Hamostaseologie 2020; 40(01): 031-037
DOI: 10.1055/s-0040-1701473
Review Article
Georg Thieme Verlag KG Stuttgart · New York

Treatment of Vascular Thrombosis in Antiphospholipid Syndrome: An Update

Lida Kalmanti
1  Coagulation Center and Cardiology-Angiology Center Bethanien Hospital (CCB), Frankfurt, Germany
,
Edelgard Lindhoff-Last
1  Coagulation Center and Cardiology-Angiology Center Bethanien Hospital (CCB), Frankfurt, Germany
› Author Affiliations
Further Information

Publication History

04 September 2019

27 November 2019

Publication Date:
28 January 2020 (online)

Abstract

The antiphospholipid syndrome (APS) is an acquired autoimmune disorder associated with arterial, venous, or microvascular thrombosis and/or pregnancy complications mainly in young age. The diagnosis is made by the persistent detection of anticardiolipin antibodies, β2-glycoprotein I antibodies (β2GPIA), and/or lupus anticoagulants (LAs) for at least 12 weeks. Patients should present with at least one clinical and one laboratory criterion. Patients presenting with all three types of antibodies and vascular events are high-risk patients and should receive vitamin K antagonists (VKAs) as long as the antibodies persist. In patients with prior arterial thrombosis, VKA with or without low-dose aspirin is the current treatment of choice. The international normalized ratio (INR) should be between 2 and 3 although in some cases keeping the target INR above 3 may be necessary. Patients with venous thrombosis and negative LA may alternatively be treated with direct oral anticoagulants although more data are needed. Minimizing vascular risk factors is always necessary in APS patients. Aspirin can be given as primary prevention in asymptomatic patients with positive antiphospholipid antibodies without thrombosis or pregnancy complications especially when additional vascular risk factors are present. Catastrophic APS occurs in less than 1% of APS patients and presents as a thrombotic storm. Early use of a combined triple therapy such as anticoagulation, plasma exchange, and steroids with either or not addition of immunoglobulins is important to reduce mortality.