Effectiveness of oral anticoagulants in precapillary pulmonary hypertension associated with systemic sclerosis: a EUSTAR cohort study

OBJECTIVES: The role of anticoagulation in systemic sclerosis (SSc) with precapillary pulmonary hypertension (PH) remains controversial, with conflicting evidence regarding its impact on survival, including potential harmful effects. Aiming to address this research gap, we evaluated both mortality and PH worsening in a large SSc-precapillary PH cohort, additionally accounting for SSc-specific risk factors in our analyses, to better inform individualized treatment decisions. METHODS: This retrospective cohort study included SSc patients from the European Scleroderma Trials and Research database with precapillary PH confirmed by right heart catheterization. The association of OAC use with survival and PH worsening was assessed using Kaplan-Meier estimates and multivariable Cox regression models. To minimize confounding, propensity score matching was additionally performed. RESULTS: Among 614 patients included, 143 (23%) received OAC at the time of RHC. At baseline, patients on OAC had worse hemodynamic parameters. No significant association was found between OAC use and survival (HR 0.983 95%CI 0.724-1.334) or PH worsening (HR 1.070 95%CI 0.811-1.412). These findings remained consistent after propensity score matching and in the subgroup of 230 SSc patients with precapillary PH without interstitial lung disease. CONCLUSIONS: In this large SSc cohort with precapillary PH, we did not observe any clinical benefit associated with OAC, neither in terms of survival nor of PH worsening. Further prospective studies are needed to determine whether specific patient subgroups may benefit from anticoagulation therapy.

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Lara D. Veeken
Published
2026-09-17
DOI
https://doi.org/10.1093/rheumatology/keag516
Primary Topic
Systemic Sclerosis and Related Diseases
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article
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article

Effectiveness of oral anticoagulants in precapillary pulmonary hypertension associated with systemic sclerosis: a EUSTAR cohort study

Kristofer Andréasson, Gonçalo Boleto, Edoardo Rosato, I. Castellví et al.
Lara D. Veeken
Systemic Sclerosis and Related Diseases
article

Effectiveness of oral anticoagulants in precapillary pulmonary hypertension associated with systemic sclerosis: a EUSTAR cohort study

Kristofer Andréasson, Gonçalo Boleto, Edoardo Rosato, I. Castellví, Sébastien Sanges, Michele Iudici, Christina Bergmann, Marco Matucci‐Cerinic, Marie‐Elise Truchetet, Jeska de Vries‐Bouwstra, Carolina de Souza Müller, Serena Guiducci, Simona Rednic, Ana Maria Gheorghiu, Elise Siegert, Luca Idolazzi, Stefan Heitmann, Vivien Hsu, Nicola Farina, Marius Cadar, Addolorata Corrado, Masataka Kuwana, M. Martin, Gabór Kumánovics, Elisabetta Zanatta, Lesley Ann Saketkoo, Philipp Klemm, Lorinda Chung, Patricia Carreira, Silvia Bellando Randone, Andra Balanescu, Kamal Solanki, Yair Levy, Branimir Anic, Yoshiya Tanaka, Yannick Allanore, Britta Maurer, Ulf Müller-Ladner, Christopher Denton, Florenzo Iannone, Madelon C Vonk, Carlomaurizio Montecucco, Hilde Jenssen-Bjørkekjær, Francesco Del Galdo, Anna-Maria Hoffmann-Vold, Dilia Giuggioli, Gabriela Riemekasten, Jörg Henes, Oliver Distler, Petros P Sfikakis, Vanessa Smith, Paolo Airò, Alexandra Balbir-Gurman, Ellen De Langhe, Cosimo Bruni, Gianluca Moroncini, Susana Oliveira, Carmen-Pilar Simeón Aznar, Alberto Cauli
article en

