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Semin Thromb Hemost 2023; 49(03): 295-304
DOI: 10.1055/s-0042-1760333
Review Article

Evolution of Antiphospholipid Syndrome

Deepa R.J. Arachchillage
1   Department of Immunology and Inflammation, Centre for Haematology, Imperial College London, London, United Kingdom
2   Department of Haematology, Imperial College Healthcare NHS Trust, London, United Kingdom
,
Charis Pericleous
3   National Heart and Lung Institute, Imperial College London, London, United Kingdom
› Author Affiliations Funding This article is not funded by any external sources. However, D.J A is funded by MRC UK (MR/V037633/1) and C.P. is funded by Versus Arthritis (21223) and the Imperial College-Wellcome Trust Institutional Strategic Support Fund.
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Abstract

Antiphospholipid syndrome (APS) is an autoimmune prothrombotic disease characterized by thrombosis and/or pregnancy complications caused by antiphospholipid antibodies (aPL). The history of APS can be traced back to observations made during screening programs for syphilis conducted in the mid-20th century, with identification of patients with the so-called biological false-positive serological reactions for syphilis. Initial observation linking aPL with recurrent miscarriages was first reported more than 40 years ago. Since then, our understanding of the pathogenesis and management of APS has evolved markedly. Although APS is an autoimmune disease, anticoagulation mainly with vitamin K antagonists (VKAs) rather than immunomodulation, is the treatment of choice for thrombotic APS. Direct acting oral anticoagulants are inferior to VKAs, especially those with triple-positive APS and arterial thrombosis. Inflammation, complement activation, and thrombosis in the placenta may contribute to pathogenesis of obstetric APS. Heparin, mainly low-molecular-weight heparin, and low-dose aspirin represent the treatments of choice for women with obstetric complications. Increasingly, immunomodulatory agents such as hydroxychloroquine for thrombotic and obstetric APS are being used, especially in patients who are refractory to present standard treatment.

Keywords

antiphospholipid syndrome - antiphospholipid antibodies - thrombosis - obstetric complications - anticoagulation


Publication History

Article published online:
16 January 2023

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