Antiphospholipid syndrome

Antiphospholipid syndrome (APS) is the association of persistently elevated antiphospholipid antibodies with a variety of clinical features characterised by thromboses and pregnancy-related morbidity.

Venous, arterial and microvascular thrombosis can affect any vessel in the body.

Main treatment goals are management of acute thrombosis and prevention of thrombosis recurrence and pregnancy morbidity.

Consideration should also be given to the presence of co-existent autoimmune disease in patients with antiphospholipid syndrome.

Catastrophic disease, which presents as multiple thrombotic events within a short period, often as multiorgan failure, occurs in <1% of patients.

High index of suspicion for antiphospholipid syndrome in any young patient (<50 years) presenting with arterial thrombosis in any vessel.

In antiphospholipid syndrome, thrombosis tends to recur in the same vascular bed, e.g., patients presenting with stroke have a high risk of recurrent stroke.

APS, also known as antiphospholipid antibody syndrome, is a clinical autoimmune syndrome characterised by persistent laboratory evidence of antiphospholipid antibodies (lupus anticoagulant, anticardiolipin antibody, and/or anti-beta2-glycoprotein I) in association with a variety of clinical features, in particular venous or arterial thrombosis and/or pregnancy-related morbidity.[1]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 Feb;4(2):295-306. https://onlinelibrary.wiley.com/doi/full/10.1111/j.1538-7836.2006.01753.x http://www.ncbi.nlm.nih.gov/pubmed/16420554?tool=bestpractice.com [2]Barbhaiya M, Zuily S, Naden R, et al. 2023 ACR/EULAR antiphospholipid syndrome classification criteria. Ann Rheum Dis. 2023 Oct;82(10):1258-70. https://ard.bmj.com/content/82/10/1258.long http://www.ncbi.nlm.nih.gov/pubmed/37640450?tool=bestpractice.com ​​​

APS can be further characterised by the type of clinical manifestation present (thrombotic or obstetric), but some individuals can present with both. Thrombotic APS is an antibody-mediated hypercoagulable state characterised by venous, arterial and/or microvascular thrombosis. Women with antiphospholipid antibodies and a history of pregnancy-related morbidity, but no history of thrombosis, are considered to have obstetric APS.

Catastrophic disease is rare but presents as multiple thrombotic events within a short period, often as multi-organ failure.