EULAR recommendations for the management of antiphospholipid syndrome in adults

Maria G. Tektonidou, Laura Andreoli, Marteen Limper, Zahir Amoura, Ricard Cervera, Nathalie Costedoat-Chalumeau, Maria Jose Cuadrado, Thomas Dörner, Raquel Ferrer-Oliveras, Karen Hambly, Munther A. Khamashta, Judith King, Francesca Marchiori, Pier Luigi Meroni, Marta Mosca, Vittorio Pengo, Luigi Raio, Guillermo Ruiz-Irastorza, Yehuda Shoenfeld, Ljudmila StojanovichElisabet Svenungsson, Denis Wahl, Angela Tincani, Michael M. Ward

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish
Pages (from-to)1296-1304
Number of pages9
JournalAnnals of the Rheumatic Diseases
Volume78
Issue number10
DOIs
Publication statusPublished - Oct 1 2019

Keywords

  • antiphospholipid antibodies
  • antiphospholipid syndrome
  • management
  • pregnancy morbidity
  • recommendations
  • systemic lupus erythematosus
  • thrombosis

ASJC Scopus subject areas

  • Immunology and Allergy
  • Rheumatology
  • Immunology
  • Biochemistry, Genetics and Molecular Biology(all)

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