Abstract

OBJECTIVES: The role of anticoagulation in systemic sclerosis (SSc) with precapillary pulmonary hypertension (PH) remains controversial, with conflicting evidence regarding its impact on survival, including potential harmful effects. Aiming to address this research gap, we evaluated both mortality and PH worsening in a large SSc-precapillary PH cohort, additionally accounting for SSc-specific risk factors in our analyses, to better inform individualized treatment decisions. METHODS: This retrospective cohort study included SSc patients from the European Scleroderma Trials and Research database with precapillary PH confirmed by right heart catheterization. The association of OAC use with survival and PH worsening was assessed using Kaplan-Meier estimates and multivariable Cox regression models. To minimize confounding, propensity score matching was additionally performed. RESULTS: Among 614 patients included, 143 (23%) received OAC at the time of RHC. At baseline, patients on OAC had worse hemodynamic parameters. No significant association was found between OAC use and survival (HR 0.983 95%CI 0.724-1.334) or PH worsening (HR 1.070 95%CI 0.811-1.412). These findings remained consistent after propensity score matching and in the subgroup of 230 SSc patients with precapillary PH without interstitial lung disease. CONCLUSIONS: In this large SSc cohort with precapillary PH, we did not observe any clinical benefit associated with OAC, neither in terms of survival nor of PH worsening. Further prospective studies are needed to determine whether specific patient subgroups may benefit from anticoagulation therapy.

Lara D. Veeken
Rutgers, The State University of New Jersey (US), University of Foggia (IT), University of Bern (CH), University of Verona (IT), University of Modena and Reggio Emilia (IT), Marche Polytechnic University (IT), Oslo University Hospital (NO), University of Padua (IT), University Hospital Centre Zagreb (HR), University of Lisbon (PT), Radboud University Nijmegen (NL), Vita-Salute San Raffaele University (IT), Carol Davila University of Medicine and Pharmacy (RO), University of Cagliari (IT), University of Zagreb (HR), Lund University (SE), University of Occupational and Environmental Health Japan (JP), University of Zurich (CH), Université Paris Cité (FR), Iuliu Hațieganu University of Medicine and Pharmacy (RO), University of Pavia (IT), Leiden University Medical Center (NL), Policlinico Umberto I (IT), University Hospital of Bern (CH), The Royal Free Hospital (GB), Ghent University Hospital (BE), Rambam Health Care Campus (IL), Hospital de Sant Pau (ES), Meir Medical Center (IL), Pulmonary Hypertension Association (US), Radboud University Medical Center (NL), Hospital of Southern Norway (NO), Centre Hospitalier Universitaire de Bordeaux (FR), Brescia University (US), Université de Poitiers (FR), Universitätsklinikum Erlangen (DE), Sorbonne Paris Cité (FR), Hôpital Cochin (FR), Hôpital Beau-Séjour (CH), Marienhospital Stuttgart (DE), Universitäres Kinderwunschzentrum Lübeck (DE), Hospital Prof. Dr. Fernando Fonseca (PT), University Hospital of Zurich (CH), Hospital Universitario 12 De Octubre (ES), Azienda Socio Sanitaria Territoriale degli Spedali Civili di Brescia (IT), Mylan (South Africa) (ZA), Institutul Cantacuzino (RO), University Hospital Schleswig-Holstein (DE), NIHR Leeds Musculoskeletal Biomedical Research Unit (GB), Azienda Ospedaliero-Universitaria Careggi (IT), Vall d'Hebron Hospital Universitari (ES), Lille Inflammation Research International Center (FR), Waikato District Health Board (NZ), Kerckhoff Klinik (DE), Hospital de Clínicas Universidade Federal do Paraná (BR), Scleroderma Foundation (US), Institut Cochin (FR), Hospital de Santa Maria (PT), Policlinico San Matteo Fondazione (IT), Istituti di Ricovero e Cura a Carattere Scientifico (IT), Nippon Medical School Hospital (JP), Istituto di Ricovero e Cura a Carattere Scientifico San Raffaele (IT), University College London (GB), University of Waikato (NZ), University of Bari Aldo Moro (IT), Athens State University (US), Charité - Universitätsmedizin Berlin (DE), University of Pecs (HU), University of Brescia (IT), University of Tübingen (DE), Sapienza University of Rome (IT), University of Lübeck (DE), Stanford University (US), KU Leuven (BE)
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