Anticoagulant Therapy in Patients with Antiphospholipid Syndrome

Abstract

Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by the persistent positivity of antiphospholipid antibodies (aPLA) together with thrombosis or obstetrical complications. Despite their recognized predominant role, aPLA are not sufficient to induce the development of thrombosis and a second hit has been proposed to be necessary. The mainstay of treatment of APS is anticoagulant therapy. However, its optimal intensity in different presentations of the disease remains undefined. Moreover, decision on which patients with aPLA would benefit from an antithrombotic prophylaxis and its optimal intensity are challenging because of the lack of stratification tools for the risk of thrombosis. Finally, decision on the optimal type of anticoagulant drug is also complex because the central pathway responsible for the development of thrombosis is so far unknown and should be carried out on an individual basis after a careful evaluation of the clinical and laboratory features of the patient. This review addresses the epidemiology, physiopathology, diagnosis and management of thrombosis and obstetrical complications in APS, with a special focus on the role of direct oral anticoagulants.

Keywords: anticoagulation; antiphospholipid antibodies; antiphospholipid syndrome; obstetrical complications; thrombosis.

Figure 1

Classification criteria of antiphospholipid syndrome. LA, lupus anticoagulant; aCL anti-cardiolipin antibodies; aβ2GPI, anti-beta-2-glicoprotein I antibodies; aPS/PT, phosphatidylserine/prothrombin complex antibodies; APS, antiphospholipid syndrome. * detected on two or more occasions at least 12 weeks apart. ** clinical relevance is currently debated.

Figure 2

Recommendations for the management of antithrombotic prophylaxis and treatment in antiphospholipid syndrome. APS, antiphospholipid syndrome; aPLA, antiphospholipid antibodies; LDA, low dose aspirin; LMWH, low molecular weight heparin; VTE, venous thromboembolism; VKA, vitamin K antagonist; INR international normalized ratio; LA, lupus anticoagulant; CV cardiovascular. APS, antiphospholipid syndrome; aPLA, antiphospholipid antibodies; LDA, low dose aspirin; LMWH, low molecular weight heparin; VTE, venous thromboembolism; VKA, vitamin K antagonist; INR international normalized ratio; LA, lupus anticoagulant. * postoperative periods, lower limb fracture, immobilization, hospitalization, central venous catheter, pregnancy/puerperium. † DOAC (dabigatran preferred) can be considered in patients with single or double aPLA positivity (2019 EULAR; 2020 BSH; 16th International Congress on Antiphospholipid Antibodies Task Force Report on Antiphospholipid Syndrome Treatment Trends).