Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by venous, arterial or microvascular thrombosis or obstetrical events in the presence of persistently positive antiphospholipid antibodies and constitutes a major cause of cardiovascular events in young people. Τhis review aims to highlight the pathophysiology of cardiovascular complications in patients with APS and possible treatment options.
Patients with APS have endothelial dysfunction, accelerated proliferation and hyperplasia, atherogenesis, platelet activation and aggregation, inflammatory products secretion and coagulation-fibrinolytic dysregulation. Cardiovascular complications constitute accelerated atherosclerosis, acute coronary syndrome, Libman- Sachs endocarditis, cardiomyopathy or intracardiac thombi. Moreover, pulmonary hypertension and peripheral microvascular dysfunction are common findings for these patients.
Management of these patients is not well documented. The role of primary thrombosis prevention remains controversial in individuals with positive antiphospholipid antibodies. Treatment of traditional cardiovascular risk factors according to current guidelines for prevention of cardiovascular disease in the general population is recommended for primary prevention of APS. Anticoagulation therapy with unfractionated or low-molecular-weight heparin overlapped with a vitamin K antagonist remains the mainstay of treatment for APS patients with venous thrombosis, whereas direct oral anticoagulants are not yet recommended. Data are scarce regarding secondary arterial thrombosis prevention and it is not clear whether dual or triple antithrombotic therapy is necessary. To date, it is recommended to follow current guidelines for the management of acute coronary syndrome in the general population. New treatment targets are promising options for patients with catastrophic APS.