A comparative analysis of risk stratification tools in SSc-associated pulmonary arterial hypertension : a EUSTAR analysis
Bjørkekjær, H.J. (University of Oslo)
Bruni, C. (Università degli Studi di Firenze)
Broch, K. (University of Oslo)
Brunborg, C. (Oslo University Hospital-Rikshospitalet)
Carreira, Patricia E. (Hospital 12 de Octubre (Madrid))
Airò, P. (9 Spedali Civili di Brescia)
Simeón-Aznar, Carmen Pilar (Hospital Universitari Vall d'Hebron)
Truchetet, M.E. (Bordeaux University Hospital)
Giollo, A. (University of Verona)
Balbir-Gurman, A. (Technion-Institute of Technology)
Martin, M. (Poitiers University Hospital)
Denton, C.P. (University College London)
Gabrielli, Armando (Università Politecnica delle Marche)
Del Galdo, F. (Leeds Teaching Hospitals Trust)
Vonk, M.C. (Radboud Universiteit)
Fretheim, H. (Lillehammer Hospital for Rheumatic Diseases)
Bitter, H. (Hospital of Southern Norway)
Midtvedt, Ø. (Oslo University Hospital (Oslo, Noruega))
Andreassen, A. (Oslo University Hospital (Oslo, Noruega))
Høie, S. (Hospital of Southern Norway)
Tanaka, Y. (University of Occupational and Environmental Health)
Riemekasten, G. (University of Lübeck)
Müller-Ladner, U. (Justus-Liebig University Giessen)
Matucci-Cerinic, Marco (University Vita Salute San Raffaele)
Castellvi, Ivan (Institut de Recerca Sant Pau)
Siegert, E. (Charite University Hospital)
Hachulla, E. (INFINITE - Institute for Translational Research in Inflammation)
Molberg, Ø. (Rikshospitalet)
Distler, Oliver (University Hospital Zurich (Suïssa))
Hoffmann-Vold, A.M. (Oslo University Hospital (Oslo, Noruega))
Guiducci, S.
Iannone, F.
Rednic, S.
Allanore, Yannick
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.
Universitat Autònoma de Barcelona. Departament de Medicina

Data:	2025
Resum:	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.
Drets:	Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, fins i tot amb finalitats comercials, sempre i quan es reconegui l'autoria de l'obra original.
Llengua:	Anglès
Document:	Article ; recerca ; Versió publicada
Matèria:	observational study ; pulmonary arterial hypertension ; risk stratification ; systemic sclerosis
Publicat a:	Rheumatology, Vol. 64 Núm. 6 (january 2025) , p. 3643-3656, ISSN 1462-0332

DOI: 10.1093/rheumatology/keaf053
PMID: 39878955

14 p, 2.1 MB

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