Antiphospholipid syndrome is a systemic autoimmune disorder.

It is usually defined
as the clinical complex of vascular occlusion and ischaemic events occurring in
patients who have circulating antiphospholipid antibodies.

Clinical presentation
Antiphospholipid syndrome is characterised by venous, arterial, or small vessel
thrombosis, potentially affecting multiple organ systems:

abdominal manifestations (e.g. renal vein thrombosis, Budd-Chiari syndrome,

mesenteric ischaemia)
cerebral manifestations (e.g. ischaemic stroke, transient ischaemic attack)
if present with livedo reticularis, is sometimes known as Sneddon syndrome
cutaneous manifestations (e.g. livedo reticularis, livedo racemosa)
thoracic manifestations
pulmonary manifestations (e.g. pulmonary embolus, diffuse alveolar haemorrhage)
cardiac manifestations (e.g. valvular thickening)
Pathology
Patients have circulating antiphospholipid antibodies cross-react with cell
membrane phospholipids. This results in a hypercoagulable state leading to vascular
thrombosis.

Aetiology
The disorder can be primary (primary antiphospholipid syndrome) or secondary (i.e.
associated with systemic lupus erythematosus).

Markers
Positive lupus anticoagulant, anticardiolipin antibodies, and anti-beta2-
glycoprotein I antibodies are seen but are not specific for antiphospholipid
syndrome if considered in isolation (see diagnostic criteria below) 6.
Paradoxically, if lupus anticoagulant is present, a patient's coagulation profile
will show an elevated aPTT suggesting a propensity to bleeding, however this is not
the case.

Diagnosis
Diagnosis is made using the revised Sapporo criteria, in which patients need to
meet at least one clinical and at least one laboratory criteria to fulfill the
diagnosis of antiphospholipid syndrome 7.

clinical criteria 7:
vascular thrombosis: =1 episode of venous, arterial, or small vessel thrombosis in
any tissue or organ
superficial thrombophlebitis does not meet this criterion
pregnancy morbidity: 3 miscarriages <10 weeks, or 1 miscarriage =10 weeks, or
premature birth before 34 weeks
laboratory criteria (detected on =2 occasions on testing at least 12 weeks apart)
7:
anticoardiolipin antibodies
anti-beta2-glycoprotein I antibodies
lupus anticoagulant
Treatment and prognosis
Asymptomatic antiphospholipid syndrome does not warrant any antiplatelet or
antithrombotic therapy 8. However, warfarin is used for acute thromboembolic events
or as secondary prevention 8. Management of organ-specific manifestations of
antiphospholipid syndrome are beyond the scope of this general article.