[Antiphospholipid syndrome: diagnosis and management]

Abstract

Antiphospholipid syndrome (APS) is an acquired thrombophilia associated with autoimmunity and is a syndrome that should always be considered when examining patients with thrombosis and pregnancy complications. As per the Sydney criteria, a diagnosis can be established with at least one clinical finding, such as arteriovenous thrombosis and the presence of at least one of the antiphospholipid antibodies (aPL), such as anticardiolipin antibodies (aCL). Moreover, phosphatidylserine-dependent anti-prothrombin antibody and anti-b2GPI-domain 1 antibody are correlated with APS manifestations and enable APS diagnosis. In addition to the inhibition of physiological coagulation inhibitors, such as protein C, the activation of vascular endothelial cells and complement activation by aPL is presumed to be the thrombus mechanism of APS. The mainstay of treatment is anticoagulant therapy with warfarin. For treating APS that has developed by arterial thrombosis, antiplatelet agent alone or in combination with warfarin is considered. In the triple positive aPL (aCL, anti-b2GPI antibodies, and lupus anticoagulant are detected at the same time) cases, sufficient anticoagulant therapy is required. Direct oral anticoagulants are not recommended in cases of triple positive aPL or arterial thrombosis. For patients with catastrophic APS, heparin therapy, plasmapheresis, and steroid pulse therapy are recommended.

Keywords: Anti-β2GPI antibodies; Anticardiolipin antibodies; Lupus anticoagulant; Warfarin.