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.
- antiphospholipid antibodies
- antiphospholipid syndrome
- systemic lupus erythematosus
- pregnancy morbidity
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Handling editor Josef S Smolen
AT and MMW contributed equally.
Correction notice This article has been corrected since it published Online First. The discussion section has been amended and the title in table one.
Contributors Full-text review, data abstraction and risk of bias assessments were performed by LA and ML, and independently double-checked by MGT and MMW. MMW supervised the methodology of the SLR and prepared the evidence report. MGT and AT prepared the first draft of recommendations, and all authors have participated in the discussion and formulation of recommendations. MGT supervised the project and drafted the manuscript. All authors reviewed the manuscript and approved its final version.
Funding This project was funded by the European League Against Rheumatism. MMW was funded by the Intramural Research Program, National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH.
Competing interests None declared.
Patient consent for publication Not required.
Provenance and peer review Not commissioned; externally peer reviewed.
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