology of LUS examination.

Dataset Information

PAH-specific therapy for pulmonary hypertension and interstitial lung disease: A systemic review and meta-analysis.

Objective

Methods

Results

Conclusion

Publications

PAH-specific therapy for pulmonary hypertension and interstitial lung disease: A systemic review and meta-analysis.

Similar Datasets

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