Skip to content
DraconiticResearch
Search Sources API
203,234 studies31 sourcesUpdated 44 min ago
Peer-reviewedCC BY-NC 4.0Journal of Scleroderma and Related Disorders

Association between bosentan and reduced detection of precapillary pulmonary hypertension in systemic sclerosis: evidence from a EUSTAR study

  • Nicola Farina1,2
  • Silvia Bellando-Randone2
  • Sebastien Sanges3,4
  • Hilde Jenssen Bjørkekjaer5
  • Lorenzo Tofani6
  • Marie-Elise Truchetet7
  • Vanessa Smith8
  • Andra Balanescu9
  • Christina Bergmann10
  • Yannick Allanore11
  • Philipp Klemm12
  • Simona Truglia13
15 more authors
  • Luca Idolazzi14
  • Christopher Denton15
  • Roberta Foti16
  • Anna Wojteczek17
  • Emmanuel Chatelus18
  • Madelon Vonk19
  • Serena Guiducci2
  • David Launay3,4
  • Eric Hachulla3,4
  • Jeska K de Vries-Bouwstra20
  • Anna-Maria Hoffmann-Vold21,22
  • Oliver Distler22
  • Marco Matucci-Cerinic1
  • Cosimo Bruni2,22
  • Ulrich Walker
22 affiliations
  1. 1Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Hospital, Milano, Italy
  2. 2Department of experimental and clinical medicine, Division of Rheumatology, Careggi University Hospital, University of Florence, Florence, Italy
  3. 3Department of Internal Medicine and Clinical Immunology - Reference Center for Rare Autoimmune and Autoinflammatory diseases (CERAINOM), U1286 -INFINITE - Institute for Translational Research in Inflammation, University of Lille - Inserm, CHU Lille, Lille, France
  4. 4National Reference Center for Pulmonary Arterial Hypertension (Pulmotension), Lille, France
  5. 5Department of Rheumatology, Hospital of Southern Norway, Kristiansand, Norway
  6. 6Department of Statistics, Informatics and Applications, University of Florence, Florence, Italy
  7. 7Rheumatology department, CHU de Bordeaux, Bordeaux, France
  8. 8Department of Internal Medicine, Ghent University, Ghent, Belgium
  9. 9Department of Rheumatology, St. Maria Hospital - Carol Davila, University of Medicine andPharmacy, Bucharest, Romania
  10. 10Department Internal Medicine 3, University Hospital Erlangen, Erlangen, Germany
  11. 11Rheumatology Department, Université Paris Cité, Hospital Cochin, Paris, France
  12. 12Department of Rheumatology and Clinical Immunology Center, JLU Giessen, Bad Nauheim, Germany
  13. 13Department of Clinical, Internal, Anestesiology and Cardiovascular sciences, Sapienza University of Rome, Rome, Italy
  14. 14Section of Rheumatology, Department of Medicine, University of Verona, Verona, Italy
  15. 15Centre for Rheumatology, Royal Free London and University College London Medical School, London, UK
  16. 16Division of Rheumatology, A.O.U. Policlinico-San Marco, Catania, Italy
  17. 17Department of Rheumatology, Clinical Immunology, Geriatrics and Internal Medicine, Medical University of Gdańsk, Gdańsk, Poland
  18. 18Department of Rheumatology, University Hospital Strasbourg, Strasbourg, France
  19. 19Department of Rheumatology, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands
  20. 20Department of Rheumatology, Leiden University Medical Center, Leiden, The Netherlands
  21. 21Department of Rheumatology, Oslo University Hospital, Oslo, Norway
  22. 22Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland
Read full textPDFDOICite

Abstract

Objectives: To evaluate whether exposure to vasoactive-vasodilating drugs (VVDs), particularly bosentan, is associated with reduced detection of precapillary pulmonary hypertension (PH) in systemic sclerosis (SSc).

Design: Post hoc retrospective case–control study.

Setting: European Scleroderma Trials and Research database.

Participants: SSc patients who underwent their first right heart catheterisation (RHC); patients with postcapillary PH (pulmonary wedge pressure >15 mm Hg) were excluded.

Interventions: Exposure to VVDs (endothelin receptor antagonists (ERAs), phosphodiesterase-5 inhibitors, prostanoids) was defined as ‘ever’ before or ‘ongoing’ at the time of RHC.

Main outcome measures: Presence of precapillary PH at RHC.

Results: Among the 944 patients included in the study, 498 (53%) were diagnosed with precapillary PH. Bosentan ‘ever’ was associated with a lower likelihood of a precapillary PH detection (OR 0.594, 95% CI 0.362 to 0.975) in the whole population. When considering ‘ongoing’ bosentan exposure, this result was confirmed only in patients with current DU (OR 0.230, 95% CI 0.081 to 0.652). ERAs in general showed a trend for protective effects towards detection of precapillary PH in patients with current DU (‘ever’: OR 0.636, 95% CI 0.349 to 1.158; ‘ongoing’: OR 0.435, 95% CI 0.193 to 1.064), while other VVD classes did not.

Conclusions: Bosentan, especially when employed in patients with current DU at the time of RHC, was associated with less detection of precapillary PH. These findings highlight the need for individualised treatment strategies and further research to optimise the timing and patient selection for VVD therapy in SSc.

