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EULAR recommendations for the management of antiphospholipid syndrome in adults
  1. Maria G Tektonidou1,
  2. Laura Andreoli2,
  3. Marteen Limper3,
  4. Zahir Amoura4,
  5. Ricard Cervera5,
  6. Nathalie Costedoat-Chalumeau6,
  7. Maria Jose Cuadrado7,
  8. Thomas Dörner8,
  9. Raquel Ferrer-Oliveras9,
  10. Karen Hambly10,
  11. Munther A Khamashta11,
  12. Judith King12,
  13. Francesca Marchiori13,
  14. Pier Luigi Meroni14,
  15. Marta Mosca15,
  16. Vittorio Pengo16,
  17. Luigi Raio17,
  18. Guillermo Ruiz-Irastorza18,
  19. Yehuda Shoenfeld19,
  20. Ljudmila Stojanovich20,
  21. Elisabet Svenungsson21,
  22. Denis Wahl22,
  23. Angela Tincani2,
  24. Michael M Ward23
  1. 1 First Department of Propaedeutic Internal Medicine, Joint Rheumatology program, National and Kapodistrian University of Athens, Athens, Greece
  2. 2 Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy
  3. 3 Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, The Netherlands
  4. 4 Sorbonne University, French National Center for SLE and Aps, Service de Medecine Interne 2, InstitutE3M, Pitié Salpétrière, Paris, France
  5. 5 Autoimmune Diseases, Hospital Clinic, IDIBAPS, University of Barcelona, Barcelona, Spain
  6. 6 Centre de référence maladies auto-immunes et systémiques rares de l'île deFrance, Cochin Hospital, Université Paris Descartes-Sorbonne Paris Cité;INSERM U 1153, CRESS, Paris, France
  7. 7 Rheumatology Department, Clinica Universidad de Navarra, Madrid, Spain
  8. 8 Department of Med/Rheumatology and Clinical Immunology, Charite University Hospital, Berlin, Germany
  9. 9 Obstetrics and Gynecology Department and Systemic Diseases Research Unit, Vall ďHebron Research Institute-VHIR, Barcelona, Spain
  10. 10 School of Sport and Exercise Sciences, University of Kent, Chatham, UK
  11. 11 Rheumatology Department, Dubai Hospital, Dubai, United Arab Emirates
  12. 12 EULAR PARE Patient Research Partner, London, UK
  13. 13 EULAR PARE Patient Research Partner, Rome, Italy
  14. 14 MaACR, Immunorheumatology Research Laboratory, Istituto Auxologico Italiano, Milan, Italy
  15. 15 Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy
  16. 16 Department of Cardiac Thoracic and Vascular Sciences and Public Health, University of Padova, Padua, Italy
  17. 17 Department of Obstetrics and Gynaecology, University Hospital of Bern, Inselspital, Bern, Switzerland
  18. 18 Autoimmune Diseases Unit, Hospital Universitario Cruces, Barakaldo, Spain
  19. 19 Zabludowicz Center for Autoimmune Diseases, Sheba Medical Center, Tel-Aviv University, Israel
  20. 20 Bezhanijska Kosa, Belgrade University, Belgrade, Serbia
  21. 21 Department of Medicine, Solna, Rheumatology Unit, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden
  22. 22 Vascular Medicine Division and Regional Competence Center for Rare Vascular and Systemic Autoimmune Diseases and Vascular Medicine Division, Nancy University Hospital, INSERM UMR-S 1116 University of Lorraine, Nancy, France
  23. 23 Intramural Research Program, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, Maryland, USA
  1. Correspondence to Prof Maria G Tektonidou, First Department of Propaedeutic Internal Medicine, University of Athens, Athens 11527, Greece; mtektonidou{at}gmail.com

Abstract

The objective was to develop evidence-based recommendations for the management of antiphospholipid syndrome (APS) in adults. Based on evidence from a systematic literature review and expert opinion, overarching principles and recommendations were formulated and voted. High-risk antiphospholipid antibody (aPL) profile is associated with greater risk for thrombotic and obstetric APS. Risk modification includes screening for and management of cardiovascular and venous thrombosis risk factors, patient education about treatment adherence, and lifestyle counselling. Low-dose aspirin (LDA) is recommended for asymptomatic aPL carriers, patients with systemic lupus erythematosus without prior thrombotic or obstetric APS, and non-pregnant women with a history of obstetric APS only, all with high-risk aPL profiles. Patients with APS and first unprovoked venous thrombosis should receive long-term treatment with vitamin K antagonists (VKA) with a target international normalised ratio (INR) of 2–3. In patients with APS with first arterial thrombosis, treatment with VKA with INR 2–3 or INR 3–4 is recommended, considering the individual’s bleeding/thrombosis risk. Rivaroxaban should not be used in patients with APS with triple aPL positivity. For patients with recurrent arterial or venous thrombosis despite adequate treatment, addition of LDA, increase of INR target to 3–4 or switch to low molecular weight heparin may be considered. In women with prior obstetric APS, combination treatment with LDA and prophylactic dosage heparin during pregnancy is recommended. In patients with recurrent pregnancy complications, increase of heparin to therapeutic dose, addition of hydroxychloroquine or addition of low-dose prednisolone in the first trimester may be considered. These recommendations aim to guide treatment in adults with APS. High-quality evidence is limited, indicating a need for more research.

  • antiphospholipid antibodies
  • antiphospholipid syndrome
  • systemic lupus erythematosus
  • thrombosis
  • pregnancy morbidity
  • management
  • recommendations
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Footnotes

  • Handling editor Josef S Smolen

  • AT and MMW contributed equally.

  • Correction notice This article has been corrected since it published Online First. The discussion section has been amended and the title in table one.

  • Contributors Full-text review, data abstraction and risk of bias assessments were performed by LA and ML, and independently double-checked by MGT and MMW. MMW supervised the methodology of the SLR and prepared the evidence report. MGT and AT prepared the first draft of recommendations, and all authors have participated in the discussion and formulation of recommendations. MGT supervised the project and drafted the manuscript. All authors reviewed the manuscript and approved its final version.

  • Funding This project was funded by the European League Against Rheumatism. MMW was funded by the Intramural Research Program, National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH.

  • Competing interests None declared.

  • Patient consent for publication Not required.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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