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Antiphospholipids syndrome

Antiphospholipid syndrome (APS) is described as a common risk factor for recurrent thromboembolic events and/or pregnancy complications resulting from circulating antiphospholipid antibodies (aPLA)(1). It is now widely accepted that the plasma phospholipid binding protein β-2-Glycoprotein 1 (β2GP1) is the main antigenic target for aPLA (2). β2GP1 is a protein of 43 kDa composed of 5 short consensus repeat domains called “sushi” domains. An overactive immune system produces anti-β2GP1 that is associated with recurrent clotting events (thrombosis) including premature stroke, myocardial infarction, repeated miscarriages, phlebitis, venous thrombosis and pulmonary thromboembolism.

Although young and middle-aged adults, between the ages of 15 and 50 represent 85 % of APS patients, people from all age groups can also be affected. While largely under-diagnosed, it is estimated that 1 in 100 people have the disorder. Early diagnosis of APS is therefore crucial for proper and efficient care (4).

Currently no specific treatments exist for APS, though management by anticoagulation therapies  is aimed at preventing thrombosis.

References:
1. Giannakopoulos B, Krilis SA.. The pathogenesis of the antiphospholipid syndrome. N Engl J Med. 2013;368(11):1033-1044.
2. Miyakis S, Lockshin MD, Atsumi T, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost. 2006;4(2):295-306.
3. Agar C, van Os GM, Morgelin M, et al. Beta2-glycoprotein I can exist in 2 conformations: implications for our understanding of the antiphospholipid syndrome. Blood. 2010;116(8):1336-1343.

4. http://www.aps-support.org.uk