Difference between revisions of "Antiphospholipid syndrome"

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==Introduction==
+
==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)
 
**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)
 
*APS can occur as a primary condition or in setting of underlying disease (eg SLE)
  
 
+
===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
 
*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
 
**“Second hit” stressors: smoking, prolonged immobilization, pregnancy, exogenous estrogen, malignancy, nephrotic syndrome, HTN, hyperlipidemia
  
 
+
==Clinical Features==
==Diagnosis==
 
*Presence of DVT, arterial thrombus, or pregnancy morbidity (eg fetal loss, preterm)
 
*Presence of aPL
 
 
 
 
 
==Clinical Manifestations==
 
 
*Thrombocytopenia, increased PT/INR and aPTT
 
*Thrombocytopenia, increased PT/INR and aPTT
 
*[[Microangiopathic Hemolytic Anemia (MAHA)]]
 
*[[Microangiopathic Hemolytic Anemia (MAHA)]]
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*Livedo reticularis
 
*Livedo reticularis
  
 +
==Differential Diagnosis==
  
==Complications==
+
==Diagnosis==
*Catastrophic APS: widespread thrombotic disease w/ multiorgan failure precipitated by some stress (eg infection)
+
*Presence of DVT, arterial thrombus, or pregnancy morbidity (eg fetal loss, preterm)
 +
*Presence of aPL
  
 
+
==Treatment==
==APS Treatment==
 
 
*Anticoagulation (unfractionated heparin, LMWH, or warfarin)
 
*Anticoagulation (unfractionated heparin, LMWH, or warfarin)
 
**No benefit in treatment or prophy using ASA or plavix
 
**No benefit in treatment or prophy using ASA or plavix
 
**Add hydroxychloroquine if pt has SLE
 
**Add hydroxychloroquine if pt has SLE
 
**Warfarin contraindicated in pregnancy!
 
**Warfarin contraindicated in pregnancy!
 
  
 
*IVIG, plasmapharesis, 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
 
*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
 
**If evidence of microangiopathy (thrombocytopenia, MAHA), add IVIG and plasma exchange to above regimen
  
 +
==Complications==
 +
*Catastrophic APS: widespread thrombotic disease w/ multiorgan failure precipitated by some stress (eg infection)
  
 
==See Also==
 
==See Also==

Revision as of 11:55, 18 July 2015

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)

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

Clinical Features

Differential Diagnosis

Diagnosis

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

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!
  • 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

Complications

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

See Also