Evidence map›Paper›PMID 39878955›Full record

ArticleRheumatology (Oxford, England)2025

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 and 21 more

Abstract readComparative StudyMulticenter Study
In one paragraph

Article in Rheumatology (Oxford, England), 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers.

0numbers the graph read from it
0cells of the map it votes in
7citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

7 citing papers in PubMed.

  1. Article
  2. Article
  3. Article
  4. Review
  5. Article
  6. Review
  7. Refining Risk Prediction in Systemic Sclerosis-Associated Pulmonary Arterial Hypertension.Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography · 2025
    Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

31 authors.

Hilde Jenssen BjørkekjærDepartment of Rheumatology, Hospital of Southern Norway, Kristiansand, Norway.ORCID 0009-0002-0321-375X
Cosimo BruniDepartment of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland.ORCID 0000-0003-2813-2083
Kaspar BrochDepartment of Cardiology, Oslo University Hospital, Rikshospitalet, Oslo, Norway.ORCID 0000-0003-4316-805X
Cathrine BrunborgInstitute of Clinical Medicine, University of Oslo, Oslo, Norway.ORCID 0000-0001-9050-2968
Patricia E CarreiraDepartment of Rheumatology, 12 de Octubre University Hospital, Madrid, Spain.ORCID 0000-0001-8279-3806
Paolo AiròUOC Reumatologia ed Immunologia Clinica, 9 Spedali Civili di Brescia, Scleroderma UNIT, Brescia, Italy.ORCID 0000-0001-5241-1918
Carmen Pilar Simeón-AznarSystemic Autoimmune Diseases Unit, Department of Internal Medicine, University Vall d'Hebron Hospital, Barcelona, Spain.ORCID 0000-0003-3390-9029
Marie-Elise TruchetetRheumatology Department, Bordeaux University Hospital, Bordeaux, France.ORCID 0000-0001-8045-0180
Alessandro GiolloDivision of Rheumatology, Department of Medicine-DIMED, University and Hospital of Padua, Padova, Italy.ORCID 0000-0001-9355-7673
Alexandra Balbir-GurmanRappaport Faculty of Medicine, Rheumatology Institute, Rambam Health Care Campus, Technion-Institute of Technology, Haifa, Israel.ORCID 0000-0003-4694-6099
Mickael MartinDepartment of Internal Medicine, Poitiers University Hospital, Poitiers, France.ORCID 0000-0002-3826-4584
Christopher P DentonCentre for Rheumatology and Connective Tissue Diseases, University College London Division of Medicine and Royal Free Hospital, London, UK.ORCID 0000-0003-3975-8938
Armando GabrielliFondazione di Medicina Molecolare e Terapia Cellulare, Università Politecnica delle Marche, Ancona, Italy.
Francesco Del GaldoLeeds Institute of Rheumatic and Musculoskeletal Medicine, LIRMM, Leeds, UK.ORCID 0000-0002-8528-2283
Madelon C VonkDepartment of Rheumatology, Radboud Universiteit, Nijmegen, Netherlands.ORCID 0000-0002-2266-9907
Håvard FretheimInstitute of Clinical Medicine, University of Oslo, Oslo, Norway.ORCID 0000-0002-0350-172X
Helle BitterDepartment of Rheumatology, Hospital of Southern Norway, Kristiansand, Norway.ORCID 0000-0002-9161-1282
Øyvind MidtvedtDepartment of Rheumatology, Oslo University Hospital, Rikshospitalet, Oslo, Norway.
Arne AndreassenDepartment of Cardiology, Oslo University Hospital, Rikshospitalet, Oslo, Norway.
Sverre HøieInstitute of Clinical Medicine, University of Oslo, Oslo, Norway.
Yoshiya TanakaFirst Department of Internal Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan.ORCID 0000-0002-0807-7139
Gabriela RiemekastenDepartment of Rheumatology and Clinical Immunology, University of Lübeck, Lübeck, Germany.ORCID 0000-0002-5406-2464
Ulf Müller-LadnerDepartment of Rheumatology and Clinical Immunology, Justus-Liebig University Giessen, Campus Kerckhoff, Bad Nauheim, Germany.ORCID 0009-0000-8832-8112
Marco Matucci-CerinicUnit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR) & Inflammation, fibrosis and ageing initiative (INFLAGE), IRCCS San Raffaele Hospital, Milan, Italy.ORCID 0000-0002-9324-3161
Ivan CastellvíDepartment of Rheumatology, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain.ORCID 0000-0002-5410-5807
Elise SiegertRheumatology, Charite University Hospital, Berlin, Germany.ORCID 0000-0002-9594-0446
Eric HachullaDepartment of Internal Medicine and Clinical Immunology, Referral Centre for Centre for Rare Systemic Autoimmune Diseases North of France, North-West, Mediterranean and Guadeloupe (CeRAINOM), CHU Lille, Univ. Lille, Inserm, U1286 - INFINITE - Institute for Translational Research in Inflammation, Lille, France.ORCID 0000-0001-7432-847X
Øyvind MolbergInstitute of Clinical Medicine, University of Oslo, Oslo, Norway.ORCID 0000-0003-4493-8853
Oliver DistlerDepartment of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland.ORCID 0000-0002-0546-8310
Anna-Maria Hoffmann-VoldDepartment of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland.ORCID 0000-0001-6467-7422
EUSTAR Collaborators

Funding

Bayer, Boehringer Ingelheim, Janssen and Medscape EP2331143Bayer, Boehringer Ingelheim, Janssen and Medscape US8247389Chair of the Executive Committee for the FOREUM FoundationGruppo Italiano Lotta alla SclerodermiaNIHRNovartis Foundation for Medical-Biological ResearchScleroderma Research FoundationSwiss Academy of Medical SciencesSwiss Clinical Quality Management in Rheumatic DiseasesWelcome Trust
6 · The paper itself

Abstract

objectivesThe 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.

methodsIn 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.

resultsThe 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.

conclusionConsidering 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.

Indexed as

Pulmonary Arterial HypertensionScleroderma, SystemicAdultAgedEuropeFemaleHumansMaleMiddle AgedPrognosisRisk Assessmentobservational studypulmonary arterial hypertensionrisk stratificationsystemic sclerosis

Identifiers

PMID39878955
PMCPMC12107028

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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.