Trial reportAmerican journal of respiratory and critical care medicine2015
Comparison of Treatment Response in Idiopathic and Connective Tissue Disease-associated Pulmonary Arterial Hypertension.
Trial report in American journal of respiratory and critical care medicine, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 68 papers, 5 of them syntheses that pooled it.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
68 citing papers in PubMed, 5 syntheses or guidelines pooled it, 113 citations in OpenAlex.
- Management of pulmonary arterial hypertension in systemic sclerosis: from classical treatments to new horizons.European respiratory review : an official journal of the European Respiratory Society · 2026Pooled it
- Treatment of pulmonary arterial hypertension in patients with connective tissue diseases: a systematic review and meta-analysis.Internal and emergency medicine · 2024Pooled it
- Long-Term Outcomes in Patients With Connective Tissue Disease-Associated Pulmonary Arterial Hypertension in the Modern Treatment Era: Meta-Analyses of Randomized, Controlled Trials and Observational Registries.Arthritis & rheumatology (Hoboken, N.J.) · 2021Pooled it
- Cost Effectiveness of Bosentan for Pulmonary Arterial Hypertension: A Systematic Review.Canadian respiratory journal · 2018Pooled it
- Enhancing Insights into Pulmonary Vascular Disease through a Precision Medicine Approach. A Joint NHLBI-Cardiovascular Medical Research and Education Fund Workshop Report.American journal of respiratory and critical care medicine · 2017Guideline
- Efficacy and safety of ambrisentan in Chinese patients with connective tissue disease-pulmonary arterial hypertension: a post-hoc analysis.BMC cardiovascular disorders · 2020Trial
- Selexipag for the treatment of connective tissue disease-associated pulmonary arterial hypertension.The European respiratory journal · 2017Trial
- Initial combination therapy with ambrisentan and tadalafil in connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH): subgroup analysis from the AMBITION trial.Annals of the rheumatic diseases · 2017Trial
- Riociguat for the treatment of pulmonary arterial hypertension associated with connective tissue disease: results from PATENT-1 and PATENT-2.Annals of the rheumatic diseases · 2017Trial
- The clinical characteristics and risk factors of pulmonary arterial hypertension in primary Sjögren disease: a single-center retrospective study.Clinical rheumatology · 2026Article
- Effectiveness of PAH Specific Treatment Is Comparable Between PAH Associated With Connective Tissue Disease and Idiopathic PAH: Data From the Multicentre National Registry.Pulmonary circulation · 2026Article
- Increased Xanthine Oxidoreductase Activity in Patients With Systemic Sclerosis-Associated Pulmonary Arterial Hypertension.CHEST pulmonary · 2026Article
- Pulmonary Venous Vasculopathy in Connective Tissue Disease-Associated Pulmonary Arterial Hypertension With Reduced Diffusion Capacity for Carbon Monoxide.Pulmonary circulation · 2026Article
- Research advances in connective tissue disease-associated pulmonary arterial hypertension with an emphasis on the Chinese population.Frontiers in medicine · 2026Review
- Plasma GDF-15 and PSP-D Predict the Development of Pulmonary Arterial Hypertension in Systemic Sclerosis.Pulmonary circulation · 2026Article
- Low DLCO Can Provide Insights into Treatment Response in PAH Patients Irrespective of the Reason for Its Decrease.Life (Basel, Switzerland) · 2025Article
- The association between STAT4 polymorphism and pulmonary arterial hypertension in Chinese patients with primary Sjögren's syndrome.Clinical rheumatology · 2025Article
- Lung Single-Cell Transcriptomics Reveal Diverging Pathobiology and Opportunities for Precision Targeting in Scleroderma-Associated Versus Idiopathic Pulmonary Arterial Hypertension.Circulation. Genomic and precision medicine · 2025Article
- Sotatercept for Connective Tissue Disease-Associated Pulmonary Arterial Hypertension with Concomitant Interstitial Lung Disease: Efficacy and Safety Insights.Journal of clinical medicine · 2025Article
- C-C Motif chemokine receptor-2 blockade ameliorates pulmonary hypertension in rats and synergizes with a pulmonary vasodilator.Cardiovascular research · 2025Article
8 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
6 authors at 2 institutions in 1 country.
Funding
Abstract
rationaleStudies suggest that patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) have a poorer treatment response to therapies for PAH compared with patients with idiopathic PAH (IPAH), but individual randomized controlled trials (RCTs) have been underpowered to examine differences within these subgroups.
objectivesTo compare the effect of therapy for PAH in CTD-PAH versus IPAH.
methodsWe obtained individual participant data from phase III placebo-controlled RCTs of therapies for PAH submitted to the U.S. Food and Drug Administration for drug approval. A treatment-by-diagnosis interaction term evaluated differences in treatment response between CTD-PAH and IPAH. Outcomes included change in 6-minute-walk distance (∆6MWD) from baseline to 12 weeks, clinical worsening, and all-cause mortality. MEASUREMENTS AND MAIN
resultsThe study sample included 827 participants with CTD-PAH and 1,935 with IPAH from 11 RCTs. Patients with CTD-PAH had less improvement in 6MWD when assigned to active treatment versus placebo compared with patients with IPAH (difference in treatment effect on ∆6MWD in CTD-PAH vs. IPAH, -17.3 m; 90% confidence interval, -31.3 to -3.3; P for interaction = 0.043). Treatment was less effective in reducing the occurrence of clinical worsening in CTD-PAH versus IPAH (P for interaction = 0.012), but there was no difference in the placebo-adjusted effect of treatment on mortality (P for interaction = 0.65).
conclusionsTreatment for PAH was less effective in CTD-PAH compared with IPAH in terms of increasing 6MWD and preventing clinical worsening. The heterogeneity of treatment response supports the need for identifying therapies that are more effective for CTD-PAH.
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Identifiers
What OpenQuestion holds
Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.