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Updated: Dec 18, 2025
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Screening for pulmonary arterial hypertension in systemic sclerosis: A systematic literature review
Cosimo Bruni1, Giacomo De Luca2, Maria-Grazia Lazzaroni3
1Dept. Experimental and Clinical Medicine, Division of Rheumatology, University of Florence, Viale Pieraccini 18, Florence, 50139, Italy.
Insights
Early screening for pulmonary arterial hypertension (PAH) in Systemic Sclerosis (SSc) is crucial. Echocardiography, particularly systolic pulmonary arterial pressure and tricuspid regurgitation velocity, is the leading tool for PAH screening in SSc patients.
Area of Science:
- Rheumatology
- Cardiology
- Pulmonology
Background:
- Systemic Sclerosis (SSc) is associated with significant morbidity and mortality due to Pulmonary Arterial Hypertension (PAH).
- Early detection and screening for PAH in SSc patients are critical for improving outcomes.
- Right heart catheterization serves as the gold standard for PAH diagnosis.
Purpose of the Study:
- To conduct a systematic literature review to identify and evaluate screening tools and modalities for SSc-PAH.
- To assess the performance of various screening methods against the gold standard diagnostic procedure.
Main Methods:
- Systematic literature review including papers from previous SLRs and a new systematic search (PubMed, EMBASE, Web of Science) from 03/10/2017 to 31/12/2018.
- Inclusion of 199 reviewed papers, with 32 extracted for analysis, assessed for bias risk using QUADAS-2.
- Focus on echocardiography, pulmonary function tests, clinical features, and serum biomarkers as screening tools.
Main Results:
- Echocardiography, pulmonary function tests, clinical features, and serum biomarkers were frequently used screening tools.
- Composite algorithms like DETECT score, ESC/ERS guidelines, ASIG, and ITINER-air were commonly employed.
- DETECT and ASIG demonstrated higher sensitivity and negative predictive value compared to ESC/ERS 2009 guidelines.
Conclusions:
- Echocardiography is identified as the primary screening tool for SSc-PAH.
- Systolic pulmonary arterial pressure (sPAP) and tricuspid regurgitation velocity (TRV) are key parameters, used individually or in composite algorithms.
- Effective screening involves a combination of echocardiographic, biomarker, clinical, and functional assessments.
Abstract:
Pulmonary arterial hypertension (PAH) carries a high morbidity and mortality burden in Systemic Sclerosis (SSc). Therefore, PAH screening and early detection are pivotal. A systematic literature review (SLR) to search for all screening tools and modalities for SSc-PAH was performed in reference to right heart catheterization as diagnostic gold standard. Papers from 2 previously published SLRs and derived from a systematic search on Pubmed, EMBASE, Web of Science for papers published from 03/10/2017 to 31/12/2018 were manually included. A total of 199 papers were reviewed and 32 were extracted, with a low bias risk according to QUADAS2. Echocardiography, pulmonary function tests, clinical features and serum biomarkers were the most frequently tools used for screening, with different parameters combined in a variable fashion, as single item or as part of composite algorithms. Among the composite algorithms, the DETECT score, ESC/ERS 2009 or 2015 guidelines, ASIG and ITINER-air algorithms were the most commonly used in a wide range of patients. In different cohorts, DETECT and ASIG showed higher sensitivity and negative predictive value than ESC/ERS 2009. In conclusion, the literature shows echocardiography as the leading screening tool for SSc-PAH. In particular, systolic pulmonary arterial pressure (sPAP) and tricuspid regurgitation velocity (TRV), both as single items or part of composite algorithms, including also serum biomarkers, clinical and functional items, are the most frequent parameters evaluated.
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