ArticleRheumatology (Oxford, England)2024
Treatment strategies and survival of patients with connective tissue disease and pulmonary arterial hypertension: a COMPERA analysis.
Article in Rheumatology (Oxford, England), 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT01347216 (Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension), which is not on this map. Cited by 27 papers, 1 of them a synthesis that pooled it.
What it found
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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.
Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension
Who cites it
27 citing papers in PubMed, 1 synthesis or guideline pooled it, 39 citations in OpenAlex.
- Management of inflammatory cardiopulmonary manifestations in systemic lupus erythematosus: a systematic review.Advances in rheumatology (London, England) · 2026Pooled it
- Cause-specific outcomes in systemic sclerosis-associated, systemic lupus erythematosus-associated, and idiopathic pulmonary hypertension with significant pre-capillary components: a competing risk analysis.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
- 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
- Treatment of Pulmonary Arterial Hypertension in Lithuania: Current Situation and Analysis of Survival of Patients Treated with Different Treatment Regimens.Journal of clinical medicine · 2026Article
- Clinical, serological and haemodynamic factors associated with poor outcomes in systemic lupus erythematosus-associated pulmonary arterial hypertension: a multicentre retrospective study from Korea.Rheumatology (Oxford, England) · 2026Article
- Article
- Review
- Connective Tissue Disease-Associated Pulmonary Arterial Hypertension: Current Therapeutic Strategies and Future Prospects.Biomolecules · 2026Review
- Time to Therapy Activation and Initial Combination Therapy (TITANIC) for Patients With Pulmonary Arterial Hypertension.Pulmonary circulation · 2026Article
- Implications of systolic pulmonary artery pressure trajectories in systemic lupus erythematosus-associated pulmonary hypertension: a CSTAR-PAH cohort study.Frontiers in immunology · 2026Article
- Refining Risk Prediction in Systemic Sclerosis-Associated Pulmonary Arterial Hypertension.Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography · 2025Article
- Association of exercise-induced pulmonary hypertension with nailfold capillary density in systemic sclerosis: a single-center retrospective observational study.Arthritis research & therapy · 2025Observational
- Insights into Pulmonary Arterial Hypertension in Connective Tissue Diseases.Journal of clinical medicine · 2025Review
- Right-sided heart failure in acute respiratory distress syndrome.European respiratory review : an official journal of the European Respiratory Society · 2025Review
- 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
- Prevalence, molecular mechanisms and diagnostic approaches to pulmonary arterial hypertension in connective tissue diseases.Rheumatology international · 2025Review
- Impact of Comorbidities on Treatments and Outcomes of Systemic Sclerosis-Associated Pulmonary Arterial Hypertension.Canadian respiratory journal · 2025Article
- Endothelin-1 as a Candidate Biomarker of Systemic Sclerosis: A GRADE-Assessed Systematic Review and Meta-Analysis With Meta-Regression.Biomarker insights · 2025Article
Corrections and comments
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Authors and funding
36 authors at 20 institutions in 9 countries.
Funding
Abstract
objectivesPulmonary arterial hypertension (PAH) occurs in various connective tissue diseases (CTDs). We sought to assess contemporary treatment patterns and survival of patients with various forms of CTD-PAH.
methodsWe analysed data from COMPERA, a European pulmonary hypertension registry, to describe treatment strategies and survival in patients with newly diagnosed PAH associated with SSc, SLE, MCTD, UCTD and other types of CTD. All-cause mortality was analysed according to the underlying CTD. For patients with SSc-PAH, we also assessed survival according to initial therapy with endothelin receptor antagonists (ERAs), phosphodiesterase type 5 inhibitors (PDE5is) or a combination of these two drug classes.
resultsThis analysis included 607 patients with CTD-PAH. Survival estimates at 1, 3 and 5 years for SSc-PAH (n = 390) were 85%, 59% and 42%; for SLE-PAH (n = 34) they were 97%, 77% and 61%; for MCTD-PAH (n = 33) they were 97%, 70% and 59%; for UCTD-PAH (n = 60) they were 88%, 67% and 52%; and for other CTD-PAH (n = 90) they were 92%, 69% and 55%, respectively. After multivariable adjustment, the survival of patients with SSc-PAH was significantly worse compared with the other conditions (P = 0.001). In these patients, the survival estimates were significantly better with initial ERA-PDE5i combination therapy than with initial ERA or PDE5i monotherapy (P = 0.016 and P = 0.012, respectively).
conclusionsMortality remains high in patients with CTD-PAH, especially for patients with SSc-PAH. However, for patients with SSc-PAH, our results suggest that long-term survival may be improved with initial ERA-PDE5i combination therapy compared with initial monotherapy.
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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.