ArticleRheumatology (Oxford, England)2023
10-year survival of pulmonary arterial hypertension associated with connective tissue disease: insights from a multicentre PAH registry.
Article in Rheumatology (Oxford, England), 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.
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Who cites it
16 citing papers in PubMed, 20 citations in OpenAlex.
- Vascular compliance phenotyping in pulmonary arterial hypertension (a PVDOMICS study).JHLT open · 2026Article
- Belimumab as a possible treatment for SLE-associated pulmonary arterial hypertension: the experience from a single centre.Lupus science & medicine · 2026Article
- Connective Tissue Disease-Associated Pulmonary Arterial Hypertension: Current Therapeutic Strategies and Future Prospects.Biomolecules · 2026Review
- Research advances in connective tissue disease-associated pulmonary arterial hypertension with an emphasis on the Chinese population.Frontiers in medicine · 2026Review
- Cardiac MRI-derived ventricular-pulmonary arterial coupling predicts outcomes in connective tissue disease-associated pulmonary arterial hypertension patients.European journal of radiology open · 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
- Cluster analysis reveals three clinical phenotypes of pulmonary artery hypertension associated with connective tissue diseases: insights into inflammation and immunity.Arthritis research & therapy · 2025Article
- The prognostic value of HFA-PEFF score in connective tissue disease-associated PAH: evidence from a cohort study.BMC cardiovascular disorders · 2025Observational
- Trends in pulmonary arterial hypertension: insights from Global Burden of Disease 1990-2021.BMJ open · 2025Article
- Impact of sodium-glucose cotransporter-2 inhibitors on pulmonary vascular cell function and arterial remodeling.World journal of cardiology · 2025Review
- Relationships Among the EmPHasis-10 Questionnaire, the Simplified Four-Strata Risk Assessment Tool, and Echocardiographic Parameters in Patients with Precapillary Pulmonary Hypertension.Journal of clinical medicine · 2024Article
- Pathophysiology of the right ventricle and its pulmonary vascular interaction.The European respiratory journal · 2024Review
- Can Combination Therapy with Endothelin Receptor Antagonist and PDE5 Inhibitors Prevent Echocardiographic Findings Suspicious for Pulmonary Arterial Hypertension? Description of a Real-Life Case Series.Diagnostics (Basel, Switzerland) · 2024Article
- Pulmonary hypertension.Nature reviews. Disease primers · 2024Review
- CTD-PAH: an updated practical approach to screening, diagnosis, and management.Therapeutic advances in respiratory diseaseReview
Corrections and comments
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Authors and funding
15 authors at 7 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectivesTo report the 10-year survival rate and prognostic factors of pulmonary arterial hypertension associated with CTD (CTD-PAH) patients, to compare treatment and survival between patients enrolled before and after 2015, and to validate the discrimination of the recommended four-strata model in predicting 10-year survival at follow-up in Chinese CTD-PAH patients.
methodsThis study was derived from a Chinese national multicentre prospective registry study from 2009 to 2019. Medical records were collected at baseline and follow-up, including PAH-targeted therapy and binary therapy (both CTD and PAH-targeted therapy).
resultsA total of 266 CTD-PAH patients were enrolled and the 10-year survival rate was 59.9% (median follow-up time: 4.85 years). Underlying CTD (SSc), baseline 6-min walking distance and SaO2 were independent risk factors for 10-year survival. The proportion of patients receiving PAH-targeted combination therapy increased from 10.1% (2009-2014) to 26.5% (2015-2019) and that of binary therapy increased from 14.8% to 35%. The 1-year survival rate increased from 89.8% (2009-2014) to 93.9%, and the 3-year survival rate increased from 80.1% (2009-2014) to 86.5% (both P > 0.05). The four-strata strategy performed well in predicting 10-year survival at follow-up (C-index = 0.742).
conclusionThe 10-year survival rate of CTD-PAH patients was reported for the first time. The 10-year prognosis was poor, but there was a tendency for more standardized treatment and better survival in patients enrolled after 2015. The recommended four-strata model at follow-up can effectively predict 10-year survival in CTD-PAH patients.
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