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Antiphospholipid Syndrome

This document summarizes antiphospholipid syndrome (APS), an autoimmune disorder characterized by circulating antiphospholipid antibodies that can cause arterial or venous thrombosis, autoimmune thrombocytopenia, and fetal loss. APS is diagnosed based on clinical criteria including vascular thrombosis or pregnancy morbidity and the presence of antiphospholipid antibodies like lupus anticoagulant, anticardiolipin, and anti-beta2 glycoprotein 1 antibodies. Complications of APS include thrombosis, thrombocytopenia, preeclampsia, fetal growth restriction, and recurrent pregnancy loss. Treatment during pregnancy involves anticoagulation and low dose aspirin to reduce risks.

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59 views18 pages

Antiphospholipid Syndrome

This document summarizes antiphospholipid syndrome (APS), an autoimmune disorder characterized by circulating antiphospholipid antibodies that can cause arterial or venous thrombosis, autoimmune thrombocytopenia, and fetal loss. APS is diagnosed based on clinical criteria including vascular thrombosis or pregnancy morbidity and the presence of antiphospholipid antibodies like lupus anticoagulant, anticardiolipin, and anti-beta2 glycoprotein 1 antibodies. Complications of APS include thrombosis, thrombocytopenia, preeclampsia, fetal growth restriction, and recurrent pregnancy loss. Treatment during pregnancy involves anticoagulation and low dose aspirin to reduce risks.

Uploaded by

gloriashirima8
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
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Download as PPTX, PDF, TXT or read online on Scribd
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Antiphospholipid Syndrome

Lauren Farmer, MD
Duke University
Autoimmune disorder
Circulating antiphospholipid antibodies

Clinical and lab diagnosis


Phospholipids
Complications
Arterial and venous thrombosis

Autoimmune thrombocytopenia

Fetal loss

Severe obstetric complications


Beta2- Glycoprotein 1 - most clinically relevant

1) Lupus anticoagulant
2) Anticardiolipin
3) Beta2- Glycoprotein 1
Diagnosis (need ⅔ positive both >12 weeks apart)
Lupus anticoagulant present in plasma two or more occasions at least 12
weeks apart (present or absent)

- Before treatment with anticoagulants

OR anticardiolipin antibody IgG and/or IgM in medium or high titer (> 40GPL
or MPL) or > 99% on two or more occasions at least 12 weeks apart

OR Anti-B2 glycoprotein I IgG or IgM ‘’ at least 12 weeks apart


Antiphospholipid antibodies
1. Present in many Systemic Lupus Erythematosus (SLE) patients
2. Assessed indirectly via a series of tests
3. Reported as “present” or “absent”
4. Most associated with clotting than the others
5. Can develop sponaneously due to medications, inefections, autoimmune
disease, SLE, EBV, syphillis, Hep C = “acquired” lupus anticoagulant”
6. 2 to 4% of general population, 15 to 34% of SLE
Clotting Cascade
Virchows Triad
Anticardiolipin Antibodies
Uncertain clinical significance

Positive if > 40 GPL or 40 MPL (> 99%)


Beta2- Glycoprotein 1 - most clinically relevant
Positive result is > 99%

Most clinically relevant


Venous Thromboembolism
Most adverse events are VTE

2% of all patients with VTE will test positive for lupus anticoagulant antibodies

If abnormal location for VTE, raises suspicion for APS

Thrombosis recurrence rate of 25% per year in untreated patients with APS

Significant increased risk in pregnancy and postpartum period (25% of all


events)
Arterial Thrombosis
Often atypical sites - retinal , subclavian, digital, brachial

Stroke, mostly MCA

Antiphospholipid ab’s in 4-6% of otherwise healthy people with stroke


Autoimmune thrombocytopenia
40-50% of individuals with APS

Hard to distinguish from Idiopathic Thrombocytopenic Purpura (ITP)

Treatment is the same


Obstetric Complications
Recurrent pregnancy loss

- Large portion greater than 10 weeks but can be throughout


- Test for 2+ losses

Preeclampsia

- 11-17% of women with preeclampsia willt est positive


- Strongest association with severe preterm preeclampsia (<34 weeks)

Intrauterine growth restriction (IUGR)

- 15-30% of APS have IUGR


Who should be tested ?
One or more episodes of arterial or venous thrombosis

Pregnancy morbidity

Unexplained fetal death beyond 10th week gestation

One or more premture births < 34 weeks because of eclampsia or severe


pereclampsia or evidence of placental insufficiency

Three or more unexplained consecutive spontaneous losses before 10th


week with maternal anatomic or hormonal abnormalities and paternal and maternal
chromosomal causes excluded
Management during pregnancy
?Hx VTE

- Prophylactic anticoagulation with heparin through pregnancy and 6 weeks


postpartum
- Low dose aspirin

?No hx VTE

- Consider anticoagulation

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