70cbbd5d6af88be31bb18a13c2b1358f 322761.pdf b51803a2e245a5976099e39284e06c7a57a45bf6 322761.pdf 642d9b9641e47e5107f258b70236db6fbbc369dc974fafa3d110438e33bec3a1 322761.pdf Title: A comparative analysis of risk stratification tools in SSc-associated pulmonary arterial hypertension: a EUSTAR analysis Subject: Doi: 10.1093/rheumatology/keaf053 Rheumatology, 00, 00, 2025 Publication Date: 29/01/2025 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. Keywords: observational study; pulmonary arterial hypertension; risk stratification; systemic sclerosis Creator: Servigistics Arbortext Advanced Print Publisher 11.1.4667/W Producer: PDFlib+PDI 9.0.7p3 (C++/Win32) CreationDate: Sun May 4 23:53:43 2025 CEST ModDate: Sun May 4 23:53:52 2025 CEST Custom Metadata: yes Metadata Stream: yes Tagged: yes UserProperties: no Suspects: no Form: none JavaScript: no Pages: 14 Encrypted: no Page size: 595.276 x 782.362 pts Page rot: 0 File size: 2223554 bytes Optimized: no PDF version: 1.5 name type encoding emb sub uni object ID ------------------------------------ ----------------- ---------------- --- --- --- --------- GANFIS+UniversLTStd-Cn Type 1C Custom yes yes yes 1042 0 TYBGDM+UniversLTStd-Bold Type 1C Custom yes yes yes 1047 0 TeX_CM_Roman Type 1 Builtin yes no yes 1052 0 TeX_CM_Maths_Symbols Type 1 Builtin yes no yes 1056 0 OZHZCA+SabonLTPro-Bold Type 1C Custom yes yes yes 1060 0 LEDAOL+SabonLTPro-Roman Type 1C Custom yes yes yes 1065 0 DKQVSF+STIX-Regular Type 1C Custom yes yes yes 1070 0 UEJIQD+UniversLTStd-CnObl Type 1C Custom yes yes yes 1075 0 NAIOTD+UniversLTStd-BoldCn Type 1C Custom yes yes yes 1080 0 SHXUMI+Trajan-Regular Type 1 Custom yes yes no 1090 0 ABPXBI+UniversLTStd-Light Type 1C Custom yes yes yes 78 0 IXEYMM+Univers-Bold CID TrueType Identity-H yes yes yes 3 0 QLRUPV+UniversLTStd Type 1C Custom yes yes yes 83 0 OCKVQS+UniversLTStd-Obl Type 1C Custom yes yes yes 88 0 TeX_CM_Maths_Italic Type 1 Builtin yes no yes 93 0 LOEZNU+STIXTwoMath-Regular CID TrueType Identity-H yes yes yes 97 0 BHAATR+STIX-Italic Type 1C Custom yes yes yes 18 0 PGCLHJ+SabonLTPro-Italic Type 1C Custom yes yes yes 130 0 UAGLXZ+UniversLTStd-LightObl Type 1C Custom yes yes yes 144 0 TSFCGY+SabonLTPro-BoldItalic Type 1C Custom yes yes yes 28 0 MDHXDL+NotoSerif CID TrueType Identity-H yes yes yes 33 0 Jhove (Rel. 1.28.0, 2023-05-18) Date: 2026-02-13 04:40:07 CET RepresentationInformation: 322761.pdf ReportingModule: PDF-hul, Rel. 1.12.4 (2023-03-16) LastModified: 2026-02-12 09:43:04 CET Size: 2223554 Format: PDF Version: 1.5 Status: Well-Formed, but not valid SignatureMatches: PDF-hul ErrorMessage: Invalid indirect destination - 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referenced object 'bkeaf053-B13' cannot be found ID: PDF-HUL-122 Offset: 2117533 ErrorMessage: edu.harvard.hul.ois.jhove.module.pdf.PdfInvalidException: Invalid indirect destination - referenced object 'bkeaf053-B25' cannot be found ID: PDF-HUL-122 Offset: 2117533 MIMEtype: application/pdf Profile: Tagged PDF PDFMetadata: Objects: 1199 FreeObjects: 1 IncrementalUpdates: 2 DocumentCatalog: ViewerPreferences: HideToolbar: false HideMenubar: false HideWindowUI: false FitWindow: false CenterWindow: false DisplayDocTitle: true NonFullScreenPageMode: UseNone Direction: L2R ViewArea: CropBox ViewClip: CropBox PrintArea: CropBox PageClip: CropBox PageLayout: SinglePage PageMode: UseOutlines Language: