EULAR recommendations for the management of antiphospholipid syndrome in adults

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.

Prevalence of confirmed antiphospholipid syndrome in 18‐50 years unselected patients with first unprovoked venous thromboembolism

“…With an APS prevalence reaching 9% in young patients with a first unprovoked VTE, systematic assessment for the presence of antiphospholipid antibodies (APLA) in this patient group may be warranted. Recent European League Against Rheumatism (EULAR) guidelines suggest avoiding direct oral anticoagulant (DOAC) use in patients with APS due to a lack of effectiveness in triple‐positive APS patients . These recommendations are based on the result of the TRAPS trial, which demonstrated that among patients with triple‐positive APS, the risk of recurrent thrombosis was six‐fold higher on rivaroxaban as compared with warfarin (19% versus 3%, P = .01.).…”

“…Recent European League Against Rheumatism (EULAR) guidelines suggest avoiding direct oral anticoagulant (DOAC) use in patients with APS due to a lack of effectiveness in triple-positive APS patients. 13 These recommendations are based on the result of the TRAPS trial, which demonstrated that among patients with triple-positive APS, the risk of recurrent thrombosis was six-fold higher on rivaroxaban as compared with warfarin (19% versus 3%, P = .01.). Importantly, all of the recurrent thrombotic events observed on rivaroxaban were either myocardial infarction or stroke, some occurring in patients treated for VTE.…”

The antiphospholipid syndrome – often overlooked cause of vascular occlusions?

“…Using combined treatment including LDA (75–100 mg day −1 ) and LMWH or unfractionated heparin in prophylactic, intermediate or therapeutic dose, depending on risk profile, has led to live births in 70 % of women with previous aPL‐related pregnancy complications . Therefore, this treatment regimen is recommended as a current standard of care treatment in patients with obstetric APS …”

“…The antiphospholipid syndrome / E. Svenungsson dose, depending on risk profile, has led to live births in 70 % of women with previous aPL-related pregnancy complications [206]. Therefore, this treatment regimen is recommended as a current standard of care treatment in patients with obstetric APS [177,178] In severe cases with recurrent late pregnancy morbidities, despite treatment with LDA and LMWH, low dose steroids (10 mg Prednisolone) and hydroxychloquine may be added [147]. In severe treatmentresistant cases intravenous gamma globulin (IvIg) or repeated plasma exchange/apheresis could, based on small case series with favourable outcomes, be considered [207].…”

Clinical and laboratory practice for lupus anticoagulant testing: An International Society of Thrombosis and Haemostasis Scientific and Standardization Committee survey

“…It may also affect the type of oral anticoagulant that is prescribed. [2][3][4][5] In addition, it identifies women who require higher than standard prophylactic-dose anticoagulation with low molecular weight heparin (LMWH) during pregnancy, [6][7][8] and who also require low-dose aspirin and monitoring for placental insufficiency, 9 the latter to guide optimal timing of delivery, reducing the risk of perinatal morbidity and mortality. Approximately 50% of APS patients have LA alone, 10 with LA detection therefore critical for APS diagnosis in these patients.…”