ArticleEuropean respiratory review : an official journal of the European Respiratory Society2019
Screening for pulmonary arterial hypertension in systemic sclerosis.
Article in European respiratory review : an official journal of the European Respiratory Society, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 45 papers, 2 of them syntheses that pooled it.
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The trial behind it
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Who cites it
45 citing papers in PubMed, 2 syntheses or guidelines pooled it, 96 citations in OpenAlex.
- Screening for Pulmonary Hypertension in Connective Tissue Diseases: Literature Review and Multidisciplinary Consensus Statement.Anatolian journal of cardiology · 2025Pooled 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
- Article
- Connective Tissue Disease-Associated Pulmonary Arterial Hypertension: Current Therapeutic Strategies and Future Prospects.Biomolecules · 2026Review
- A matter arising: When should inflammatory and autoimmune rheumatic diseases be considered 'early'?European journal of clinical investigation · 2026Review
- Article
- Bridging knowledge gaps: A case report of pulmonary Veno-occlusive disease in systemic sclerosis.Lung India : official organ of Indian Chest Society · 2025Article
- Targeting type-2 inflammation in pulmonary hypertension: lessons from a novel model.American journal of physiology. Lung cellular and molecular physiology · 2025Article
- How to Assess Pulmonary Circulation and Right Heart Chambers in Systemic Sclerosis Patients?Diagnostics (Basel, Switzerland) · 2025Review
- Prevalence, molecular mechanisms and diagnostic approaches to pulmonary arterial hypertension in connective tissue diseases.Rheumatology international · 2025Review
- Prognostic Value of Echocardiographic Coupling Metrics in Systemic Sclerosis-Associated Pulmonary Vascular Disease.Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography · 2025Article
- Predictive Value of the DETECT Algorithm for Pulmonary Arterial Hypertension in Systemic Sclerosis: Findings from an Italian Observational Study.Journal of clinical medicine · 2025Article
- N-terminal pro-brain natriuretic peptide is associated with pulmonary hypertension or diastolic dysfunction in patients with systemic sclerosis: An Australian prospective cross-sectional study.Journal of scleroderma and related disorders · 2024Article
- Towards a Better Prognosis in Patients with Systemic Sclerosis-Related Pulmonary Arterial Hypertension: Recent Developments and Perspectives.Journal of clinical medicine · 2024Review
- Managing Symptoms of Systemic Sclerosis for the Allergist-Immunologist.Current allergy and asthma reports · 2024Review
- The Management of Mild Pulmonary Hypertension in Clinical Practice.Annals of the American Thoracic Society · 2024Review
- Aberrant long-chain fatty acid metabolism associated with evolving systemic sclerosis-associated pulmonary arterial hypertension.American journal of physiology. Lung cellular and molecular physiology · 2024Observational
- Low-Carbon Monoxide Diffusing Capacity, Patient-Reported Measures and Reduced Nailfold Capillary Density Are Associated with Interstitial Lung Disease in Systemic Sclerosis.Journal of personalized medicine · 2024Article
- Exercise pulmonary hypertension in patients with systemic sclerosis based on updated guidelines.Scientific reports · 2024Article
- Attitudes and barriers to pulmonary arterial hypertension screening in systemic sclerosis patients: A survey of UK-based rheumatologists.Journal of scleroderma and related disorders · 2024Article
Corrections and comments
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Authors and funding
6 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Pulmonary arterial hypertension (PAH) is a dreaded complication of systemic sclerosis (SSc) that occurs in ∼10% of patients. Most individuals present with severe symptoms, significant functional impairment and severe haemodynamics at diagnosis, and survival after PAH diagnosis is poor. Therefore, early diagnosis through systematic screening of asymptomatic patients has the potential to identify PAH at an early stage. Current evidence suggests that early diagnosis and treatment of PAH in patients with SSc may lead to better clinical outcomes. Annual screening may include echocardiography, but this can miss some patients due to suboptimal visualisation or insufficient tricuspid regurgitation. Other options for screening include the DETECT algorithm or the use of a combination of pulmonary function testing (forced vital capacity/diffusing capacity of the lung for carbon monoxide ratio) and N-terminal-pro-brain natriuretic peptide levels. Symptomatic patients, those with an elevated tricuspid regurgitation velocity on echocardiogram with or without secondary echocardiographic features of PAH, and those who screen positive on the DETECT or other pulmonary function test algorithms should undergo right heart catheterisation. Exercise echocardiography or cardiopulmonary exercise testing, nailfold capillaroscopy and molecular biomarkers are promising but, as yet, unproven potential options. Future screening studies should employ systematic catheterisation to define the true predictive values for PAH.
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Registered trials
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