Difference between revisions of "Antiphospholipid syndrome"

(Text replacement - "prophy " to "prophylaxis ")
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==Management==
 
==Management==
*Anticoagulation (unfractionated heparin, LMWH, or warfarin)
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*Anticoagulation ([[unfractionated heparin]], [[LMWH]], or [[warfarin]])
**No benefit in treatment or prophylaxis using ASA or plavix
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**No benefit in treatment or prophylaxis using [[ASA]] or [[plavix]]
**Add hydroxychloroquine if patient has SLE
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**Add [[hydroxychloroquine]] if patient has SLE
 
**Warfarin contraindicated in pregnancy!
 
**Warfarin contraindicated in pregnancy!
  
*IVIG, plasmapharesis, and steroids have not been proven to be of benefit in APS
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*[[IVIG]], [[plasmapheresis]], and steroids have not been proven to be of benefit in APS
  
 
===Catastrophic APS Treatment===
 
===Catastrophic APS Treatment===
*Treat stress that preceipitated catastrophic APS (eg infection), anticoagulation, high dose steroids
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*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
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**If evidence of microangiopathy ([[thrombocytopenia]], [[MAHA]]), add [[IVIG]] and [[plasma exchange]] to above regimen
  
 
==Disposition==
 
==Disposition==

Revision as of 23:51, 13 November 2016

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 finbrinolysis
    • “Second hit” stressors: smoking, prolonged immobilization, pregnancy, exogenous estrogen, malignancy, nephrotic syndrome, hypertension, hyperlipidemia

Clinical Features

Differential Diagnosis

Microangiopathic Hemolytic Anemia (MAHA)

Evaluation

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

Management

Catastrophic APS Treatment

  • Treat stress that precipitated catastrophic APS (eg infection), anticoagulation, high dose steroids

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.