Evidence map›Paper›PMID 40008525›Full record

ArticleJournal of the American Heart Association2025

Evaluation of Systemic Sclerosis Primary Heart Involvement and Chronic Heart Failure in the European Scleroderma Trials and Research Cohort.

Andrea-Hermina Györfi, Tim Filla, Amin Polzin, Koray Tascilar, Maya Buch, Monique Tröbs, Alexandru-Emil Matei, Paolo Airo, Alexandra Balbir-Gurman, Frederic Kuwert and 25 more

Abstract readMulticenter Study
In one paragraph

Article in Journal of the American Heart Association, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
14citing papers in PubMed, 1 pooled it
–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

14 citing papers in PubMed, 1 synthesis or guideline pooled it.

  1. Pooled it
  2. Article
  3. Pulmonary arterial hypertension with signs of venous/capillary involvement in connective tissue diseases: paradigms and paradoxes.European respiratory review : an official journal of the European Respiratory Society · 2026
    Review
  4. Review
  5. Heart failure in autoimmune rheumatic diseases.Rheumatology international · 2026
    Review
  6. Article
  7. Article
  8. Article
  9. Article
  10. Review
  11. Article
  12. Article
  13. Observational
  14. Review
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

35 authors.

Andrea-Hermina GyörfiDepartment of Rheumatology University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University Düsseldorf Germany.ORCID 0000-0002-4960-3369
Tim FillaDepartment of Rheumatology University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University Düsseldorf Germany.
Amin PolzinDepartment of Cardiology, Pneumology, and Angiology University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University Düsseldorf Germany.ORCID 0000-0002-5891-9672
Koray TascilarDepartment of Internal Medicine 3, Rheumatology and Clinical Immunology Friedrich-Alexander-University (FAU) Erlangen-Nürnberg and Universitätsklinikum Erlangen Erlangen Germany.ORCID 0000-0002-8109-826X
Maya BuchDepartment of Rheumatology University of Manchester Manchester UK.ORCID 0000-0002-8962-5642
Monique TröbsDepartment of Medicine 2-Cardiology and Angiology Friedrich-Alexander-Universität Erlangen-Nürnberg Erlangen Germany.ORCID 0000-0002-5661-1017
Alexandru-Emil MateiDepartment of Rheumatology University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University Düsseldorf Germany.ORCID 0000-0003-1248-3145
Paolo AiroScleroderma Unit, Rheumatology and Clinical Immunology Unit, ASST Spedali Civili Brescia Italy.ORCID 0000-0001-5241-1918
Alexandra Balbir-GurmanRheumatology Institute, Rambam Health Care Campus, The Rappaport Faculty of Medicine Technion Haifa Israel.ORCID 0000-0003-4694-6099
Frederic KuwertDepartment of Internal Medicine 3, Rheumatology and Clinical Immunology Friedrich-Alexander-University (FAU) Erlangen-Nürnberg and Universitätsklinikum Erlangen Erlangen Germany.
Carina MihaiDepartment of Rheumatology University Hospital Zurich, University of Zurich Zurich Switzerland.ORCID 0000-0002-8627-8817
Anna KabalaRheumatology Centre Hospitalier Universitaire de Bordeaux Groupe hospitalier Pellegrin Bordeaux France.
Hanna GraßhoffDepartment of Rheumatology and Clinical Immunology Universitätsklinikum Schleswig-Holstein Lübeck Germany.ORCID 0000-0003-1395-3268
Julia CallaghanDepartment of Rheumatology and Clinical Immunology Charité-Universitätsmedizin Berlin Berlin Germany.
Yohei IsomuraDepartment of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine Tokyo Japan.ORCID 0000-0003-1449-573X
Jennifer MansourScleroderma and Sarcoidosis Patient Care and Research Center University Medical Center Comprehensive Pulmonary Hypertension Center, Tulane University School of Medicine New Orleans LA.
Julia SpieringsDepartment of Rheumatology & Clinical Immunology University Medical Center Utrecht Utrecht the Netherlands.ORCID 0000-0002-2546-312X
Anders Heiervang TennoeDepartment of Rheumatology Oslo University Hospital Oslo Norway.ORCID 0000-0003-1756-2256
Enrico SelviRheumatology Unit, Department of Medical Sciences, Surgery and Neurosciences University of Siena Siena Italy.
Valeria RiccieriRheumatology Unit University of Rome La Sapienza Rome Italy.
Anna-Maria Hoffmann-VoldDepartment of Rheumatology Oslo University Hospital Oslo Norway.ORCID 0000-0001-6467-7422
Christina BergmannDepartment of Internal Medicine 3, Rheumatology and Clinical Immunology Friedrich-Alexander-University (FAU) Erlangen-Nürnberg and Universitätsklinikum Erlangen Erlangen Germany.ORCID 0000-0001-5257-9171
Georg SchettDepartment of Internal Medicine 3, Rheumatology and Clinical Immunology Friedrich-Alexander-University (FAU) Erlangen-Nürnberg and Universitätsklinikum Erlangen Erlangen Germany.ORCID 0000-0001-8740-9615
Nicolas HunzelmannDepartment of Dermatology and Venereology University Hospital Cologne Cologne Germany.
Jacob M van LaarDepartment of Rheumatology & Clinical Immunology University Medical Center Utrecht Utrecht the Netherlands.ORCID 0000-0001-5544-5785
Lesley Ann SaketkooScleroderma and Sarcoidosis Patient Care and Research Center University Medical Center Comprehensive Pulmonary Hypertension Center, Tulane University School of Medicine New Orleans LA.ORCID 0000-0002-1878-8591
Masataka KuwanaDepartment of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine Tokyo Japan.ORCID 0000-0001-8352-6136
Elise SiegertDepartment of Rheumatology and Clinical Immunology Charité-Universitätsmedizin Berlin Berlin Germany.ORCID 0000-0002-9594-0446
Gabriela RiemekastenDepartment of Rheumatology and Clinical Immunology Universitätsklinikum Schleswig-Holstein Lübeck Germany.ORCID 0000-0002-5406-2464
Oliver DistlerDepartment of Rheumatology University Hospital Zurich, University of Zurich Zurich Switzerland.ORCID 0000-0002-0546-8310
Tessa du FourDepartment of Internal Medicine and Department of Rheumatology Ghent University Hospital Ghent Belgium.ORCID 0009-0008-4420-5477
Vanessa SmithDepartment of Internal Medicine and Department of Rheumatology Ghent University Hospital Ghent Belgium.ORCID 0000-0001-6271-7945
Marie-Elise TruchetetRheumatology Centre Hospitalier Universitaire de Bordeaux Groupe hospitalier Pellegrin Bordeaux France.ORCID 0000-0001-8045-0180
Jörg H W DistlerDepartment of Rheumatology University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University Düsseldorf Germany.ORCID 0000-0001-7408-9333
EUSTAR collaborators

