Abstract
Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by a presence of antiphospholipid antibodies and heterogeneous clinical manifestations, dominated by thrombosis and/or pregnancy complications. The most frequent thrombotic event is venous thromboembolism, followed by ischemic stroke.
The treatment in acute phase does not differ from the cases without APS. However, because of high risk of recurrence, secondary antithrombotic prophylaxis is of critical importance.
Anticoagulation represents the cornerstone of therapy, including the cases of arterial thrombosis (even after an intervention in arterial bed). Novel (direct) anticoagulants are not recommended in APS, based on the recent data.
Warfarin remains the first-choice therapy, usually with target INR (international normalized ratio) 2-3, in the patients with extremely high thrombotic risk or in the case of recurrent thrombosis despite therapy, more intensive anticoagulation (target INR 3-4) or combination with acetylsalicylic acid is necessary. The interruption of full anticoagulation (e.g. due to planned surgery) must be minimalized and adequate thromboprophylaxis with low-molecular-weight heparin provided.
APS is very complex and requires an interdisciplinary approach.