Related

  1. Interocular symmetry of retinal and choroidal OCT parameters in precapillary pulmonary hypertension: a brief research report
    Diana A. Dmuchowska, Katarzyna Ptaszyńska, Joanna Konopińska +1
    Frontiers in medicinePeer-reviewedCC BYFull textGeneral medicineEurope PMC
  2. Sotatercept-related peripheral microvascular alterations in systemic sclerosis–associated pulmonary arterial hypertension: insights from nailfold capillaroscopy
    Yuichiro Segawa, Takehiro Takahashi, Jun Yamamoto +12
    Rheumatology Advances in PracticePeer-reviewedCC BY 4.0Full textGeneral medicinePMCEurope PMC
  3. Case Report: Upfront sotatercept-based triple therapy in high-risk systemic sclerosis-associated pulmonary arterial hypertension
    Yuichi Tamura, Sarasa Isobe, Hiroyuki Fujii +2
    Frontiers in Cardiovascular MedicinePeer-reviewedCC BY 4.0Full textCardiovascular medicineCrossrefEurope PMC
  4. Endothelial and platelet dysfunction in systemic sclerosis with pulmonary hypertension
    Thamonwan Imerbtham, Thanaporn Sriwantana, Pintip Ngamjanyaporn +8
    PLOS OnePeer-reviewedCC BY 4.0Full textPhysiologyPLOS
  5. Single-view echocardiography-based deep learning for pulmonary hypertension detection: a multi-level supervised and explainable approach
    Qiumeng Xi, Hebin Liu, Xinpeng Dai +3
    Quantitative imaging in medicine and surgeryPeer-reviewedCC BY-NC-NDFull textMedical imaging & radiologyEurope PMC

Cite

BibTeXRIS
BibTeX
@article{farina2026association,
  title = {{Association between bosentan and reduced detection of precapillary pulmonary hypertension in systemic sclerosis: evidence from a EUSTAR study}},
  author = {Nicola Farina and Silvia Bellando-Randone and Sebastien Sanges and Hilde Jenssen Bjørkekjaer and Lorenzo Tofani and Marie-Elise Truchetet and Vanessa Smith and Andra Balanescu and Christina Bergmann and Yannick Allanore and Philipp Klemm and Simona Truglia and Luca Idolazzi and Christopher Denton and Roberta Foti and Anna Wojteczek and Emmanuel Chatelus and Madelon Vonk and Serena Guiducci and David Launay and Eric Hachulla and Jeska K de Vries-Bouwstra and Anna-Maria Hoffmann-Vold and Oliver Distler and Marco Matucci-Cerinic and Cosimo Bruni and Ulrich Walker},
  journal = {Journal of Scleroderma and Related Disorders},
  year = {2026},
  month = nov,
  doi = {10.1136/jsrd-2026-000044},
  url = {https://doi.org/10.1136/jsrd-2026-000044},
  note = {CC BY-NC 4.0},
}
RIS
TY  - JOUR
TI  - Association between bosentan and reduced detection of precapillary pulmonary hypertension in systemic sclerosis: evidence from a EUSTAR study
AU  - Nicola Farina
AU  - Silvia Bellando-Randone
AU  - Sebastien Sanges
AU  - Hilde Jenssen Bjørkekjaer
AU  - Lorenzo Tofani
AU  - Marie-Elise Truchetet
AU  - Vanessa Smith
AU  - Andra Balanescu
AU  - Christina Bergmann
AU  - Yannick Allanore
AU  - Philipp Klemm
AU  - Simona Truglia
AU  - Luca Idolazzi
AU  - Christopher Denton
AU  - Roberta Foti
AU  - Anna Wojteczek
AU  - Emmanuel Chatelus
AU  - Madelon Vonk
AU  - Serena Guiducci
AU  - David Launay
AU  - Eric Hachulla
AU  - Jeska K de Vries-Bouwstra
AU  - Anna-Maria Hoffmann-Vold
AU  - Oliver Distler
AU  - Marco Matucci-Cerinic
AU  - Cosimo Bruni
AU  - Ulrich Walker
PY  - 2026
DA  - 2026/11/02
JO  - Journal of Scleroderma and Related Disorders
DO  - 10.1136/jsrd-2026-000044
UR  - https://doi.org/10.1136/jsrd-2026-000044
AB  - Objectives: To evaluate whether exposure to vasoactive-vasodilating drugs (VVDs), particularly bosentan, is associated with reduced detection of precapillary pulmonary hypertension (PH) in systemic sclerosis (SSc).  Design: Post hoc retrospective case–control study.  Setting: European Scleroderma Trials and Research database.  Participants: SSc patients who underwent their first right heart catheterisation (RHC); patients with postcapillary PH (pulmonary wedge pressure >15 mm Hg) were excluded.  Interventions: Exposure to VVDs (endothelin receptor antagonists (ERAs), phosphodiesterase-5 inhibitors, prostanoids) was defined as ‘ever’ before or ‘ongoing’ at the time of RHC.  Main outcome measures: Presence of precapillary PH at RHC.  Results: Among the 944 patients included in the study, 498 (53%) were diagnosed with precapillary PH. Bosentan ‘ever’ was associated with a lower likelihood of a precapillary PH detection (OR 0.594, 95% CI 0.362 to 0.975) in the whole population. When considering ‘ongoing’ bosentan exposure, this result was confirmed only in patients with current DU (OR 0.230, 95% CI 0.081 to 0.652). ERAs in general showed a trend for protective effects towards detection of precapillary PH in patients with current DU (‘ever’: OR 0.636, 95% CI 0.349 to 1.158; ‘ongoing’: OR 0.435, 95% CI 0.193 to 1.064), while other VVD classes did not.  Conclusions: Bosentan, especially when employed in patients with current DU at the time of RHC, was associated with less detection of precapillary PH. These findings highlight the need for individualised treatment strategies and further research to optimise the timing and patient selection for VVD therapy in SSc.
SN  - 2397-1983
SN  - 2397-1991
ER  -