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundSystemic sclerosis (SSc) primary heart involvement (SSc-pHI) is one of the leading causes of mortality in SSc. We aimed to evaluate risk factors for SSc-pHI and its progression and the outcomes in the EUSTAR (European Scleroderma Trials and Research) cohort.

methodsSSc-pHI was defined according to the World Scleroderma Foundation/Heart Failure Association definition. Data from 5741 patients with SSc in the EUSTAR cohort were analyzed. Additional cardiovascular data were collected from a subcohort of 838 patients with SSc. Lasso regression was used for risk factor analyses. Kaplan-Meier estimator was used for survival analyses. Progression of SSc-pHI was evaluated by a study definition developed by rheumatology and cardiology experts.

resultsRisk factors for the presence of SSc-pHI comprised skeletal muscle atrophy (odds ratio [OR], 2.00 [95% CI, 1.00-2.68]), age (OR, 1.91 [95% CI, 1.73-2.03]), male sex (OR, 1.77 [95% CI, 1.42-2.05]), swollen joints (OR, 1.70 [95% CI, 1.47-1.98]), skeletal muscle weakness (OR, 1.38 [95% CI, 1.00-1.85]), and tendon friction rubs (OR, 1.46 [95% CI, 1.00-1.77]) (n=3276). Telangiectasia (OR, 2.10 [95% CI, 1.38-2.72]), intestinal symptoms (OR, 1.70 [95% CI, 1.04-2.42]), age (OR, 1.47 [95% CI, 1.21-1.62]), and antitopoisomerase I antibodies (OR, 1.37 [95% CI, 1.00-1.77]) were associated with an increased risk for new onset of SSc-pHI (n=1000). Survival rate was significantly lower in patients with SSc-pHI than in those without (

conclusionsWe defined progressive SSc-pHI and identified risk factors for new onset and progression of SSc-pHI and for SSc-pHI-associated heart failure with preserved ejection fraction in the largest cohort with SSc. These findings may guide patient stratification for diagnostic workup and therapy.

Indexed as

Heart FailureScleroderma, SystemicAdultAgedChronic DiseaseDisease ProgressionEuropeFemaleHumansMaleMiddle AgedRisk AssessmentRisk FactorsEUSTAR cohortmortalityrisk factorssystemic sclerosissystemic sclerosis primary heart involvement

Identifiers

PMID40008525
PMCPMC12132611

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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.