A comparative analysis of risk stratification tools in SSc-associated pulmonary arterial hypertension

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...

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Detalles Bibliográficos
Autores: Bjørkekjær, H.J., Bruni, C.|||0000-0003-2813-2083, Broch, K.|||0000-0003-4316-805X, Brunborg, C.|||0000-0001-9050-2968, Carreira, Patricia E.|||0000-0001-8279-3806, Airò, P.|||0000-0001-5241-1918, Simeón-Aznar, Carmen Pilar|||0000-0003-3390-9029, Truchetet, M.E.|||0000-0001-8045-0180, Giollo, A.|||0000-0001-9355-7673, Balbir-Gurman, A.|||0000-0003-4694-6099, Martin, M.|||0000-0002-3826-4584, Denton, C.P.|||0000-0003-3975-8938, Gabrielli, Armando, Del Galdo, F.|||0000-0002-8528-2283, Vonk, M.C.|||0000-0002-2266-9907, Fretheim, H.|||0000-0002-0350-172X, Bitter, H.|||0000-0002-9161-1282, Midtvedt, Ø., Andreassen, A., Høie, S., Tanaka, Y.|||0000-0002-0807-7139, Riemekasten, G.|||0000-0002-5406-2464, Müller-Ladner, U., Matucci-Cerinic, Marco|||0000-0002-9324-3161, Castellvi, Ivan|||0000-0002-5410-5807, Siegert, E.|||0000-0002-9594-0446, Hachulla, E.|||0000-0001-7432-847X, Molberg, Ø.|||0000-0003-4493-8853, Distler, Oliver|||0000-0002-0546-8310, Hoffmann-Vold, A.M.|||0000-0001-6467-7422, Guiducci, S., Iannone, F., Rednic, S., Allanore, Yannick|||0000-0002-6149-0002, Montecucco, C., Kumánovics, G., Iudici, M., Moroncini, G., Andréasson, K., Idolazzi, L., Henes, J., Pflugfelder, J., Da Silva, J.A.P., Hughes, M., Riccieri, V., Balanescu, A., Gheorghiu, A.M., Bergmann, C., Cantatore, F.P., De Langhe, E., Ani, B., Müller, C.d.S., Solanki, K., Rosato, E., Maurer, B., Saketkoo, L.A., Limonta, M., Hsu, V.M., Chung, L.S., Levy, Y., Sfikakis, P., Oliveira, S., Kuwana, M.
Tipo de recurso: artículo
Fecha de publicación:2025
País:España
Institución:Universitat Autònoma de Barcelona
Repositorio:Dipòsit Digital de Documents de la UAB
Idioma:inglés
OAI Identifier:oai:ddd.uab.cat:322761
Acceso en línea:https://ddd.uab.cat/record/322761
https://dx.doi.org/urn:doi:10.1093/rheumatology/keaf053
Access Level:acceso abierto
Palabra clave:observational study
pulmonary arterial hypertension
risk stratification
systemic sclerosis
Descripción
Sumario: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.

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