Antiphospholipid syndrome: Difference between revisions

 
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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]])
*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
**3.0 for arterial
**3.0-4.0 recurrent thrombosis
**[[ASA]] plus [[warfarin]] for severe/refractory cases


*APS can occur as a primary condition or in setting of underlying disease (eg SLE)
===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==
==Pathophysiology==
*[[Thrombocytopenia]], [[coagulopathy|increased PT/INR and aPTT]]
*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
 
 
==Diagnosis==
*Presence of DVT, arterial thrombus, or pregnancy morbidity (eg fetal loss, preterm)
*Presence of aPL
 
 
==Clinical Manifestations==
*Thrombocytopenia, increased PT/INR and aPTT
*[[Microangiopathic Hemolytic Anemia (MAHA)]]
*[[Microangiopathic Hemolytic Anemia (MAHA)]]
*DVT/PE
*[[DVT]]/PE
*Fetal loss
*Fetal loss
*Heart valve disease
*Heart [[valvular emergencies|valve disease]]
*aPL-nephropathy
*aPL-nephropathy
*Stroke/TIA, other neuro sx
*[[Stroke]]/[[TIA]], other neuro symptoms
*Livedo reticularis
*Livedo reticularis


==Differential Diagnosis==
{{Hemolytic anemia DDX}}


==Complications==
==Management==
*Catastrophic APS: widespread thrombotic disease w/ multiorgan failure precipitated by some stress (eg infection)
*[[Anticoagulation]] ([[unfractionated heparin]], [[LMWH]], or [[warfarin]])
 
**No benefit in treatment or prophylaxis using [[ASA]] or [[plavix]]
 
**Add [[hydroxychloroquine]] if patient has SLE
==APS 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!
**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


*IVIG, plasmapharesis, and steroids have not been proven to be of benefit in APS
==Disposition==
 
*Acute complications, admission with hematology consult
 
==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 with multiorgan failure precipitated by some stress (eg infection)


==See Also==
==See Also==
*[[DIC]], [[TTP]], [[HUS]], [[Microangiopathic Hemolytic Anemia (MAHA)]]
<references/>
*[[HELLP]], [[Paroxysmal Nocturnal Hemoglobinuria (PNH)]], [[HIT]]


[[Category:Heme/Onc]] [[Category:Rheum]]
[[Category:Heme/Onc]] [[Category:Rheumatology]]

Latest revision as of 01:18, 13 February 2020

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.