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Antiphospholipid syndrome (APS)


Presentation of antiphospholipid syndrome

  • Recurrent venous or arterial thrombosis
    • Venous more common than arterial
    • Arterial most commonly cerebral
  • Recurrent foetal loss or miscarriage
  • Can occur in association with SLE or rheumatoid arthritis (secondary) or alone (primary)


Pathogenesis of antiphospholipid syndrome

  • Antibody-related (lupus anticoagulant, anti-cardioplipin, anti B2 glycoprotein)
  • Underlying defect may  involve mammalian target of rapamycin complex (mTORC)
    • See NEJM 2014; 371:369-371


Diagnostic criteria for antiphospholipid syndrome (requires at least one clinical and at least one laboratory feature)

  • One or more episodes of arterial, venous or small vessel thrombosis
  • One or more foetal loss >10/40
  • Three or more unexplained miscarriages at <10/40
  • One or more pre-term (<34/40) due to eclampsia or placental insufficiency
  • Lupus anticoagulant, anti-cardioplipin or anti B2 glycoprotein present on at least 2 occasions, 12 weeks apart


Treatment of antiphospholipid syndrome

  • Treatment dose anticoagulation post-thrombotic event (usually life-long if significant)
  • Consider low dose aspirin for prophylaxis
  • In pregnancy
    • Prophylactic LMWH if no history of thrombosis
    • Treatment dose LMWH if history of thrombosis


Complications of antiphospholipid syndrome

  • Catastrophic antiphospholipid syndrome (rare)
    • Acute thrombotic microangiopathy, multiple organ thrombosis, occasionally with skin necrosis