{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,28]],"date-time":"2026-04-28T00:01:02Z","timestamp":1777334462491,"version":"3.51.4"},"reference-count":41,"publisher":"BMJ","issue":"Suppl 1","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["RMD Open"],"accepted":{"date-parts":[[2018,8,28]]},"published-print":{"date-parts":[[2018,10]]},"abstract":"<jats:p>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\u2019 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.<\/jats:p>","DOI":"10.1136\/rmdopen-2018-000785","type":"journal-article","created":{"date-parts":[[2018,10,18]],"date-time":"2018-10-18T19:15:34Z","timestamp":1539890134000},"page":"e000785","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":39,"title":["Antiphospholipid syndrome: state of the art on clinical practice guidelines"],"prefix":"10.1136","volume":"4","author":[{"given":"Marteen","family":"Limper","sequence":"first","affiliation":[{"name":"Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7451-0271","authenticated-orcid":false,"given":"Carlo Alberto","family":"Scir\u00e8","sequence":"additional","affiliation":[{"name":"Section of Rheumatology, Department of Medical Sciences, University of Ferrara, Ferrara, Italy"}]},{"given":"Rosaria","family":"Talarico","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, AOU Pisana, Pisa, Italy"}]},{"given":"Zahir","family":"Amoura","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Hospital Piti\u00e9-Salp\u00eatri\u00e8re, Assistance Publique-H\u00f4pitaux de Paris, Paris, France"}]},{"given":"Tadej","family":"Avcin","sequence":"additional","affiliation":[{"name":"Department of Allergology, Rheumatology and Clinical Immunology, University Children's Hospital, University Medical Centre Ljubljana, Ljubljana, Slovenia"}]},{"given":"Martina","family":"Basile","sequence":"additional","affiliation":[{"name":"Lissone, Lissone, Italy"}]},{"given":"Gerd","family":"Burmester","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Clinical Immunology, Charit\u00e9 University Hospital Berlin, Berlin, Germany"}]},{"given":"Linda","family":"Carli","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, AOU Pisana, Pisa, Italy"}]},{"given":"Ricard","family":"Cervera","sequence":"additional","affiliation":[{"name":"Department of Autoimmune Diseases, Hospital Cl\u00ednic de Barcelona, Barcelona, Spain"}]},{"given":"Nathalie","family":"Costedoat-Chalumeau","sequence":"additional","affiliation":[{"name":"Service de M\u00e9decine Interne, H\u00f4pital Cochin, Centre de R\u00e9f\u00e9rence Maladies syst\u00e9miques Autoimmunes Rares d\u2019Ile de France, Assistance Publique-H\u00f4pitaux de Paris (AP-HP), Universit\u00e9 Paris Descartes, Paris, France"}]},{"given":"Andrea","family":"Doria","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, Department of Medicine, AO Padova and University of Padua, Padua, Italy"}]},{"given":"Thomas","family":"D\u00f6rner","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Clinical Immunology, Charit\u00e9 University Hospital Berlin, Berlin, Germany"}]},{"given":"Jo\u00e3o Eurico","family":"Fonseca","sequence":"additional","affiliation":[{"name":"Rheumatology Department, Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, Lisbon Academic Medical Centre, Lisbon, Portugal"}]},{"given":"Ilaria","family":"Galetti","sequence":"additional","affiliation":[{"name":"Federation of European Scleroderma Associations (FESCA), Milan, Italy"}]},{"given":"Eric","family":"Hachulla","sequence":"additional","affiliation":[{"name":"D\u00e9partement de M\u00e9decine Interne et Immunologie Clinique, Centre de R\u00e9f\u00e9rence des Maladies Syst\u00e9miques et Auto-Immunes Rares du Nord-Ouest (CERAINO), LIRIC, INSERM, Univ. Lille, CHU Lille, Lille, France"}]},{"given":"David","family":"Launay","sequence":"additional","affiliation":[{"name":"D\u00e9partement de M\u00e9decine Interne et Immunologie Clinique, Centre de R\u00e9f\u00e9rence des Maladies Syst\u00e9miques et Auto-Immunes Rares du Nord-Ouest (CERAINO), LIRIC, INSERM, Univ. Lille, CHU Lille, Lille, France"}]},{"given":"Filipa","family":"Lourenco","sequence":"additional","affiliation":[{"name":"Auto-immune Disease Unit, Hospital de Curry Cabral, Centro Hospitalar de Lisboa Central, EPE, Lisbon, Portugal"}]},{"given":"Carla","family":"Macieira","sequence":"additional","affiliation":[{"name":"Rheumatology Department, Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, Lisbon Academic Medical Centre, Lisbon, Portugal"}]},{"given":"Pierluigi","family":"Meroni","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, ASST.G Pini, Department of Clinical Sciences and Community Health, University of Milan and Istituto Auxologico Italiano, Milan, Italy"}]},{"given":"Carlo Maurizio","family":"Montecucco","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8917-6592","authenticated-orcid":false,"given":"Maria Francisca","family":"Moraes-Fontes","sequence":"additional","affiliation":[{"name":"Auto-immune Disease Unit, Hospital de Curry Cabral, Centro Hospitalar de Lisboa Central, EPE, Lisbon, Portugal"}]},{"given":"Luc","family":"Mouthon","sequence":"additional","affiliation":[{"name":"Service de M\u00e9decine Interne, H\u00f4pital Cochin, Centre de R\u00e9f\u00e9rence Maladies syst\u00e9miques Autoimmunes Rares d\u2019Ile de France, Assistance Publique-H\u00f4pitaux de Paris (AP-HP), Universit\u00e9 Paris Descartes, Paris, France"}]},{"given":"Cecilia","family":"Nalli","sequence":"additional","affiliation":[{"name":"Rheumatology and Clinical Immunology Unit, Civil Hospital, Brescia, Italy"}]},{"given":"Veronique","family":"Ramoni","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy"}]},{"given":"Maria","family":"Tektonidou","sequence":"additional","affiliation":[{"name":"Joint Rheumatology Academic Program, First Department of Propaedeutic Internal Medicine, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece"}]},{"given":"Jacob M","family":"van Laar","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands"}]},{"given":"Stefano","family":"Bombardieri","sequence":"additional","affiliation":[{"name":"University of Pisa, Pisa, Italy"}]},{"given":"Matthias","family":"Schneider","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Universit\u00e4tsklinikum D\u00fcsseldorf, D\u00fcsseldorf, Germany"}]},{"given":"Vanessa","family":"Smith","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Ghent University Hospital, Ghent, Belgium"},{"name":"Department of Internal Medicine, Ghent University, Ghent, Belgium"}]},{"given":"Ana","family":"Vieira","sequence":"additional","affiliation":[{"name":"Liga Portuguesa Contra as Doen\u00e7as Reum\u00e1ticas, N\u00facleo S\u00edndrome de Sj\u00f6gren, Lisbon, Portugal"}]},{"given":"Maurizio","family":"Cutolo","sequence":"additional","affiliation":[{"name":"Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, IRCCS Polyclinic Hospital San Martino, University of Genoa, Genoa, Italy"}]},{"given":"Marta","family":"Mosca","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, AOU Pisana, Pisa, Italy"},{"name":"Rheumatology Unit, University of Pisa, Pisa, Italy"}]},{"given":"Angela","family":"Tincani","sequence":"additional","affiliation":[{"name":"Rheumatology and Clinical Immunology Unit, Civil Hospital, Brescia, Italy"}]}],"member":"239","published-online":{"date-parts":[[2018,10,18]]},"reference":[{"key":"2025090312423385000_4.Suppl_1.e000785.1","unstructured":"The AGREE Collaboration. Appraisal of guidelines for research and evaluation, 2001. Available from: