Bjørkekjær, Hilde Jenssen;
Bruni, Cosimo;
Broch, Kaspar;
Brunborg, Cathrine;
Carreira, Patricia E;
Airò, Paolo;
Simeón-Aznar, Carmen Pilar;
... EUSTAR, collaborators; + view all
(2025)
A comparative analysis of risk stratification tools in systemic sclerosis-associated pulmonary arterial hypertension: a EUSTAR analysis.
Rheumatology
10.1093/rheumatology/keaf053.
(In press).
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Abstract
OBJECTIVES: The 2022 European Society of Cardiology and European Respiratory Society (ESC/ERS) Guidelines for pulmonary arterial hypertension (PAH) recommend risk stratification to optimize management. However, the performance of generic PAH risk stratification tools in patients with systemic sclerosis (SSc)-associated PAH remains unclear. Our objective was to identify the most accurate approach for risk stratification at SSc-PAH diagnosis. METHODS: In this multicentre, international cohort study from the European Scleroderma Trials and Research (EUSTAR) group database, we screened eleven risk stratification tools upon SSc-PAH diagnosis. We compared the performance of the three top-ranked tools to predict mortality with the ESC/ERS three-strata model, the currently recommended tool for baseline risk assessment. We also assessed the impact of incorporating SSc-specific characteristics into the tools. Kaplan-Meier analyses and Cox regression with area under the ROC curve (AUC) were conducted. RESULTS: The ESC/ERS three-strata model had a lower ability to predict mortality than the ESC/ERS four-strata model, "SPAHR updated", and "REVEAL Lite 2". The ESC/ERS four-strata model divided "intermediate-risk" patients into two groups with significantly different long-term survival rates and is the easiest applicable tool. Incorporating SSc-specific characteristics did not significantly improve the predictive ability of any model, but a low DLCO was an independent predictor of mortality. CONCLUSION: Considering its ability to predict mortality, risk segregation capabilities, and clinical applicability, this study provides a rationale for using the simplified ESC/ERS four-strata model at SSc-PAH diagnosis as an alternative to the comprehensive ESC/ERS three-strata model. We propose considering DLCO as an individual prognostic marker in SSc-PAH.
| Type: | Article |
|---|---|
| Title: | A comparative analysis of risk stratification tools in systemic sclerosis-associated pulmonary arterial hypertension: a EUSTAR analysis |
| Location: | England |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.1093/rheumatology/keaf053 |
| Publisher version: | https://doi.org/10.1093/rheumatology/keaf053 |
| Language: | English |
| Additional information: | © The Author(s) 2025. Published by Oxford University Press on behalf of the British Society for Rheumatology. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by/4.0/). |
| Keywords: | Observational Study, Pulmonary Arterial Hypertension, Risk Stratification, Systemic Sclerosis |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Inflammation |
| URI: | https://discovery.ucl.ac.uk/id/eprint/10204445 |
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