Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized
by a persistently high titer of antiphospholipid antibodies (aPLs). In addition to pregnancy
morbidity, arterial and/or venous thrombosis is another clinical feature of APS. Regardless
of the type of APS, the thrombi formed by the induction of aPLs can lead to deep
vein thrombosis, pulmonary embolism, myocardial infarction, stroke and gangrene. Although
the concept of APS was introduced approximately 32 years ago, its thrombogenic
pathophysiology is still unclear. Therefore, patients are treated with anticoagulant and/or
antiplatelet regimens just as in other thrombotic disorders even though the thrombotic
pathophysiology is mainly aPLs-mediated. In this review, we provided an update of the
cellular, auto-immune and genetic factors known to play important roles in the generation
of thrombi. Current successful regimens are also outlined along with potential emerging
treatment strategies that may lead to the optimum management of thrombotic APS patients.
Keywords: Antiphospholipid syndrome, antiphospholipid antibodies, thrombosis, pathophysiology, management.
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