http:\/\/www.agreetrust.org\/"},{"key":"2025090312423385000_4.Suppl_1.e000785.2","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2014.05.001"},{"key":"2025090312423385000_4.Suppl_1.e000785.3","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2012.09037.x"},{"key":"2025090312423385000_4.Suppl_1.e000785.4","doi-asserted-by":"publisher","DOI":"10.1002\/art.33349"},{"key":"2025090312423385000_4.Suppl_1.e000785.5","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2009.03555.x"},{"key":"2025090312423385000_4.Suppl_1.e000785.6","doi-asserted-by":"publisher","DOI":"10.1191\/0961203303lu388oa"},{"key":"2025090312423385000_4.Suppl_1.e000785.7","doi-asserted-by":"publisher","DOI":"10.1136\/ard.2007.070367"},{"key":"2025090312423385000_4.Suppl_1.e000785.8","doi-asserted-by":"publisher","DOI":"10.1136\/ard.2010.130476"},{"key":"2025090312423385000_4.Suppl_1.e000785.9","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2016-211001"},{"key":"2025090312423385000_4.Suppl_1.e000785.10","doi-asserted-by":"publisher","DOI":"10.1177\/0961203310395803"},{"key":"2025090312423385000_4.Suppl_1.e000785.11","first-page":"3","article-title":"Evidence based treatment of the antiphospholipid syndrome II. Optimal anticoagulant therapy for thrombosis","volume":"115","author":"Crowther","year":"2005","journal-title":"Thromb Res"},{"key":"2025090312423385000_4.Suppl_1.e000785.12","doi-asserted-by":"publisher","DOI":"10.1191\/0961203303lu389oa"},{"key":"2025090312423385000_4.Suppl_1.e000785.13","doi-asserted-by":"publisher","DOI":"10.1378\/chest.11-2300"},{"key":"2025090312423385000_4.Suppl_1.e000785.14","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2014.02.003"},{"key":"2025090312423385000_4.Suppl_1.e000785.15","doi-asserted-by":"crossref","first-page":"18","DOI":"10.1016\/j.ejogrb.2016.02.015","article-title":"Pregnancy loss: French clinical practice guidelines","volume":"201","author":"Huchon","year":"2016","journal-title":"Eur J Obstet Gynecol Reprod Biol"},{"key":"2025090312423385000_4.Suppl_1.e000785.16","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2016-209770"},{"key":"2025090312423385000_4.Suppl_1.e000785.17","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2015.01.002"},{"key":"2025090312423385000_4.Suppl_1.e000785.18","doi-asserted-by":"publisher","DOI":"10.1191\/0961203303lu394oa"},{"key":"2025090312423385000_4.Suppl_1.e000785.19","doi-asserted-by":"publisher","DOI":"10.1038\/nrrheum.2015.88"},{"key":"2025090312423385000_4.Suppl_1.e000785.20","doi-asserted-by":"publisher","DOI":"10.1111\/jth.12865"},{"key":"2025090312423385000_4.Suppl_1.e000785.21","doi-asserted-by":"crossref","first-page":"201","DOI":"10.1016\/j.cca.2015.04.033","article-title":"Detection of IgG anti-Domain I beta2 Glycoprotein I antibodies by chemiluminescence immunoassay in primary antiphospholipid syndrome","volume":"446","author":"Meneghel","year":"2015","journal-title":"Clin Chim Acta"},{"key":"2025090312423385000_4.Suppl_1.e000785.22","doi-asserted-by":"publisher","DOI":"10.3899\/jrheum.170044"},{"key":"2025090312423385000_4.Suppl_1.e000785.23","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2013.10.014"},{"key":"2025090312423385000_4.Suppl_1.e000785.24","doi-asserted-by":"publisher","DOI":"10.1002\/art.22663"},{"key":"2025090312423385000_4.Suppl_1.e000785.25","doi-asserted-by":"crossref","unstructured":"Erkan D , Unlu O , Sciascia S , et al . Hydroxychloroquine in the primary thrombosis prophylaxis of antiphospholipid antibody positive patients without systemic autoimmune disease. Lupus 2018;27.doi:10.1177\/0961203317724219","DOI":"10.1177\/0961203317724219"},{"key":"2025090312423385000_4.Suppl_1.e000785.26","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1007\/s12026-016-8812-z","article-title":"Long-term use of hydroxychloroquine reduces antiphospholipid antibodies levels in patients with primary antiphospholipid syndrome","volume":"65","author":"Nuri","year":"2017","journal-title":"Immunol Res"},{"key":"2025090312423385000_4.Suppl_1.e000785.27","doi-asserted-by":"publisher","DOI":"10.3899\/jrheum.161364"},{"key":"2025090312423385000_4.Suppl_1.e000785.28","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2014.01.053"},{"key":"2025090312423385000_4.Suppl_1.e000785.29","doi-asserted-by":"crossref","DOI":"10.1136\/annrheumdis-2016-210485","article-title":"Monocyte type I interferon signature in antiphospholipid syndrome is related to proinflammatory monocyte subsets, hydroxychloroquine and statin use","volume":"75","author":"van