Abstract
Antiphospholipid antibody syndrome (APS) requires long-term anticoagulation to prevent recurrent thrombosis. Direct oral anticoagulants (DOACs) have been increasingly used in APS patients, but contradictory guidelines recommendations on their use do exist. We performed a systematic review of literature including studies investigating the role of DOACs in APS patients. At this aim, PubMed and Cochrane databases were searched according to PRISMA guidelines. We identified 14 studies which investigated the use of DOACs in patients with APS, of which 3 randomized clinical trials (RCTs), 1 post-hoc analysis of 3 RCTs, 7 case series and 3 cohort studies (2 prospective and 1 retrospective). Among DOACs, rivaroxaban was the most used (n = 531), followed by dabigatran (n = 90) and apixaban (n = 46). Regarding guidelines indications, the 2019 European Society of Cardiology (ESC) and American Society of Hematology (ASH) guidelines recommend against the use of DOACs in all APS patients. The European League Against Rheumatism (EULAR), British Society for Haematology (BSH), and International Society on Thrombosis and Haemostasis (ISTH) guidance provided more detailed indications stating that warfarin should be the first-choice treatment but DOACs may be considered in patients (1) already on a stable anticoagulation with a DOAC, (2) with low-quality anticoagulation by warfarin, (3) unwilling/unable to undergo INR monitoring, (4) with contraindications or serious adverse events under warfarin. Patients with arterial APS or triple positivity should be treated with warfarin while venous APS with single or double positivity may be candidate to DOACs, but high-quality studies are needed.
Highlights
The incidence and prevalence of antiphospholipid antibody syndrome (APS) are difficult to estimate given that the definition of APS has evolved over the years making epidemiological studies published before 2000 not adhering to the new classification criteria (1)
APS is diagnosed in case of persistent positivity of anticardiolipin, anti-β2 glycoprotein I (β2GPI), and lupus anticoagulant (LAC) assays, which play a pathogenic role in determining the risk of thrombotic events (3)
We identified 14 studies which investigated the use of Direct oral anticoagulants (DOACs) in patients with APS, of which 3 randomized clinical trials (RCTs), 1 post-hoc analysis of 3 RCTs, 7 case series and 3 cohort studies (2 prospective and 1 retrospective) (Table 2)
Summary
The incidence and prevalence of antiphospholipid antibody syndrome (APS) are difficult to estimate given that the definition of APS has evolved over the years making epidemiological studies published before 2000 not adhering to the new classification criteria (1). A large recent study estimated an incidence of APS of 2.1 per 100,000 per year and a prevalence of 50 per 100,000 inhabitants (2)
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