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

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 P...

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Autores: Bjorkekjaer, HJ, Bruni, C, Broch, K, Brunborg, C, Carreira, PE, Airò, P, Simeón-Aznar, CP, Truchetet, ME, Giollo, A, Balbir-Gurman, A, Martin, M, Denton, CP, Gabrielli, A, Del Galdo, F, Vonk, MC, Fretheim, H, Bitter, H, Midtvedt,O, Andreassen, A, Hoie, S, Tanaka, Y, Riemekasten, G, Mueller-Ladner, U, Matucci-Cerinic, M, Castellví, I, Siegert, E, Hachulla, E, Molberg,O, Distler, O, Hoffmann-Vold, AM, EUSTAR Collaborators
Formato: artículo
Estado:Versión publicada
Fecha de publicación:2025
País:España
Recursos:Institut d’Investigació Biomèdica Sant Pau (IIB Sant Pau)
Repositorio:r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pau
OAI Identifier:oai:iibsantpau.fundanetsuite.com:p20033
Acesso em linha:https://iibsantpau.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=20033
Access Level:acceso abierto
Palavra-chave:observational study
pulmonary arterial hypertension
risk stratification
systemic sclerosis
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spelling A comparative analysis of risk stratification tools in SSc-associated pulmonary arterial hypertension: a EUSTAR analysisBjorkekjaer, HJBruni, CBroch, KBrunborg, CCarreira, PEAirò, PSimeón-Aznar, CPTruchetet, MEGiollo, ABalbir-Gurman, AMartin, MDenton, CPGabrielli, ADel Galdo, FVonk, MCFretheim, HBitter, HMidtvedt,OAndreassen, AHoie, STanaka, YRiemekasten, GMueller-Ladner, UMatucci-Cerinic, MCastellví, ISiegert, EHachulla, EMolberg,ODistler, OHoffmann-Vold, AMEUSTAR Collaboratorsobservational studypulmonary arterial hypertensionrisk stratificationsystemic sclerosisObjectives 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.OXFORD UNIV PRESS2025info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionhttps://iibsantpau.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=20033RHEUMATOLOGYISSN: 14620324ISSNe: 14620332reponame:r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pauinstname:Institut d’Investigació Biomèdica Sant Pau (IIB Sant Pau)Inglésinfo:eu-repo/semantics/openAccessoai:iibsantpau.fundanetsuite.com:p200332026-06-14T12:41:47Z
dc.title.none.fl_str_mv A comparative analysis of risk stratification tools in SSc-associated pulmonary arterial hypertension: a EUSTAR analysis
title A comparative analysis of risk stratification tools in SSc-associated pulmonary arterial hypertension: a EUSTAR analysis
spellingShingle A comparative analysis of risk stratification tools in SSc-associated pulmonary arterial hypertension: a EUSTAR analysis
Bjorkekjaer, HJ
observational study
pulmonary arterial hypertension
risk stratification
systemic sclerosis
title_short A comparative analysis of risk stratification tools in SSc-associated pulmonary arterial hypertension: a EUSTAR analysis
title_full A comparative analysis of risk stratification tools in SSc-associated pulmonary arterial hypertension: a EUSTAR analysis
title_fullStr A comparative analysis of risk stratification tools in SSc-associated pulmonary arterial hypertension: a EUSTAR analysis
title_full_unstemmed A comparative analysis of risk stratification tools in SSc-associated pulmonary arterial hypertension: a EUSTAR analysis
title_sort A comparative analysis of risk stratification tools in SSc-associated pulmonary arterial hypertension: a EUSTAR analysis
dc.creator.none.fl_str_mv Bjorkekjaer, HJ
Bruni, C
Broch, K
Brunborg, C
Carreira, PE
Airò, P
Simeón-Aznar, CP
Truchetet, ME
Giollo, A
Balbir-Gurman, A
Martin, M
Denton, CP
Gabrielli, A
Del Galdo, F
Vonk, MC
Fretheim, H
Bitter, H
Midtvedt,O
Andreassen, A
Hoie, S
Tanaka, Y
Riemekasten, G
Mueller-Ladner, U
Matucci-Cerinic, M
Castellví, I
Siegert, E
Hachulla, E
Molberg,O
Distler, O
Hoffmann-Vold, AM
EUSTAR Collaborators
author Bjorkekjaer, HJ
author_facet Bjorkekjaer, HJ
Bruni, C
Broch, K
Brunborg, C
Carreira, PE
Airò, P
Simeón-Aznar, CP
Truchetet, ME
Giollo, A
Balbir-Gurman, A
Martin, M
Denton, CP
Gabrielli, A
Del Galdo, F
Vonk, MC
Fretheim, H
Bitter, H
Midtvedt,O
Andreassen, A
Hoie, S
Tanaka, Y
Riemekasten, G
Mueller-Ladner, U
Matucci-Cerinic, M
Castellví, I
Siegert, E
Hachulla, E
Molberg,O
Distler, O
Hoffmann-Vold, AM
EUSTAR Collaborators
author_role author
author2 Bruni, C
Broch, K
Brunborg, C
Carreira, PE
Airò, P
Simeón-Aznar, CP
Truchetet, ME
Giollo, A
Balbir-Gurman, A
Martin, M
Denton, CP
Gabrielli, A
Del Galdo, F
Vonk, MC
Fretheim, H
Bitter, H
Midtvedt,O
Andreassen, A
Hoie, S
Tanaka, Y
Riemekasten, G
Mueller-Ladner, U
Matucci-Cerinic, M
Castellví, I
Siegert, E
Hachulla, E
Molberg,O
Distler, O
Hoffmann-Vold, AM
EUSTAR Collaborators
author2_role author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
dc.subject.none.fl_str_mv observational study
pulmonary arterial hypertension
risk stratification
systemic sclerosis
topic observational study
pulmonary arterial hypertension
risk stratification
systemic sclerosis
description 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.
publishDate 2025
dc.date.none.fl_str_mv 2025
dc.type.none.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv https://iibsantpau.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=20033
url https://iibsantpau.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=20033
dc.language.none.fl_str_mv Inglés
language_invalid_str_mv Inglés
dc.rights.none.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.publisher.none.fl_str_mv OXFORD UNIV PRESS
publisher.none.fl_str_mv OXFORD UNIV PRESS
dc.source.none.fl_str_mv RHEUMATOLOGY
ISSN: 14620324
ISSNe: 14620332
reponame:r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pau
instname:Institut d’Investigació Biomèdica Sant Pau (IIB Sant Pau)
instname_str Institut d’Investigació Biomèdica Sant Pau (IIB Sant Pau)
reponame_str r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pau
collection r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pau
repository.name.fl_str_mv
repository.mail.fl_str_mv
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score 15.812429

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