en Outlines: Item: Title: Active Content List Destination: mkchap_artid Children: Item: Title: Introduction Destination: mkchap5__sec Item: Title: Study design and methods Destination: mkchap6__sec Item: Title: Results Destination: mkchap11__sec Item: Title: Discussion Destination: mkchap16__sec Item: Title: Supplementary material Destination: mkchap17__sec Item: Title: Data availability Destination: mkchap18__sec Item: Title: Author contributions Destination: mkchap19__sec Item: Title: Funding Destination: mkchap20__sec Item: Title: Acknowledgements Destination: mkchap21_ack1_ack Item: Title: Ethics statement Destination: mkchap21__sec Item: Title: AI statement Destination: mkchap22__sec Item: Title: References Destination: mkchap23_ref1_ref-list Info: Title: A comparative analysis of risk stratification tools in SSc-associated pulmonary arterial hypertension: a EUSTAR analysis Subject: Doi: 10.1093/rheumatology/keaf053 Rheumatology, 00, 00, 2025 Publication Date: 29/01/2025 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. Keywords: observational study; pulmonary arterial hypertension; risk stratification; systemic sclerosis Creator: Servigistics Arbortext Advanced Print Publisher 11.1.4667/W Producer: PDFlib+PDI 9.0.7p3 (C++/Win32) CreationDate: Mon May 05 00:23:43 CEST 2025 ModDate: Mon May 05 00:23:52 CEST 2025 ID: 0x415fd137998651a2083b2691d4e22599, 0x9d68765fcc003f5f7bea45ec58579120 Filters: FilterPipeline: DCTDecode Images: Image: NisoImageMetadata: FormatName: image/jpg CompressionScheme: JPEG ImageWidth: 1510 ImageHeight: 1434 BitsPerSample: 8 BitsPerSampleUnit: integer Image: NisoImageMetadata: FormatName: image/jpg CompressionScheme: JPEG ImageWidth: 1700 ImageHeight: 1665 ColorSpace: RGB BitsPerSample: 8 BitsPerSampleUnit: integer Image: NisoImageMetadata: FormatName: image/jpg CompressionScheme: JPEG ImageWidth: 1375 ImageHeight: 1806 ColorSpace: RGB BitsPerSample: 8 BitsPerSampleUnit: integer Image: NisoImageMetadata: FormatName: image/jpg CompressionScheme: JPEG ImageWidth: 1925 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uuid:13ec6bf3-3e97-2b82-723d-4c59e52f747f uuid:E93D458B-E11A-56FA-B40D-F0347BEF89EE 2025-05-05T03:23:43+05:30 2025-05-05T03:23:52+05:30 Servigistics Arbortext Advanced Print Publisher 11.1.4667/W 2025-05-05T03:23:52+05:30 PDFlib+PDI 9.0.7p3 (C++/Win32) observational study; pulmonary arterial hypertension; risk stratification; systemic sclerosis A comparative analysis of risk stratification tools in SSc-associated pulmonary arterial hypertension: a EUSTAR analysis 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 Serena Guiducci Florenzo Iannone Simona Rednic Yannick Allanore Carlomaurizio Montecucco Gábor Kumánovics Michele Iudici Gianluca Moroncini Kristofer Andréasson Luca Idolazzi Jörg Henes Johannes Pflugfelder José António Pereira da Silva Michael Hughes Valeria Riccieri Andra Balanescu Ana Maria Gheorghiu Christina Bergmann Francesco Paolo Cantatore Ellen De Langhe Branimir Ani Carolina de Souza Müller Kamal Solanki Edoardo Rosato Britta Maurer Lesley Ann Saketkoo Massimiliano Limonta Vivien M Hsu Lorinda S Chung Yair Levy Petros Sfikakis Susana Oliveira Masataka Kuwana Doi: 10.1093/rheumatology/keaf053 Rheumatology, 00, 00, 2025 Publication Date: 29/01/2025 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. © The Author(s) 2025. 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