Antiphospholipid syndrome: Difference between revisions
m (Rossdonaldson1 moved page Antiphospholipid Syndrome (APS) to Antiphospholipid syndrome) |
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== | ==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== | |||
==Clinical | |||
*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== | |||
== | ==Diagnosis== | ||
* | *Presence of DVT, arterial thrombus, or pregnancy morbidity (eg fetal loss, preterm) | ||
*Presence of aPL | |||
==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
- Thrombocytopenia, increased PT/INR and aPTT
- Microangiopathic Hemolytic Anemia (MAHA)
- DVT/PE
- Fetal loss
- Heart valve disease
- aPL-nephropathy
- Stroke/TIA, other neuro sx
- Livedo reticularis
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)