#132 Antiphospholipid Syndrome Flashcards

1
Q

What % of people with antiphospholipid syndrome are female?

A

70%

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2
Q

What is the role of beta2-glycoprotein I?

A

Regulatory role in coagulation, fibrinolysis, and other physiologic systems

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3
Q

What medical/obstetrical problems are associated with antiphospholipids antibodies?

A

Arterial thrombosis, venous thrombosis, autoimmune thrombocytopenia, fetal loss, preeclampsia, IUGR, placental insufficiency, preterm delivery, hemolytic anemia, amaurosis fugax, livedo reticularis, SLE, false-positive RPR

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4
Q

What antiphospholipid antibodies contribute to the diagnosis of antiphospholipid syndrome?

A
  1. Lupus anticoagulant
  2. Anticardiolipin
  3. anti-beta2-glycoprotein I
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5
Q

Which antibody associated with antiphospholipid syndrome is most specific?

A

Lupus anticoagulant. But it is less sensitive than the other two.

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6
Q

Do you need all three antibodies to be diagnosed with antiphospholipid syndrome?

A

No.

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7
Q

What are laboratory criteria for diagnosis of antiphospholipid syndrome?

A
  1. Lupus anticoagulant present in plasma on two or more occasions at least 12 weeks apart (when no on anticoagulants)
  2. Anticardiolipin antibody IgG and/or IgM >99%tile on two or more occasions at least 12wks apart
  3. Anti-beta2-glycoprotein I IgG and/or IgM >99%tile on two or more occasions at least 12wks apart
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8
Q

How do you screen for lupus anticoagulant?

A

Combination of sensitive clotting assays, such as lupus anticoagulant-sensitive activated PTT and dilute Russell’s viper venom time

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9
Q

Which isotypes of anticardiolipin antibodies matter?

A

IgG and IgM. Clinical relevance of IgA is uncertain

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10
Q

What test is used to measure anticardiolipin antibodies?

A

Enzyme-linked immunosorbent assay

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11
Q

What test is used to measure anti-beta2-glycoprotein I?

A

Enzyme-linked immunosorbent assay

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12
Q

What is considered a positive anti-beta2-glycoprotein antibody test?

A

> 99th percentile

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13
Q

What percentage of patients with venous thrombosis will test positive for lupus anticoagulant antibodies?

A

2%

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14
Q

What is the risk of thrombosis during pregnancy or puerperium in women with antiphospholipid syndrome?

A

5-12%

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15
Q

What is the most common consequence of an arterial occlusion?

A

Stroke

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16
Q

What percentage of individuals <50yo (otherwise healthy) with stroke have antiphospholipid antibodies?

A

4-6%

17
Q

What percentage of individuals with antiphospholipid syndome will have autoimmune thrombocytopenia?

A

40-50%

18
Q

Is thombocytopenia from antiphospholipid syndrome vs immune thrombocytopenic purpura treated differently?

A

No, same therapy. steroids. immune globulin.

19
Q

What is the definition of the fetal period?

A

Greater than 10wks

20
Q

What % of pregnancy losses occur during the fetal period (>10wk) in women with antiphospholipid antibodies vs general population?

A

50% vs 10%

21
Q

What % of women with antiphospholipid antibodies had at least one fetal death compared to those without antibodies?

A

84% vs 24%

22
Q

What percentage of women with recurrent pregnancy loss test positive for antiphospholipid antibodies?

A

5-20%. Not known if these cases would meet current international criteria for APLS

23
Q

What percentage of women with preeclampsia test positive for antiphospholipid antibodies?

A

11-17%

24
Q

What percentage of pregnancies in women with APLS are IUGR?

A

15-30%

25
Q

What are clinical criteria for diagnosis of APLS?

A
  1. vascular thrombosis: one or more clinical episodes of arterial, venous, or small vessel thrombosis or
  2. Pregnancy morbidity:
    • one or more unexplained deaths of normal fetus at or beyond 10wks, or
    • one or more premature births of normal neonate before 34wks d/t SPEC, eclampsia, placental insufficiency, or
    • three or more unexplained consecutive spontaneous pregnancy losses before 10th week
26
Q

How should a women with APLS w/ hx thrombosis be managed during pregnancy?

A

Prophylactic anticoagulation with heparin throughout pregnancy and 6wks postpartum. Many people add ASA, but benefit is unknown

27
Q

How should you treat women with APLS without hx of thrombosis?

A

Clinical surveillance or prophylactic heparin antepartum in addition to 6wk postpartum

28
Q

How should women with APLS and recurrent pregnancy loss be managed?

A

Prophylactic heparin and low-dose ASA. May reduce pregnancy loss by 50%

29
Q

Is antepartum surveillance (fetal testing) recommended for patients with APLS?

A

Many experts recommend serial ultrasound assessment and antepartum testing in 3rd trimester. Data insufficient to support or refute specific practice.

30
Q

Is APLS a contraindication to estrogen-containing contraceptives?

A

Yes.