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

IF 4.7 2区 医学 Q1 RHEUMATOLOGY Rheumatology Pub Date : 2025-01-29 DOI:10.1093/rheumatology/keaf053
Hilde Jenssen Bjørkekjær,Cosimo Bruni,Kaspar Broch,Cathrine Brunborg,Patricia E Carreira,Paolo Airò,Carmen Pilar Simeón-Aznar,Marie-Elise Truchetet,Alessandro Giollo,Alexandra Balbir-Gurman,Mickael Martin,Christopher P Denton,Armando Gabrielli,Francesco Del Galdo,Madelon C Vonk,Håvard Fretheim,Helle Bitter,Øyvind Midtvedt,Arne Andreassen,Sverre Høie,Yoshiya Tanaka,Gabriela Riemekasten,Ulf Müller-Ladner,Marco Matucci-Cerinic,Ivan Castellví,Elise Siegert,Eric Hachulla,Øyvind Molberg,Oliver Distler,Anna-Maria Hoffmann-Vold,
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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.
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来源期刊
Rheumatology
Rheumatology 医学-风湿病学
CiteScore
9.40
自引率
7.30%
发文量
1091
审稿时长
2 months
期刊介绍: Rheumatology strives to support research and discovery by publishing the highest quality original scientific papers with a focus on basic, clinical and translational research. The journal’s subject areas cover a wide range of paediatric and adult rheumatological conditions from an international perspective. It is an official journal of the British Society for Rheumatology, published by Oxford University Press. Rheumatology publishes original articles, reviews, editorials, guidelines, concise reports, meta-analyses, original case reports, clinical vignettes, letters and matters arising from published material. The journal takes pride in serving the global rheumatology community, with a focus on high societal impact in the form of podcasts, videos and extended social media presence, and utilizing metrics such as Altmetric. Keep up to date by following the journal on Twitter @RheumJnl.
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