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Publications

Journal Article Ahead of print

PubMed: 42758145 DOI: 10.1093/rheumatology/keag516

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

Rheumatology (Oxford)

Authors: Nicola Farina, Silvia Bellando Randone, Hilde Jenssen-Bjørkekjær, Sebastien Sanges, Patricia Carreira, Paolo Airò, Serena Guiducci, Dilia Giuggioli, Gabriela Riemekasten, Carmen-Pilar Simeón Aznar, Christina Bergmann, Elise Siegert, Ivan Castellví, Lesley Ann Saketkoo, Jeska de Vries-Bouwstra, Philipp Klemm, Ulf Müller-Ladner, Alexandra Balbir-Gurman, Vanessa Smith, Florenzo Iannone, Luca Idolazzi, Christopher Denton, Edoardo Rosato, Britta Maurer, Yannick Allanore, Yoshiya Tanaka, Elisabetta Zanatta, Marie-Elise Truchetet, Masataka Kuwana, Mickaël Martin, Alberto Cauli, Kamal Solanki, Francesco Del Galdo, Ana Maria Gheorghiu, Branimir Anic, Gonçalo Boleto, Gábor Kumánovics, Kristofer Andréasson, Simona Rednic, Lorinda S Chung, Susana Oliveira, Marius Cadar, Addolorata Corrado, Carolina de Souza Müller, Vivien M Hsu, Yair Levy, Gianluca Moroncini, Jörg Henes, Andra Balanescu, Ellen De Langhe … and 9 other authors

Affiliations

  • Department of Experimental and Clinical Medicine, Scleroderma Unit, Division of Rheumatology, University of Florence, Azienda Ospedaliera Universitaria Careggi, Florence, Italy.
  • Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Hospital, Milan, Italy.
  • Department of Rheumatology, Hospital of Southern Norway, Kristiansand, Norway.
  • National Reference Center for Pulmonary Arterial Hypertension (Pulmotension), Lille, France.
  • Inserm, CHU Lille, Department of Internal Medicine and Clinical Immunology, Reference Center for Rare Autoimmune and Autoinflammatory diseases (CERAINOM), U1286 - INFINITE - Institute for Translational Research in Inflammation, Univ. Lille, Lille, France.
  • Rheumatology Department, Hospital Universitario 12 de Octubre, Madrid, Spain.
  • Rheumatology and Clinical Immunology Unit, ASST Spedali Civili of Brescia, University of Brescia, Brescia, Italy.
  • Scleroderma Unit, Rheumatology Unit, University Hospital of Modena and Reggio Emilia, Modena, Italy.
  • Klinik für Rheumatologie und klinische Immunologie, Universitätsklinikum Schleswig-Holstein, Lübeck, Germany.
  • Department of Internal Medicine, Systemic Autoimmune Diseases Unit, Hospital Universitario Vall d'Hebron, Barcelona, Spain.
  • Department Internal Medicine 3, University Hospital Erlangen, Erlangen, Germany.
  • Department of Rheumatology, Charité University Hospital, Berlin, Germany.
  • Department of Rheumatology, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain.
  • New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans, USA.
  • Department of Rheumatology, Leiden University Medical Center, Leiden, The Netherlands.
  • Department of Rheumatology and Clinical Immunology Center, JLU Giessen, Campus Kerckhoff, Bad Nauheim, Germany.
  • Rheumatology Institute, Rambam Health Care Campus, Haifa, Israel.
  • Department of Rheumatology, University of Ghent, Ghent, Belgium.
  • Rheumatology DiMePReJ, University of Bari, School of Medicine, Bari, 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, United Kingdom.
  • Department of Translational and Precision Medicine, Azienda Ospedaliero-Universitaria Policlinico Umberto 1-Centro di riferimento regionale per la sclerosi sistemica, Sapienza University of Rome, Rome, Italy.
  • Department of Rheumatology and Immunology, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.
  • Rheumatology Department, Université Paris Cité, Cochin Hospital, Paris, France.
  • Department of Molecular Targeted Therapeutics, First Department of Internal Medicine, University of Occupational and Environmental Health, Kitakyūshū, Japan.
  • Rheumatology Unit, Padova University Hospital, Padova, Italy.
  • Rheumatology department, CHU de Bordeaux, Bordeaux, France.
  • Nippon Medical School Hospital, Tokyo, Japan.
  • Department of Internal Medicine, Poitiers University Hospital, Poitiers, France.
  • Rheumatology Unit, AOU and University of Cagliari, Department of Medical Sciences and Public Health, Monserrato, Cagliari, Italy.
  • Te Whatu Ora Health NZ- Waikato, Waikato School of Medicine, Hamilton, New Zealand.
  • Leeds Raynaud's and Scleroderma Program, NIHR Biomedical Research Centre, Leeds Institute of Rheumatic and Musculoskeletal Medicine, Leeds, United Kingdom.
  • Cantacuzino Hospital, Carol Davila University of Medicine and Pharmacy, Ion Cantacuzino Hospital, Bucharest, Romania.
  • Division of Clinical Immunology and Rheumatology, Department of Internal Medicine, University of Zagreb, School of Medicine, University Hospital Center Zagreb, Zagreb, Croatia.
  • Department of Rheumatology, ULS Santa Maria, Centro Académico de Medicina de Lisboa; Faculdade de Medicina, Universidade de Lisboa, Lisbon, Portugal.
  • Department of Rheumatology and Immunology, University of Pécs, Medical School, Pecs, Hungary.
  • Section of Rheumatology, Department of Clinical Sciences, Lund University, Lund, Sweden.
  • Clinica Reumatologie, University of Medicine and Pharmacy Iuliu Hatieganu Cluj, Cluj-Napoca, Romania.
  • Stanford University School of Medicine, Stanford, USA.
  • Systemic Immunomediated Diseases Unit, Fernando Fonseca Hospital, Department of Medicine IV, Amadora, Portugal.
  • Rheumatology Clinic, Sapienza University of Rome, Rome, Italy.
  • Rheumatology Unit, Department of Medical and Surgical Sciences, University of Foggia, Foggia, Italy.
  • Hospital de Clinicas da Universidade Federal do Parana, Curitiba, Brazil.
  • Rutgers - RWJ Scleroderma Program, New Brunswick, USA.
  • Meir Medical Center, Tel-Aviv university, Kfar-Saba, Israel.
  • Department of Internal Medicine, Marche University Hospital, Clinica Medica, Ancona, Italy.
  • Department for Internal Medicine II (Haematology, Oncology, Rheumatology and Clinical Immunology), University Hospital Tuebingen, Germany.
  • Department of Internal Medicine and Rheumatology, "Carol Davila" University of Medicine and Pharmacy, "Sf. Maria" Hospital, Bucharest, Romania.
  • Department of Development and Regeneration, University Hospital Leuven, Laboratory of Tissue Homeostasis and Disease, KU Leuven, Leuven, Belgium.

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.

MeSH

Sclerosis, Hypertension, Anticoagulants, Hypertension, Pulmonary, Cohort Studies, Propensity Score, Rheumatic Diseases, Therapeutics, Scleroderma, Systemic