Antiphospholipid syndrome


  • APS definition (need 1 from each category):
    • Presence of at least 1 of the following: DVT, arterial thrombosis, or pregnancy morbidity (eg fetal loss, preterm)
    • Presence of at least 1 of the following antiphospholipid antibodies (aPL): lupus anticoagulant (LA), anticardiolipin (aCL), β2-glycoprotein-1 (β2-GP-1)
  • APS can occur as a primary condition or in setting of underlying disease (eg SLE)


  • Currently accepted theory: Susceptible pts (eg SLE) develop aPL after infection. After development of aPL, “second hit” stress required to develop full-blown APS. aPL affects coagulation by interacting with protein C, annexin V, platelets, proteases, tissue factor, and impairing finbrinolysis
    • “Second hit” stressors: smoking, prolonged immobilization, pregnancy, exogenous estrogen, malignancy, nephrotic syndrome, HTN, hyperlipidemia

Clinical Features

Differential Diagnosis


  • Presence of DVT, arterial thrombus, or pregnancy morbidity (eg fetal loss, preterm)
  • Presence of aPL


  • Anticoagulation (unfractionated heparin, LMWH, or warfarin)
    • No benefit in treatment or prophy using ASA or plavix
    • Add hydroxychloroquine if pt has SLE
    • Warfarin contraindicated in pregnancy!
  • IVIG, plasmapharesis, and steroids have not been proven to be of benefit in APS

Catastrophic APS Treatment

  • Treat stress that preceipitated catastrophic APS (eg infection), anticoagulation, high dose steroids
    • If evidence of microangiopathy (thrombocytopenia, MAHA), add IVIG and plasma exchange to above regimen


  • Catastrophic APS: widespread thrombotic disease w/ multiorgan failure precipitated by some stress (eg infection)

See Also