PT - JOURNAL ARTICLE AU - Maria G Tektonidou AU - Laura Andreoli AU - Marteen Limper AU - Zahir Amoura AU - Ricard Cervera AU - Nathalie Costedoat-Chalumeau AU - Maria Jose Cuadrado AU - Thomas Dörner AU - Raquel Ferrer-Oliveras AU - Karen Hambly AU - Munther A Khamashta AU - Judith King AU - Francesca Marchiori AU - Pier Luigi Meroni AU - Marta Mosca AU - Vittorio Pengo AU - Luigi Raio AU - Guillermo Ruiz-Irastorza AU - Yehuda Shoenfeld AU - Ljudmila Stojanovich AU - Elisabet Svenungsson AU - Denis Wahl AU - Angela Tincani AU - Michael M Ward TI - EULAR recommendations for the management of antiphospholipid syndrome in adults AID - 10.1136/annrheumdis-2019-215213 DP - 2019 May 15 TA - Annals of the Rheumatic Diseases PG - annrheumdis-2019-215213 4099 - http://ard.bmj.com/content/early/2019/06/14/annrheumdis-2019-215213.short 4100 - http://ard.bmj.com/content/early/2019/06/14/annrheumdis-2019-215213.full AB - 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.