den Hoogen","year":"2016","journal-title":"Ann Rheum Dis"},{"key":"2025090312423385000_4.Suppl_1.e000785.30","doi-asserted-by":"crossref","first-page":"e21","DOI":"10.1016\/j.semarthrit.2016.08.002","article-title":"Repeated low-dose courses of rituximab in SLE-associated antiphospholipid syndrome: Data from a tertiary dedicated centre","volume":"46","author":"Emmi","year":"2017","journal-title":"Semin Arthritis Rheum"},{"key":"2025090312423385000_4.Suppl_1.e000785.31","first-page":"877","article-title":"Long-term treatment of antiphospholipid syndrome with intravenous immunoglobulin in addition to conventional therapy","volume":"31","author":"Tenti","year":"2013","journal-title":"Clin Exp Rheumatol"},{"key":"2025090312423385000_4.Suppl_1.e000785.32","doi-asserted-by":"crossref","DOI":"10.1097\/MD.0000000000000143","article-title":"Efficacy of eculizumab in a patient with immunoadsorption-dependent catastrophic antiphospholipid syndrome: a case report","volume":"93","author":"Kronbichler","year":"2014","journal-title":"Medicine"},{"key":"2025090312423385000_4.Suppl_1.e000785.33","doi-asserted-by":"crossref","unstructured":"Pengo V , Denas G , Zoppellaro G , et al . Rivaroxaban vs warfarin in high-risk patients with antiphospholipid syndrome. Blood 2018. doi:10.1182\/blood-2018-04-848333","DOI":"10.1182\/blood-2018-04-848333"},{"key":"2025090312423385000_4.Suppl_1.e000785.34","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2015.01.012"},{"key":"2025090312423385000_4.Suppl_1.e000785.35","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-02-339234"},{"key":"2025090312423385000_4.Suppl_1.e000785.36","doi-asserted-by":"crossref","first-page":"14","DOI":"10.1016\/j.jri.2016.03.004","article-title":"Apheresis and intravenous immunoglobulins used in addition to conventional therapy to treat high-risk pregnant antiphospholipid antibody syndrome patients. A prospective study","volume":"115","author":"Ruffatti","year":"2016","journal-title":"J Reprod Immunol"},{"key":"2025090312423385000_4.Suppl_1.e000785.37","doi-asserted-by":"publisher","DOI":"10.1172\/JCI86957"},{"key":"2025090312423385000_4.Suppl_1.e000785.38","doi-asserted-by":"crossref","first-page":"464","DOI":"10.1002\/art.37759","article-title":"A pilot open-label phase II trial of rituximab for non-criteria manifestations of antiphospholipid syndrome","volume":"65","author":"Erkan","year":"2013","journal-title":"Arthritis Rheum"},{"key":"2025090312423385000_4.Suppl_1.e000785.39","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.jaut.2018.05.007","article-title":"The diagnosis and clinical management of the catastrophic antiphospholipid syndrome: a comprehensive review","volume":"92","author":"Cervera","year":"2018","journal-title":"J Autoimmun"},{"key":"2025090312423385000_4.Suppl_1.e000785.40","doi-asserted-by":"publisher","DOI":"10.1002\/art.34440"},{"key":"2025090312423385000_4.Suppl_1.e000785.41","doi-asserted-by":"crossref","unstructured":"Legault K , Schunemann H , Hillis C , et al . McMaster RARE-Bestpractices clinical practice guideline on diagnosis and management of the catastrophic antiphospholipid syndrome. J Thromb Haemost 2018:1656\u201364. doi:10.1111\/jth.14192","DOI":"10.1111\/jth.14192"}],"container-title":["RMD Open"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/rmdopen-2018-000785","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,9,3]],"date-time":"2025-09-03T19:42:43Z","timestamp":1756928563000},"score":1,"resource":{"primary":{"URL":"https:\/\/rmdopen.bmj.com\/lookup\/doi\/10.1136\/rmdopen-2018-000785"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,10]]},"references-count":41,"journal-issue":{"issue":"Suppl 1","published-online":{"date-parts":[[2018,10,18]]},"published-print":{"date-parts":[[2018,10]]}},"alternative-id":["10.1136\/rmdopen-2018-000785"],"URL":"https:\/\/doi.org\/10.1136\/rmdopen-2018-000785","relation":{},"ISSN":["2056-5933"],"issn-type":[{"value":"2056-5933","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,10]]}}}