Antiphospholipid syndrome: Difference between revisions
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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) | **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]]) | ||
*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> | *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 | **[[ASA]] plus [[warfarin]] for severe/refractory cases | ||
===Pathophysiology=== | ===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 | *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 | **“Second hit” stressors: smoking, prolonged immobilization, pregnancy, exogenous estrogen, malignancy, nephrotic syndrome, hypertension, hyperlipidemia | ||
==Clinical Features== | ==Clinical Features== | ||
*Thrombocytopenia, increased PT/INR and aPTT | *[[Thrombocytopenia]], [[coagulopathy|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 symptoms | *[[Stroke]]/[[TIA]], other neuro symptoms | ||
*Livedo reticularis | *Livedo reticularis | ||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
{{Hemolytic anemia DDX}} | {{Hemolytic anemia DDX}} | ||
==Management== | ==Management== | ||
*Anticoagulation ([[unfractionated heparin]], [[LMWH]], or [[warfarin]]) | *[[Anticoagulation]] ([[unfractionated heparin]], [[LMWH]], or [[warfarin]]) | ||
**No benefit in treatment or prophylaxis using [[ASA]] or [[plavix]] | **No benefit in treatment or prophylaxis using [[ASA]] or [[plavix]] | ||
**Add [[hydroxychloroquine]] if patient has SLE | **Add [[hydroxychloroquine]] if patient has SLE | ||
**Warfarin contraindicated in pregnancy! | **Warfarin contraindicated in pregnancy! | ||
*[[IVIG]], [[plasmapheresis]], and steroids have ''not'' been proven to be of benefit in APS | |||
*[[IVIG]], [[plasmapheresis]], and steroids have not been proven to be of benefit in APS | |||
===Catastrophic APS Treatment=== | ===Catastrophic APS Treatment=== | ||
*Treat stress that precipitated catastrophic APS (eg infection), anticoagulation, high dose steroids | *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 | **If evidence of microangiopathy ([[thrombocytopenia]], [[MAHA]]), add [[IVIG]] and [[plasma exchange]] to above regimen | ||
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]
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
- Thrombocytopenia, increased PT/INR and aPTT
- Microangiopathic Hemolytic Anemia (MAHA)
- DVT/PE
- Fetal loss
- Heart valve disease
- aPL-nephropathy
- Stroke/TIA, other neuro symptoms
- Livedo reticularis
Differential Diagnosis
Microangiopathic Hemolytic Anemia (MAHA)
- Disseminated Intravascular Coagulation (DIC)
- Thrombotic Thrombocytopenic Purpura (TTP)
- Hemolytic Uremic Syndrome (HUS)
- HELLP syndrome
- Heparin-Induced Thrombocytopenia (HIT)
- Hereditary spherocytosis
- Paroxysmal nocturnal hemoglobinuria (PNH)
- Malignant hypertension
- Scleroderma
- Antiphospholipid Syndrome (APS)
- Other medical causes: malignancy, renal allograft rejection, vasculitides like polyarteritis nodosa and Wegener's granulomatosis
- Drugs: chemotherapy; Clopidogrel (Plavix) associated with TTP
- Nonvascular causes: prosthetic valve (more common with mechanical, more common at aortic valve), LVAD, TIPS, coil embolization, patched AV shunt, AVM
Management
- Anticoagulation (unfractionated heparin, LMWH, or warfarin)
- No benefit in treatment or prophylaxis using ASA or plavix
- Add hydroxychloroquine if patient has SLE
- Warfarin contraindicated in pregnancy!
- 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
Disposition
- Acute complications, admission with hematology consult
Complications
- Catastrophic APS: widespread thrombotic disease with multiorgan failure precipitated by some stress (eg infection)
See Also
- ↑ Movva S et a. Antiphospholipid Syndrome. eMedicine. Mar 24, 2015. http://emedicine.medscape.com/article/333221-treatment.