Abstract
The antiphospholipid syndrome (APS) is characterized by thrombosis and/or pregnancy morbidity with the persistent presence of antiphospholipid antibodies (aPLs). Laboratory criteria for the classification of APS include the detection of lupus anticoagulant (LAC), anti-cardiolipin (aCL) antibodies and anti-β2glycoprotein I (aβ2GPI) antibodies. Clinical criteria for the classification of thrombotic APS include venous and arterial thrombosis, along with microvascular thrombosis. Several aPLs, including LAC, aβ2GPI and anti-phosphatidylserine/prothrombin antibodies (aPS/PT) have been associated with arterial thrombosis. The Von Willebrand Factor (VWF) plays an important role in arterial thrombosis by mediating platelet adhesion and aggregation. Studies have shown that aPLs antibodies present in APS patients are able to increase the risk of arterial thrombosis by upregulating the plasma levels of active VWF and by promoting platelet activation. Inflammatory reactions induced by APS may also provide a suitable condition for arterial thrombosis, mostly ischemic stroke and myocardial infarction. The presence of other cardiovascular risk factors can enhance the effect of aPLs and increase the risk for thrombosis even more. These factors should therefore be taken into account when investigating APS-related arterial thrombosis. Nevertheless, the exact mechanism by which aPLs can cause thrombosis remains to be elucidated.
Highlights
The antiphospholipid syndrome (APS) is characterized by thrombosis and/or pregnancy morbidity with the persistent presence of antiphospholipid antibodies
The results showed that lupus anticoagulant (LAC), aCL, anti-β2glycoprotein I (aβ2GPI), aPT and aPS were all significantly correlated with arterial thrombosis, but especially LAC and aCL had the highest odds ratio for arterial thrombosis compared to the other aPLs [100]
Due to the physiological properties of Von Willebrand Factor (VWF), platelet recruitment via VWF is more likely to appear in the region of high shear force, for example, in a coronary artery
Summary
The antiphospholipid syndrome (APS) is characterized by thrombosis and/or pregnancy morbidity with the persistent presence of antiphospholipid antibodies (aPLs). Active VWF is able to bind to the platelet-receptor glycoprotein-Iba (GPIba) and plays an important role in arterial thrombosis by mediating platelet adhesion and aggregation [4]. Aβ2GPI antibodies are able to inhibit the binding of VWF to β2GPI, thereby increasing the amount of active VWF present in the circulation. This indicates that β2GPI may act as a regulator of VWF-platelet interactions that are altered in the presence of aβ2GPI antibodies. This manuscript reviews existing literature on the role of VWF and platelets in the occurrence of arterial thrombosis in APS. Possible mechanisms could be the activation of the endothelium, secretion of VWF, assembly of hyper-adhesive VWF strings and fibers, reduced cleavage by ADAMTS13, as well as adhesion and deposition of VWF-platelet-thrombi in the vasculature [42]
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