APLS - Noon conference february 7 2019

1. Noon Conference
Abdullah Arjomand, R2
02/07/2019

2. © 2016 Virginia Mason Medical Center 2
Antiphospholipid antibody
syndrome(APS)
• Autoimmune disease characterized by
venous or arterial thrombosis and/or
pregnancy morbidity
• Can be primary or secondary
• SLE
• Medications
• Caused by antibodies against
phospholipid-binding proteins
• Also known as antiphospholipid syndrome

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Epidemiology
• Male: female ratio 1:3.5 in primary APS
• 1:7 in SLE-associated APS
• Retrospective analysis of patients w/o
known autoimmune diseases studied
antiphospholipid antibodies in various
diseases*
• 9% in patients with pregnancy losses
• 14% in patients with stroke
• 11% in patients with MI
• 10% with DVT
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*Andreoli L. et al. Estimated frequency of antiphospholipid antibodies in patients with pregnancy morbidity, stroke, myocardial
infarction, and deep vein thrombosis: a critical review of the literature, Arthritis care res (2013 Nov)

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Diagnosis
• Must meet one clinical and one lab criteria
• Clinical criteria:
• Vascular thrombosis
• Can be arterial or venous
• Pregnancy morbidity (any of the following)
• Unexplained death of a morphologically
normal fetus at gestational age >10
weeks
• One or more premature births (<34
weeks) due to eclampsia or severe
preeclampsia
• Three or more consecutive spontaneous
abortions <10 weeks gestation
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Diagnosis
• Lab criteria (testing for antiphsopholipid
antibodies [APA]):
• Anticardiolipin antibody
• Anti beta-2 glycoprotein
• Lupus anticoagulant
• Repeat confirmatory testing should be
performed 12 weeks after initial positive
test
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Catastrophic APS
Defined as APS leading to multiorgan
failure. Must meet all four criteria:
• Involvement of three or more
organs, systems, and/or tissues
• Development of manifestations
within 1 week of each other
• Confirmation of small vessel
occlusion
• Lab confirmation of APA
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Treatment
• Warfarin (INR 2-3) for secondary
ppx of thrombosis
• Aspirin + unfractionated heparin for
prevention of pregnancy morbidity
• Catastrophic APS treatment
• IVIg
• Steroids
• Heparin gtt
• Plasmapharesis
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Illness Scripts
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Antiphospholipid syndrome Factor V Leiden Mutation
Pathophysiology
Antibodies to phospholipid-binding proteins
cause endothelial activation
Mutant factor V leads to decreased protein C
activity, increased thrombin generation
Epidemiology
Female predominance
APA+ in 10% of DVT
Relative risk of VTE is 3-8 in heterozygote,
80 in homozygote
5% of Caucasians are heterozygotes
20% of patients with VTE are heterozygotes
Etiology
Can be primary or secondary to SLE,
medications, infection, cancer
Autosomal dominant, with more higher VTE
risk in homozygotes
Clinical
presentation
Arterial/venous thrombi
Pregnancy morbidity
Catastrophic APS
Increased VTE risk, especially during
pregnancy
Diagnostics APA testing Genetic testing
Therapeutics
Warfarin for secondary thrombosis ppx
Unfractionated heparin for decreased
pregnancy morbidity
Treatment of VTE as per usual guidelines
Avoid OCP in homozygous women

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Multiple choice questions
A 30F G4P0 is seen for consultation after
four spontaneous abortions. She would like
to carry a pregnancy to term. Workup
reveals high titers of anticardiolipin
antibodies. Which of the following would be
not be appropriate management?
A. Review medication list
B. Warfarin
C. Repeat serological testing after 12 weeks
D. All of the above are appropriate
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10. © 2016 Virginia Mason Medical Center
Multiple choice questions
A 30F G4P0 is seen for consultation after four spontaneous
abortions. She would like to carry a pregnancy to term.
Workup reveals high titers of anticardiolipin antibodies. Which
of the following would be not be appropriate management?
A. Review medication list
B. Warfarin (Unfractionated heparin should
be used. Warfarin contraindicated in
pregnancy, also primary thrombosis ppx
not indicated in APS)
C. Repeat serological testing after 12 weeks
D. All of the above are appropriate
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Multiple choice questions
In which of the following patients
would APS testing not be
recommended?
A. 27F G4P0 with recurrent fetal losses
B. 34F with VTE during pregnancy
C. 48M with ischemic stroke
D.75F with unprovoked VTE
E. Testing recommended for all of the
above
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12. © 2016 Virginia Mason Medical Center
Multiple choice questions
In which of the following patients
would APS testing not be
recommended?
A. 27F G4P0 with recurrent fetal losses
B. 34F with VTE during pregnancy
C. 48M with ischemic stroke
D.75F with unprovoked VTE (arguable)
E. Testing recommended for all of the
above
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13. © 2016 Virginia Mason Medical Center
Multiple choice questions
• British committee for standards of
hemaetology recommends APS
testing for ischemic stroke <50 y/o,
all unprovoked VTE
• Complicated, because
recommendation is to test 7 days
after d/c anticoagulation
• American College of Obstetricians
and Gynecologists recommends
testing in patients with VTE during
pregnancy 13

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