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

Bjørkekjær 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 Ø, Andreassen A, Høie S, Tanaka Y, Riemekasten G, Müller-Ladner U, Matucci-Cerinic M, Castellví I, Siegert E, Hachulla E, Molberg Ø, Distler O, Hoffmann-Vold AM, Guiducci S, Iannone F, Rednic S, Allanore Y, Montecucco C, Kumánovics G, Iudici M, Moroncini G, Andréasson K, Idolazzi L, Henes J, Pflugfelder J, Da Silva JAP, Hughes M, Riccieri V, Balanescu A, Gheorghiu AM, Bergmann C, Cantatore FP, De Langhe E, Ani B, Müller CdS, Solanki K, Rosato E, Maurer B, Saketkoo LA, Limonta M, Hsu VM, Chung LS, Levy Y, Sfikakis P, Oliveira S, Kuwana M (2025)

Publication Type: Journal article

Publication year: 2025

Journal

Book Volume: 64

Pages Range: 3643-3656

Journal Issue: 6

DOI: 10.1093/rheumatology/keaf053

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 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.

Involved external institutions

Oslo University Hospital / Oslo Universitetssykehus Rikshospitalet Norway (NO) Universität zu Lübeck Germany (DE) University of Leeds United Kingdom (GB) Ospedale San Raffaele (früher: Centro San Raffaele del Monte Tabor Foundation) Italy (IT) University of Oslo Norway (NO) Vall d'Hebron University Hospital / Hospital Universitari Vall d'Hebron Spain (ES) Hospital Universitario 12 de Octubre Spain (ES) Radboud University Nijmegen Netherlands (NL) Centre Hospitalier Universitaire de Bordeaux / CHU Bordeaux France (FR) Justus-Liebig-Universität Gießen Germany (DE) Hospital of Southern Norway / Sørlandet sykehus Kristiansand Norway (NO) Universitätsspital Zürich (USZ) Switzerland (CH) Centre hospitalier universitaire de Poitiers (CHU de Poitiers) France (FR) Università Politecnica delle Marche (UNIVPM) / Marche Polytechnic University Italy (IT) Institute for Translational Research in Inflammation (INFINITE) France (FR) University College London (UCL) United Kingdom (GB) Azienda Ospedale Università Padova / University Hospital of Padova Italy (IT) University of Occupational and Environmental Health Japan / 産業医科大学 Japan (JP) Charité - Universitätsmedizin Berlin Germany (DE) Hospital de la Santa Creu i Sant Pau Spain (ES) Technion - Israel Institute of Technology Israel (IL) Azienda Socio Sanitaria Territoriale degli Spedali Civili di Brescia Italy (IT)

How to cite

APA:

Bjørkekjær, H.J., Bruni, C., Broch, K., Brunborg, C., Carreira, P.E., Airò, P.,... Kuwana, M. (2025). A comparative analysis of risk stratification tools in SSc-associated pulmonary arterial hypertension: a EUSTAR analysis. Rheumatology, 64(6), 3643-3656. https://doi.org/10.1093/rheumatology/keaf053

MLA:

Bjørkekjær, Hilde Jenssen, et al. "A comparative analysis of risk stratification tools in SSc-associated pulmonary arterial hypertension: a EUSTAR analysis." Rheumatology 64.6 (2025): 3643-3656.

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