EULAR recommendations for the management of antiphospholipid syndrome in adults

• Published in: Annals of the rheumatic diseases Vol. 78; no. 10; pp. 1296 - 1304
• Main Authors: Tektonidou, Maria G, Andreoli, Laura, Limper, Marteen, Amoura, Zahir, Cervera, Ricard, Costedoat-Chalumeau, Nathalie, Cuadrado, Maria Jose, Dörner, Thomas, Ferrer-Oliveras, Raquel, Hambly, Karen, Khamashta, Munther A, King, Judith, Marchiori, Francesca, Meroni, Pier Luigi, Mosca, Marta, Pengo, Vittorio, Raio, Luigi, Ruiz-Irastorza, Guillermo, Shoenfeld, Yehuda, Stojanovich, Ljudmila, Svenungsson, Elisabet, Wahl, Denis, Tincani, Angela, Ward, Michael M
• Format: Journal Article
• Language: English
• Published: England BMJ Publishing Group Ltd and European League Against Rheumatism 01.10.2019; BMJ Publishing Group LTD
• Subjects: Adult; Antagonists; Antibodies, Antiphospholipid - blood; Anticoagulants - therapeutic use; Antiphospholipid antibodies; Antiphospholipid syndrome; Antiphospholipid Syndrome - blood; Antiphospholipid Syndrome - immunology; Aspirin; Autoimmune diseases; Cardiovascular diseases; Clinical medicine; Dosage; Female; Heparin; Humans; Hydroxychloroquine; Hälso- och sjukvårdsorganisation, hälsopolitik och hälsoekonomi; Hälsovetenskap; Immunoglobulins; Internal medicine; Klinisk medicin; Laboratories; Literature reviews; Lupus; Male; Management; Medicin och hälsovetenskap; Medicine; Miscarriage; Molecular weight; Obstetrics; Patients; Practice Guidelines as Topic; Prednisolone; Pregnancy; Pregnancy complications; Pregnancy Complications - blood; Pregnancy Complications - immunology; pregnancy morbidity; Recommendation; recommendations; Reumatologi och inflammation; Rheumatology - standards; Risk Factors; Systemic lupus erythematosus; Task forces; Thromboembolism; Thrombosis; Venous Thrombosis - immunology; Vitamin K; Womens health; antiphospholipid syndrome; systemic lupus erythematosus; thrombosis; management; pregnancy morbidity; recommendations; antiphospholipid antibodies
• ISSN: 0003-4967; 1468-2060; 1468-2060
• DOI: 10.1136/annrheumdis-2019-215213

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.