A comparative analysis of risk stratification tools in SSc-associated pulmonary arterial hypertension: a EUSTAR analysis
Por:
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
Publicada:
1 jun 2025
Ahead of Print:
1 feb 2025
Resumen:
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.
Filiaciones:
Bjorkekjaer, HJ:
Hosp Southern Norway, Dept Rheumatol, Kristiansand, Norway
Univ Oslo, Inst Clin Med, Oslo, Norway
Bruni, C:
Univ Zurich, Univ Hosp Zurich, Dept Rheumatol, Zurich, Switzerland
Univ Florence, Dept Expt & Clin Med, Florence, Italy
Broch, K:
Oslo Univ Hosp, Dept Cardiol, Rikshosp, Oslo, Norway
Univ Oslo, Inst Expt Med Res, KG Jebsen Ctr, Oslo, Norway
Brunborg, C:
Univ Oslo, Inst Clin Med, Oslo, Norway
Oslo Univ Hosp, Oslo Ctr Biostat & Epidemiol, Res Support Serv, Rikshosp, Oslo, Norway
Carreira, PE:
12 Octubre Univ Hosp, Dept Rheumatol, Madrid, Spain
Airò, P:
9 Sped Civili Brescia, Scleroderma UNIT, UOC Reumatol Immunol Clin, Brescia, Italy
Simeón-Aznar, CP:
Univ Vall dHebron Hosp, Dept Internal Med, Syst Autoimmune Dis Unit, Barcelona, Spain
Truchetet, ME:
Bordeaux Univ Hosp, Rheumatol Dept, Bordeaux, France
Giollo, A:
Univ & Hosp Padua, Dept Med DIMED, Div Rheumatol, Padua, Italy
Balbir-Gurman, A:
Technion Israel Inst Technol, Rheumatol Inst, Rappaport Fac Med, Rambam Hlth Care Campus, Haifa, Israel
Martin, M:
Univ Poitiers Hosp, Dept Internal Med, Poitiers, France
Denton, CP:
Univ Coll London Med Sch, Royal Free Hosp, Ctr Rheumatol & Connect Tissue Dis, London, England
Gabrielli, A:
Univ Verona, Dept Med, Rheumatol Sect, Verona, Italy
Univ Politecn Marche, Fdn Med Mol & Terapia Cellulare, Ancona, Italy
Del Galdo, F:
Leeds Inst Rheumat & Musculoskeletal Med, LIRMM, Leeds, England
Leeds Teaching Hosp Trust, NIHR Leeds Biomed Res Ctr, Leeds, England
Vonk, MC:
Radboud Univ Nijmegen, Dept Rheumatol, Nijmegen, Netherlands
Fretheim, H:
Lillehammer Hosp Rheumat Dis, Lillehammer, Norway
Bitter, H:
Hosp Southern Norway, Dept Rheumatol, Kristiansand, Norway
Midtvedt,O:
Oslo Univ Hosp, Dept Rheumatol, Rikshosp, Pb 4950 Nydalen, Oslo, Norway
Andreassen, A:
Oslo Univ Hosp, Dept Cardiol, Rikshosp, Oslo, Norway
Hoie, S:
Hosp Southern Norway, Dept Cardiol, Arendal, Norway
Tanaka, Y:
Univ Occupat & Environm Hlth, Dept Internal Med 1, Kitakyushu, Japan
Riemekasten, G:
Univ Lubeck, Dept Rheumatol & Clin Immunol, Lubeck, Germany
Mueller-Ladner, U:
Justus Liebig Univ Giessen, Dept Rheumatol & Clin Immunol, Campus Kerckhoff, Bad Nauheim, Germany
Matucci-Cerinic, M:
IRCCS San Raffaele Hosp, Unit Immunol Rheumatol Allergy & Rare Dis UnIRAR, Milan, Italy
IRCCS San Raffaele Hosp, Inflammat Fibrosis & Ageing Initiat INFLAGE, Milan, Italy
Univ Vita Salute San Raffaele, Milan, Italy
Castellví, I:
Hosp Santa Creu I St Pau, Dept Rheumatol, Barcelona, Spain
Siegert, E:
Charite, Rheumatol, Berlin, Germany
Hachulla, E:
Univ Lille, CHU Lille, Referral Ctr Ctr Rare Syst Autoimmune Dis North Fr, Dept Internal Med & Clin Immunol,North West Medite, F-59000 Lille, France
Molberg,O:
Univ Oslo, Inst Clin Med, Oslo, Norway
Oslo Univ Hosp, Dept Rheumatol, Rikshosp, Pb 4950 Nydalen, Oslo, Norway
Distler, O:
Univ Zurich, Univ Hosp Zurich, Dept Rheumatol, Zurich, Switzerland
Hoffmann-Vold, AM:
Oslo Univ Hosp, Dept Rheumatol, Rikshosp, Pb 4950 Nydalen, Oslo, Norway
Green Submitted, hybrid
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