Abstract
Antiphospholipid syndrome (APS), is an acquired autoimmune disorder characterised by thrombosis, pregnancy morbidity, and the presence of antiphospholipid antibodies (aPL). Although venous thromboembolism is the most common manifestation, thrombotic events in APS may also occur in virtually any vascular bed, with cerebral circulation being the arterial territory most commonly affected. As APS is a heterogeneous condition, its management should be tailored with a patient-centred approach based on individual risk assessment, which includes the aPL profile, concomitant auto-immune diseases, and traditional cardiovascular risk factors. Although literature data are conflicting regarding primary prophylaxis, there is some evidence indicating that antiplatelet agents may reduce the risk of a first thrombotic event in individuals with a high-risk profile. In patients with thrombotic APS, current evidence-based guidelines recommend lifelong vitamin K antagonists (VKAs), preferably warfarin. The optimal intensity of anticoagulation following arterial thrombosis remains controversial. Arterial thrombosis should be treated either with high-intensity warfarin at a target INR > 3.0, or low-dose aspirin (LDA) combined with moderate-intensity warfarin (INR 2.0–3.0). It is recommended to avoid direct oral anticoagulants (DOACs) in patients with high-risk APS, mainly those with triple-positive PL and previous arterial events. They would only be used exceptionally in selected patients with low-risk venous thromboembolism (VTE). In low-risk VTE patients currently treated with a DOAC due to warfarin intolerance or a previous unstable International Normalized Ratio on warfarin, the decision of continuing DOACs would be taken in carefully selected patients. In women with obstetric APS, the combination therapy with LDA plus heparin remains the conventional strategy.
Highlights
Antiphospholipid syndrome (APS) is an acquired autoimmune disorder first described in 1983, characterised by thrombosis and pregnancy morbidity, in the setting of persistently positive antiphospholipid antibodies: lupus anticoagulant (LA), anti-beta 2 glycoprotein 1, and anticardiolipin antibodies
CAPS is still associated with a significant mortality rate that might reach 30%; it represents less than 1% of cases of APS [25]
The role of low-dose aspirin (LDA) for primary prevention in asymptomatic aPL carriers was corroborated by a meta-analysis including 11, mainly observational, studies, with a more significant effect in patients with arterial thrombosis
Summary
Antiphospholipid syndrome (APS) is an acquired autoimmune disorder first described in 1983, characterised by thrombosis (in the venous, arterial, or microvascular circulation) and pregnancy morbidity, in the setting of persistently positive antiphospholipid antibodies (aPL): lupus anticoagulant (LA), anti-beta 2 glycoprotein 1 (anti-ß2GP1), and anticardiolipin antibodies (aCL). Thrombosis events after pregnancy morbidity related to APS occurred in 63.5% of cases Those women were more likely to be younger, had more cardiovascular risk factors, superficial venous thrombosis, and heart valve disease than patients with only obstetric APS [9]. Score (aGAPSS) is a validated tool, including both aPL and conventional cardiovascular risk factors, predicting the likelihood of developing new thrombosis in an APS patient [10]. In some cases, both thrombotic and obstetric APS may coexist in the same woman (the so-called full-blown APS) [11]. We will summarize the current best evidence on the management of patients with APS, with a special focus on the use of direct oral anticoagulants
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