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Autoimmunity
Review
Antiphospholipid syndrome: state of the art on clinical practice guidelines
  1. Marteen Limper1,
  2. Carlo Alberto Scirè2,
  3. Rosaria Talarico3,
  4. Zahir Amoura4,
  5. Tadej Avcin5,
  6. Martina Basile6,
  7. Gerd Burmester7,
  8. Linda Carli3,
  9. Ricard Cervera8,
  10. Nathalie Costedoat-Chalumeau9,
  11. Andrea Doria10,
  12. Thomas Dörner7,
  13. João Eurico Fonseca11,
  14. Ilaria Galetti12,
  15. Eric Hachulla13,
  16. David Launay13,
  17. Filipa Lourenco14,
  18. Carla Macieira11,
  19. Pierluigi Meroni15,
  20. Carlo Maurizio Montecucco16,
  21. Maria Francisca Moraes-Fontes14,
  22. Luc Mouthon9,
  23. Cecilia Nalli17,
  24. Veronique Ramoni16,
  25. Maria Tektonidou18,
  26. Jacob M van Laar1,
  27. Stefano Bombardieri19,
  28. Matthias Schneider20,
  29. Vanessa Smith21,22,
  30. Ana Vieira23,
  31. Maurizio Cutolo24,
  32. Marta Mosca3,25 and
  33. Angela Tincani17
  1. 1 Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands
  2. 2 Section of Rheumatology, Department of Medical Sciences, University of Ferrara, Ferrara, Italy
  3. 3 Rheumatology Unit, AOU Pisana, Pisa, Italy
  4. 4 Department of Internal Medicine, Hospital Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, Paris, France
  5. 5 Department of Allergology, Rheumatology and Clinical Immunology, University Children's Hospital, University Medical Centre Ljubljana, Ljubljana, Slovenia
  6. 6 Lissone, Lissone, Italy
  7. 7 Department of Rheumatology and Clinical Immunology, Charité University Hospital Berlin, Berlin, Germany
  8. 8 Department of Autoimmune Diseases, Hospital Clínic de Barcelona, Barcelona, Spain
  9. 9 Service de Médecine Interne, Hôpital Cochin, Centre de Référence Maladies systémiques Autoimmunes Rares d’Ile de France, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Descartes, Paris, France
  10. 10 Rheumatology Unit, Department of Medicine, AO Padova and University of Padua, Padua, Italy
  11. 11 Rheumatology Department, Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, Lisbon Academic Medical Centre, Lisbon, Portugal
  12. 12 Federation of European Scleroderma Associations (FESCA), Milan, Italy
  13. 13 Département de Médecine Interne et Immunologie Clinique, Centre de Référence des Maladies Systémiques et Auto-Immunes Rares du Nord-Ouest (CERAINO), LIRIC, INSERM, Univ. Lille, CHU Lille, Lille, France
  14. 14 Auto-immune Disease Unit, Hospital de Curry Cabral, Centro Hospitalar de Lisboa Central, EPE, Lisbon, Portugal
  15. 15 Division of Rheumatology, ASST.G Pini, Department of Clinical Sciences and Community Health, University of Milan and Istituto Auxologico Italiano, Milan, Italy
  16. 16 Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy
  17. 17 Rheumatology and Clinical Immunology Unit, Civil Hospital, Brescia, Italy
  18. 18 Joint Rheumatology Academic Program, First Department of Propaedeutic Internal Medicine, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece
  19. 19 University of Pisa, Pisa, Italy
  20. 20 Department of Rheumatology, Universitätsklinikum Düsseldorf, Düsseldorf, Germany
  21. 21 Department of Rheumatology, Ghent University Hospital, Ghent, Belgium
  22. 22 Department of Internal Medicine, Ghent University, Ghent, Belgium
  23. 23 Liga Portuguesa Contra as Doenças Reumáticas, Núcleo Síndrome de Sjögren, Lisbon, Portugal
  24. 24 Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, IRCCS Polyclinic Hospital San Martino, University of Genoa, Genoa, Italy
  25. 25 Rheumatology Unit, University of Pisa, Pisa, Italy
  1. Correspondence to Angela Tincani; angela.tincani{at}unibs.it

Abstract

Antiphospholipid syndrome (APS) is a rare disease characterised by venous and/or arterial thrombosis, pregnancy complications and the presence of specific autoantibodies called antiphospholipid antibodies. This review aims to identify existing clinical practice guidelines (CPG) as part of the ERN ReCONNET project, aimed at evaluating existing CPGs or recommendations in rare and complex diseases. Seventeen papers providing important data were identified; however, the literature search highlighted the scarceness of reliable clinical data to develop CPGs. With no formal clinical guidelines in place, diagnosis and treatment of APS is largely based on consensus and expert opinion. Patients’ unmet need refers to the understanding of the disease and its clinical picture and implications, the need of education for patients, family members and healthcare providers, as well as to the development of monitoring pathways involving multiple healthcare providers.

  • antiphospholipid syndrome
  • European reference networks
  • ERN ReCONNET
  • clinical practice guidelines
  • unmet needs

This is an Open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited, appropriate credit is given, any changes made indicated, and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/.

https://doi.org/10.1136/rmdopen-2018-000785

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    Footnotes

    • Contributors LM, NCC, TA: substantial contributions to the analysis and interpretation of data; substantial contributions to the conception and design of the work, the acquisition, analysis and interpretation of data; revising the work critically for important intellectual content; drafting the work and revising it critically for important intellectual content; final approval of the version to be published; agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. CAS, TR, ZA, LC, AD, DL, FL, CM, CMM, VR, MT, JMvL, SB, VS, AV, MM: substantial contributions to the analysis and interpretation of data; final approval of the version to be published; agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. AT, MB, GB, RC, NCC, TD, FJE, IG, EH, PM, MFMF, LM, MS, MC: substantial contributions to the analysis and interpretation of data; revising the work critically for important intellectual content; drafting the work and revising it critically for important intellectual content; final approval of the version to be published; agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

    • Funding This publication was funded by the European Union’s Health Programme (2014-2020).

    • Disclaimer ERN ReCONNET is one of the 24 European Reference Networks (ERNs) approved by the ERN Board of Member States. The ERNs are co-funded by the European Commission. The content of this publication represents the views of the authors only and it is their sole responsibility; it cannot be considered to reflect the views of the European Commission and/or the Consumers, Health, Agriculture and Food Executive Agency (CHAFEA) or any other body of the European Union. The European Commission and the Agency do not accept any responsibility for use that may be made of the information it contains.

    • Competing interests None declared.

    • Patient consent Not required.

    • Provenance and peer review Commissioned; externally peer reviewed.

    • Data sharing statement No additional data are available.