Antiphospholipid syndrome (APS) is a complex autoimmune disease often connected to systemic lupus erythematosus. Main features are specific antiphospholipid antibodies (aPL), thrombosis (arterial, venous or microvascular) and/or pregnancy complications and failure. The involved antibodies are directed against plasma proteins such as beta-2-glycoprotein1 (β2GPI) or prothrombin which depends on negatively charged phospholipids. Antibody- β2GPI complexes bind to a variety of receptors on different cell types, including endothelial cells, platelets, monocytes and trophoblasts and may trigger intracellular signalling and inflammatory responses. There is increased expression of tissue factor on monocytes and endothelial cells, interference in protein C anticoagulant pathways, inhibition of fibrinolysis and inhibition of annexin V binding to phospholipids. Pregnancy failure may be due to thrombosis in the placental bed, although alternative mechanism may explain the tendency to very early losses prior to placentation.  Clotting tests such as activated partial thromboplastin time (aPTT) and diluted Russell`s viper venom test (dRVVT) show a prolonged time for coagulation despite prothrombotic state. Clotting test could be misleading so clinical judgement is mandatory.

APS has been described secondary if there is an associated autoimmune disorder, such as systemic lupus erythematosus (SLE) or rheumatoid arthritis, and primary if not. There is high morbidity and mortality in the patients during perioperative period. The surgical experience with APS patients mainly relies on few case reports describing one or two patients.  The risk of thrombosis and bleeding are present in patients with APS. Early institution of anticoagulation with antiplatelets and other anticoagulants is considered as safe treatment.

In addition to thrombosis and pregnancy morbidity, patient with APS may have other clinical associations e.g. Myocardial ischemia/infarction, heart valve disease, thrombocytopenia, ischemic strokes, chorea, livedo reticularis/ racemosa, nephropathy, transverse myelopathy, migraine.