Difference between revisions of "Antiphospholipid syndrome"

 
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==Background==
 
==Background==
 
*APS definition (need 1 from each category):  
 
*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)
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**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)
 
**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)
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*APS can occur as a primary condition or in setting of underlying disease (eg [[SLE]])
*Lifelong anticoagulation with warfarin, with the following target INRs<ref>Movva S et a. Antiphospholipid Syndrome. eMedicine. Mar 24, 2015. http://emedicine.medscape.com/article/333221-treatment.</ref>
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*Lifelong anticoagulation with [[warfarin]], with the following target INRs<ref>Movva S et a. Antiphospholipid Syndrome. eMedicine. Mar 24, 2015. http://emedicine.medscape.com/article/333221-treatment.</ref>
 
**2.0-3.0 for venous
 
**2.0-3.0 for venous
 
**3.0 for arterial
 
**3.0 for arterial
 
**3.0-4.0 recurrent thrombosis
 
**3.0-4.0 recurrent thrombosis
**ASA plus warfarin for severe/refractory cases
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**[[ASA]] plus [[warfarin]] for severe/refractory cases
  
 
===Pathophysiology===
 
===Pathophysiology===
*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
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*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, HTN, hyperlipidemia
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**“Second hit” stressors: smoking, prolonged immobilization, pregnancy, exogenous estrogen, malignancy, nephrotic syndrome, hypertension, hyperlipidemia
  
 
==Clinical Features==
 
==Clinical Features==
*Thrombocytopenia, increased PT/INR and aPTT
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*[[Thrombocytopenia]], [[coagulopathy|increased PT/INR and aPTT]]
 
*[[Microangiopathic Hemolytic Anemia (MAHA)]]
 
*[[Microangiopathic Hemolytic Anemia (MAHA)]]
*DVT/PE
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*[[DVT]]/PE
 
*Fetal loss
 
*Fetal loss
*Heart valve disease
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*Heart [[valvular emergencies|valve disease]]
 
*aPL-nephropathy
 
*aPL-nephropathy
*Stroke/TIA, other neuro sx
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*[[Stroke]]/[[TIA]], other neuro symptoms
 
*Livedo reticularis
 
*Livedo reticularis
  
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{{Hemolytic anemia DDX}}
 
{{Hemolytic anemia DDX}}
  
==Diagnosis==
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==Management==
*Presence of DVT, arterial thrombus, or pregnancy morbidity (eg fetal loss, preterm)
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*[[Anticoagulation]] ([[unfractionated heparin]], [[LMWH]], or [[warfarin]])
*Presence of aPL
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**No benefit in treatment or prophylaxis using [[ASA]] or [[plavix]]
 
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**Add [[hydroxychloroquine]] if patient has SLE
==Treatment==
 
*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!
 
**Warfarin contraindicated in pregnancy!
 
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*[[IVIG]], [[plasmapheresis]], and steroids have ''not'' been proven to be of benefit in APS
*IVIG, plasmapharesis, 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==
*Acute complications, admission with hematology c/s
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*Acute complications, admission with hematology consult
  
 
==Complications==
 
==Complications==
*Catastrophic APS: widespread thrombotic disease w/ multiorgan failure precipitated by some stress (eg infection)
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*Catastrophic APS: widespread thrombotic disease with multiorgan failure precipitated by some stress (eg infection)
  
 
==See Also==
 
==See Also==
 
<references/>
 
<references/>
  
[[Category:Heme/Onc]] [[Category:Rheum]]
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[[Category:Heme/Onc]] [[Category:Rheumatology]]

Latest revision as of 23:35, 30 September 2019

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

Differential Diagnosis

Microangiopathic Hemolytic Anemia (MAHA)

Management

Catastrophic APS Treatment

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.