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

Bjorkekjaer, HJ.; Bruni, C.; Broch, K.; Brunborg, C.; Carreira, PE.; Airo, P.; Simeon-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.; Muller-Ladner, U.; Matucci-Cerinic, M.; Castellvi, I.; Siegert, E.; Hachulla, E.; Molberg, O.; Distler, O.; Hoffmann-Vold, AM.

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

All Authors

Bjorkekjaer, HJ.

Bruni, C.

Broch, K.

Brunborg, C.

Carreira, PE.

Airo, P.

Simeon-Aznar, CP.

Truchetet, ME.

Giollo, A.

Balbir-Gurman, A.

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LTHT Author

Del Galdo, Francesco
Del Galdo, Francesco

LTHT Department

NIHR Leeds Biomedical Research Centre
Rheumatology

Publication Date

2025

Item Type

Journal Article
Comparative Study
Multicenter Study

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.