Antiphospholipid syndrome

Persistently elevated antiphospholipid antibodies associated with thromboses and pregnancy-related morbidity are key diagnostic criteria.

Predisposes to arterial and microvascular thrombosis, as well as venous thromboembolism, and 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 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.

Thrombosis tends to recur in the same vascular bed; that is, patients presenting with stroke have a high risk of recurrent stroke.

Antiphospholipid syndrome (APS), also known as antiphospholipid antibody syndrome, is the association of antiphospholipid antibodies (lupus anticoagulant, anticardiolipin antibody, and/or anti-beta2-glycoprotein I) with a variety of clinical features characterised by thromboses and 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 Pregnancy morbidity is defined as the loss of 3 or more embryos before the 10th week of gestation and/or 1 or more otherwise unexplained fetal deaths beyond the 10th week of gestation, and/or the premature birth of a morphologically normal neonate before the 34th week of gestation because of eclampsia, severe pre-eclampsia, or placental insufficiency. [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

Female patients with antiphospholipid antibodies and a history of pregnancy-related morbidity but no history of thrombosis are considered to have obstetric APS.

Patients who have antiphospholipid antibodies but no thrombotic or related obstetric complications (i.e., do not fit criteria for APS) are considered to have incidental antiphospholipid antibodies (aPL). Such patients are at increased risk of thrombosis, but it is not possible to identify which specific patients are at risk.