@article{3000358,
    title = "EULAR recommendations for the management of antiphospholipid syndrome in adults",
    author = "Tektonidou, M.G. and Andreoli, L. and Limper, M. and Amoura, Z. and Cervera, R. and Costedoat-Chalumeau, N. and Cuadrado, M.J. and Dörner, T. and Ferrer-Oliveras, R. and Hambly, K. and Khamashta, M.A. and King, J. and Marchiori, F. and Meroni, P.L. and Mosca, M. and Pengo, V. and Raio, L. and Ruiz-Irastorza, G. and Shoenfeld, Y. and Stojanovich, L. and Svenungsson, E. and Wahl, D. and Tincani, A. and Ward, M.M.",
    journal = "Annals of the Rheumatic Diseases",
    year = "2019",
    volume = "78",
    number = "10",
    pages = "1296-1304",
    publisher = "BMJ Publishing Group",
    issn = "0003-4967, 1468-2060",
    doi = "10.1136/annrheumdis-2019-215213",
    keywords = "acetylsalicylic acid;  antivitamin K;  hydroxychloroquine;  low molecular weight heparin;  phospholipid antibody;  prednisolone;  anticoagulant agent;  phospholipid antibody, adult;  antiphospholipid syndrome;  artery thrombosis;  Article;  cardiovascular risk;  counseling;  evidence based medicine;  high risk population;  human;  international normalized ratio;  lifestyle;  low drug dose;  patient compliance;  patient education;  pregnancy;  priority journal;  prophylaxis;  screening;  systematic review (topic);  systemic lupus erythematosus;  vein thrombosis;  antiphospholipid syndrome;  blood;  female;  immunology;  male;  practice guideline;  pregnancy complication;  rheumatology;  risk factor;  vein thrombosis, Adult;  Antibodies, Antiphospholipid;  Anticoagulants;  Antiphospholipid Syndrome;  Female;  Humans;  Male;  Practice Guidelines as Topic;  Pregnancy;  Pregnancy Complications;  Rheumatology;  Risk Factors;  Venous Thrombosis",
    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. © © Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and permissions. Published by BMJ."
}