SynthesisArthritis & rheumatology (Hoboken, N.J.)2021
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.
Synthesis in Arthritis & rheumatology (Hoboken, N.J.), 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 36 papers, 2 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
36 citing papers in PubMed, 2 syntheses or guidelines pooled it, 58 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
- 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
- Trends and future projections of the disease burden of pulmonary arterial Hypertension-related heart failure in China and G20 countries: Based on the global burden of disease 2023 database.International journal of cardiology. Cardiovascular risk and prevention · 2026Article
- Treatment of systemic sclerosis-associated pulmonary hypertension with and without interstitial lung disease and its impact on survival: a large cohort study of the German network for systemic sclerosis.Rheumatology (Oxford, England) · 2026Article
- Real-World Experience with Selexipag in Patients with Pulmonary Arterial Hypertension: Treatment Patterns and Outcomes across Baseline Risk Strata (SPHERE and EXPOSURE Studies).Drugs - real world outcomes · 2026Article
- Defining quality standards of care in CTD-PAH and management best practices: a Delphi panel consensus.Rheumatology advances in practice · 2026Article
- Reevaluating risk assessment in connective tissue disease-associated pulmonary arterial hypertension: The prognostic superiority of stroke volume index.Rheumatology and immunology research · 2025Article
- Current landscape for connective tissue disease associated-pulmonary arterial hypertension: a focus on right ventricular adaptation and failure.Future cardiology · 2025Review
- Tadalafil plus endothelin receptor antagonists in connective tissue disease-associated pulmonary arterial hypertension: A multicenter study on exercise capacity and cardiac outcomes.Rheumatology and immunology research · 2025Article
- A comparative analysis of risk stratification tools in SSc-associated pulmonary arterial hypertension: a EUSTAR analysis.Rheumatology (Oxford, England) · 2025Article
- Molecular Pathogenesis of Connective Tissue Disease-Associated Pulmonary Arterial Hypertension: A Narrative Review.Biomolecules · 2025Review
- Impact of treatment escalation on rehospitalization among patients with pulmonary arterial hypertension.Scientific reports · 2025Article
- Economic burden of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) in Finland.International journal of cardiology. Heart & vasculature · 2024Article
- Treatment algorithm for pulmonary arterial hypertension.The European respiratory journal · 2024Review
- Definition, classification and diagnosis of pulmonary hypertension.The European respiratory journal · 2024Article
- Towards a Better Prognosis in Patients with Systemic Sclerosis-Related Pulmonary Arterial Hypertension: Recent Developments and Perspectives.Journal of clinical medicine · 2024Review
- Macitentan in Pulmonary Arterial Hypertension Associated with Connective Tissue Disease (CTD-PAH): Real-World Evidence from the Combined OPUS/OrPHeUS Dataset.Cardiology and therapy · 2024Article
- Systemic Sclerosis-Associated Pulmonary Arterial Hypertension: From Bedside to Bench and Back Again.International journal of molecular sciences · 2024Review
Corrections and comments
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Authors and funding
9 authors at 6 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectiveData on the magnitude of benefit of modern therapies for pulmonary arterial hypertension (PAH) in connective tissue disease (CTD)-associated PAH are limited. In this study, we performed meta-analyses of randomized, controlled trials (RCTs) and registries to quantify the benefit of these modern therapies in patients with CTD-PAH.
methodsThe PubMed and Embase databases were searched for articles reporting data from RCTs or registries published between January 1, 2000 and November 25, 2019. Eligibility criteria included multicenter studies with ≥30 CTD-PAH patients. For an RCT to be included, the trial had to evaluate an approved PAH therapy, and long-term risks of clinical morbidity and mortality or 6-minute walk distance had to be reported. For a registry to be included, survival rates had to be reported. Random-effects models were used to pool the data.
resultsEleven RCTs (total of 4,329 patients; 1,267 with CTD-PAH) and 19 registries (total of 9,739 patients; 4,008 with CTD-PAH) were included. Investigational therapy resulted in a 36% reduction in the risk of clinical morbidity/mortality events both in the overall PAH population (hazard ratio [HR] 0.64, 95% confidence interval [95% CI] 0.54, 0.75; P < 0.001) and in CTD-PAH patients (HR 0.64, 95% CI 0.51, 0.81; P < 0.001) as compared to control subjects. The survival rate was lower in CTD-PAH patients compared to all PAH patients (survival rate 62%, 95% CI 57, 67% versus 72%, 95% CI 69, 75% at 3 years). The survival rate in CTD-PAH patients treated primarily after 2010 was higher than that in CTD-PAH patients treated before 2010 (survival rate 73%, 95% CI 62, 81% versus 65%, 95% CI 59, 71% at 3 years).
conclusionModern therapy provides a similar reduction in morbidity/mortality risk in patients with CTD-PAH when compared to the PAH population overall. Risk of death is higher in CTD-PAH patients than in those with PAH overall, but survival has improved in the last 10 years, which may be related to increased screening and/or new treatment approaches. Early detection of PAH in patients with CTD and up-front intensive treatment are warranted.
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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.