TY - JOUR
T1 - A comparative analysis of risk stratification tools in SSc-associated pulmonary arterial hypertension
T2 - a EUSTAR analysis
AU - EUSTAR Collaborators
AU - Bjørkekjær, Hilde Jenssen
AU - Bruni, Cosimo
AU - Broch, Kaspar
AU - Brunborg, Cathrine
AU - Carreira, Patricia E.
AU - Airò, Paolo
AU - Simeón-Aznar, Carmen Pilar
AU - Truchetet, Marie Elise
AU - Giollo, Alessandro
AU - Balbir-Gurman, Alexandra
AU - Martin, Mickael
AU - Denton, Christopher P.
AU - Gabrielli, Armando
AU - Del Galdo, Francesco
AU - Vonk, Madelon C.
AU - Fretheim, Håvard
AU - Bitter, Helle
AU - Midtvedt, Øyvind
AU - Andreassen, Arne
AU - Høie, Sverre
AU - Tanaka, Yoshiya
AU - Riemekasten, Gabriela
AU - Müller-Ladner, Ulf
AU - Matucci-Cerinic, Marco
AU - Castellví, Ivan
AU - Siegert, Elise
AU - Hachulla, Eric
AU - Molberg, Øyvind
AU - Distler, Oliver
AU - Hoffmann-Vold, Anna Maria
AU - Guiducci, Serena
AU - Iannone, Florenzo
AU - Rednic, Simona
AU - Allanore, Yannick
AU - Montecucco, Carlomaurizio
AU - Kumánovics, Gábor
AU - Iudici, Michele
AU - Moroncini, Gianluca
AU - Andréasson, Kristofer
AU - Idolazzi, Luca
AU - Henes, Jörg
AU - Pflugfelder, Johannes
AU - Da Silva, José António Pereira
AU - Hughes, Michael
AU - Riccieri, Valeria
AU - Balanescu, Andra
AU - Gheorghiu, Ana Maria
AU - Bergmann, Christina
AU - Cantatore, Francesco Paolo
AU - De Langhe, Ellen
N1 - Publisher Copyright:
© 2025 The Author(s). Published by Oxford University Press on behalf of the British Society for Rheumatology.
PY - 2025/6/1
Y1 - 2025/6/1
N2 - 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 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 11 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 diffusing capacity of the lung for carbon monoxide (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.
AB - 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 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 11 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 diffusing capacity of the lung for carbon monoxide (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.
UR - http://www.scopus.com/inward/record.url?scp=105006718953&partnerID=8YFLogxK
UR - https://www.mendeley.com/catalogue/faa00aa8-7f3f-3f81-a6d8-3814896ba0e8/
U2 - 10.1093/rheumatology/keaf053
DO - 10.1093/rheumatology/keaf053
M3 - Journal articles
C2 - 39878955
AN - SCOPUS:105006718953
SN - 1462-0324
VL - 64
SP - 3643
EP - 3656
JO - Rheumatology
JF - Rheumatology
IS - 6
ER -