Antiphospholipid syndrome

Background

  • 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)
  • Lifelong anticoagulation with warfarin, with the following target INRs[1]
    • 2.0-3.0 for venous
    • 3.0 for arterial
    • 3.0-4.0 recurrent thrombosis
    • ASA plus warfarin for severe/refractory cases

Pathophysiology

  • Currently accepted theory: Susceptible patients (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 fibrinolysis
    • “Second hit” stressors: smoking, prolonged immobilization, pregnancy, exogenous estrogen, malignancy, nephrotic syndrome, hypertension, hyperlipidemia

Clinical Features

  • Thrombocytopenia, increased PT/INR and aPTT
  • Microangiopathic Hemolytic Anemia (MAHA)
  • DVT/PE
  • Fetal loss
  • Heart valve disease
  • aPL-nephropathy
  • Stroke/TIA, other neuro symptoms
  • Livedo reticularis

Differential Diagnosis

Microangiopathic Hemolytic Anemia (MAHA)

Management

  • Anticoagulation (unfractionated heparin, LMWH, or warfarin)
    • No benefit in treatment or prophylaxis using ASA or plavix
    • Add hydroxychloroquine if patient has SLE
    • Warfarin contraindicated in pregnancy!
    • DOACs (such as rivaroxaban, apixaban, and dabigatran) have not been shown to be effective in APS
  • IVIG, plasmapheresis, and steroids have not been proven to be of benefit in APS

Catastrophic APS Treatment

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

Disposition

  • Acute complications, admission with hematology consult

Complications

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

See Also

  1. Movva S et a. Antiphospholipid Syndrome. eMedicine. Mar 24, 2015. http://emedicine.medscape.com/article/333221-treatment.
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