EULAR recommendations for the management of antiphospholipid syndrome in adults
Tektonidou MG(1), Andreoli L(2), Limper M(3), Amoura Z(4), Cervera R(5), Costedoat-Chalumeau N(6), Cuadrado MJ(7), Dörner T(8), Ferrer-Oliveras R(9), Hambly K(10), Khamashta MA(11), King J(12), Marchiori F(13), Meroni PL(14), Mosca M(15), Pengo V(16), Raio L(17), Ruiz-Irastorza G(18), Shoenfeld Y(19), Stojanovich L(20), Svenungsson E(21), Wahl D(22), Tincani A#(2), Ward MM#(23).
(1) First Department of Propaedeutic Internal Medicine, Joint Rheumatology program, National and Kapodistrian University of Athens, Athens, Greece
(2) Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy.
(3) Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, The Netherlands.
(4) Sorbonne University, French National Center for SLE and Aps, Service de Medecine Interne 2, InstitutE3M, Pitié Salpétrière, Paris, France.
(5) Autoimmune Diseases, Hospital Clinic, IDIBAPS, University of Barcelona, Barcelona, Spain.
(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) Rheumatology Department, Clinica Universidad de Navarra, Madrid, Spain.
(8) Department of Med/Rheumatology and Clinical Immunology, Charite University Hospital, Berlin, Germany.
(9) Obstetrics and Gynecology Department and Systemic Diseases Research Unit, Vall ďHebron Research Institute-VHIR, Barcelona, Spain.
(10) School of Sport and Exercise Sciences, University of Kent, Chatham, UK.
(11) Rheumatology Department, Dubai Hospital, Dubai, United Arab Emirates.
(12) EULAR PARE Patient Research Partner, London, UK.
(13) EULAR PARE Patient Research Partner, Rome, Italy.
(14) MaACR, Immunorheumatology Research Laboratory, Istituto Auxologico Italiano, Milan, Italy.
(15) Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.
(16) Department of Cardiac Thoracic and Vascular Sciences and Public Health, University of Padova, Padua, Italy.
(17 Department of Obstetrics and Gynaecology, University Hospital of Bern, Inselspital, Bern, Switzerland.
(18) Autoimmune Diseases Unit, Hospital Universitario Cruces, Barakaldo, Spain.
(19) Zabludowicz Center for Autoimmune Diseases, Sheba Medical Center, Tel-Aviv University, Israel.
(20) Bezhanijska Kosa, Belgrade University, Belgrade, Serbia.
(21) Department of Medicine, Solna, Rheumatology Unit, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden.
(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) Intramural Research Program, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, Maryland, USA.
# Contributed equally
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.
CITA DEL ARTÍCULO Ann Rheum Dis. 2019 Oct;78(10):1296-1304. doi: 10.1136/annrheumdis-2019-215213. Epub 2019 May 15