{ "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).\n\nDesign: Post hoc retrospective case–control study.\n\nSetting: European Scleroderma Trials and Research database.\n\nParticipants: SSc patients who underwent their first right heart catheterisation (RHC); patients with postcapillary PH (pulmonary wedge pressure >15 mm Hg) were excluded.\n\nInterventions: Exposure to VVDs (endothelin receptor antagonists (ERAs), phosphodiesterase-5 inhibitors, prostanoids) was defined as ‘ever’ before or ‘ongoing’ at the time of RHC.\n\nMain outcome measures: Presence of precapillary PH at RHC.\n\nResults: 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.\n\nConclusions: 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.", "abstract_shown": true, "affiliations": [ "Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Hospital, Milano, Italy", "Department of experimental and clinical medicine, Division of Rheumatology, Careggi University Hospital, University of Florence, Florence, Italy", "Department 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", "National Reference Center for Pulmonary Arterial Hypertension (Pulmotension), Lille, France", "Department of Rheumatology, Hospital of Southern Norway, Kristiansand, Norway", "Department of Statistics, Informatics and Applications, University of Florence, Florence, Italy", "Rheumatology department, CHU de Bordeaux, Bordeaux, France", "Department of Internal Medicine, Ghent University, Ghent, Belgium", "Department of Rheumatology, St. Maria Hospital - Carol Davila, University of Medicine andPharmacy, Bucharest, Romania", "Department Internal Medicine 3, University Hospital Erlangen, Erlangen, Germany", "Rheumatology Department, Université Paris Cité, Hospital Cochin, Paris, France", "Department of Rheumatology and Clinical Immunology Center, JLU Giessen, Bad Nauheim, Germany", "Department of Clinical, Internal, Anestesiology and Cardiovascular sciences, Sapienza University of Rome, Rome, Italy", "Section of Rheumatology, Department of Medicine, University of Verona, Verona, Italy", "Centre for Rheumatology, Royal Free London and University College London Medical School, London, UK", "Division of Rheumatology, A.O.U. Policlinico-San Marco, Catania, Italy", "Department of Rheumatology, Clinical Immunology, Geriatrics and Internal Medicine, Medical University of Gdańsk, Gdańsk, Poland", "Department of Rheumatology, University Hospital Strasbourg, Strasbourg, France", "Department of Rheumatology, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands", "Department of Rheumatology, Leiden University Medical Center, Leiden, The Netherlands", "Department of Rheumatology, Oslo University Hospital, Oslo, Norway", "Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland" ], "article_type": "research article", "arxiv": null, "author_total": 27, "authors": [ { "affs": [ 0, 1 ], "name": "Nicola Farina" }, { "affs": [ 1 ], "name": "Silvia Bellando-Randone" }, { "affs": [ 2, 3 ], "name": "Sebastien Sanges" }, { "affs": [ 4 ], "name": "Hilde Jenssen Bjørkekjaer" }, { "affs": [ 5 ], "name": "Lorenzo Tofani" }, { "affs": [ 6 ], "name": "Marie-Elise Truchetet" }, { "affs": [ 7 ], "name": "Vanessa Smith" }, { "affs": [ 8 ], "name": "Andra Balanescu" }, { "affs": [ 9 ], "name": "Christina Bergmann" }, { "affs": [ 10 ], "name": "Yannick Allanore" }, { "affs": [ 11 ], "name": "Philipp Klemm" }, { "affs": [ 12 ], "name": "Simona Truglia" }, { "affs": [ 13 ], "name": "Luca Idolazzi" }, { "affs": [ 14 ], "name": "Christopher Denton" }, { "affs": [ 15 ], "name": "Roberta Foti" }, { "affs": [ 16 ], "name": "Anna Wojteczek" }, { "affs": [ 17 ], "name": "Emmanuel Chatelus" }, { "affs": [ 18 ], "name": "Madelon Vonk" }, { "affs": [ 1 ], "name": "Serena Guiducci" }, { "affs": [ 2, 3 ], "name": "David Launay" }, { "affs": [ 2, 3 ], "name": "Eric Hachulla" }, { "affs": [ 19 ], "name": "Jeska K de Vries-Bouwstra" }, { "affs": [ 20, 21 ], "name": "Anna-Maria Hoffmann-Vold", "orcid": "0000-0001-6467-7422" }, { "affs": [ 21 ], "name": "Oliver Distler", "orcid": "0000-0002-0546-8310" }, { "affs": [ 0 ], "name": "Marco Matucci-Cerinic" }, { "affs": [ 1, 21 ], "name": "Cosimo Bruni" }, { "name": "Ulrich Walker" } ], "bibtex": "https://research.draconitic.com/paper/e6sdtr5915kcb.bib", "cited_by": 0, "doi": "10.1136/jsrd-2026-000044", "fulltext_url": "https://pmc.ncbi.nlm.nih.gov/articles/PMC13735301/", "id": "e6sdtr5915kcb", "issns": [ "2397-1983", "2397-1991" ], "landing_url": "https://doi.org/10.1136/jsrd-2026-000044", "language": "en", "licence": "CC-BY-NC-4.0", "licence_label": "CC BY-NC 4.0", "licence_source": "article", "licence_url": "https://creativecommons.org/licenses/by-nc/4.0/", "metadata_licence": null, "native_subjects": [ [ "heading", "Original Research" ], [ "openalex", "2740|Pulmonary Hypertension Research and Treatments" ] ], "openalex": "W7225078153", "pdf_url": "https://jsrd.bmj.com/content/jsrd/11/2/e000044.full.pdf", "pmcid": "PMC13735301", "pmid": "42991134", "published": "2026-11-02", "publisher": "BMJ Publishing Group", "ris": "https://research.draconitic.com/paper/e6sdtr5915kcb.ris", "sources": [ { "first_seen": "2026-11-09T12:21:00Z", "native_id": "PMC13735301", "source": "pmc", "url": "https://doi.org/10.1136/jsrd-2026-000044" } ], "subjects": [ { "field": "medicine", "origin": "openalex:2740|Pulmonary Hypertension Research and Treatments", "subfield": "general" } ], "title": "Association between bosentan and reduced detection of precapillary pulmonary hypertension in systemic sclerosis: evidence from a EUSTAR study", "title_text": "Association between bosentan and reduced detection of precapillary pulmonary hypertension in systemic sclerosis: evidence from a EUSTAR study", "type": "peer-reviewed", "updated_at": "2026-11-09T12:21:00Z", "url": "https://research.draconitic.com/paper/e6sdtr5915kcb", "venue": "Journal of Scleroderma and Related Disorders", "versions": [] }