PB#132: Antiphospholipid Syndrome Flashcards

(48 cards)

1
Q

APL antibody that is primarily clinically relevant

A

B2-glycoprotein I

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2
Q

Characterization of B2-glycoprotein I

A

Ubiquitous, multifunctional plasma protein w/ affinity for negatively charged phospholipids

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3
Q

Role of B2-glycoprotein I (3)

A

Regulatory role in coagulation, fibrinolysis, and other physiologic systems

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4
Q

Adverse outcomes associated w/ APL antibodies (7)

A

Arterial/Venous thrombosis, autoimmune thrombocytopenia, fetal loss, pre-E, FGR, placental insufficiency, PTD

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5
Q

Three APL antibodies that contribute to APLS

A

Lupus anticoagulant, anticardiolipin antibody, anti-B2-glycoprotein I

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6
Q

Testing for which APL antibody is the most specific, but less sensitive, compared to the other two

A

Lupus anticoagulant

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7
Q

How is APLS diagnosed based on lab testing?

A

Two positive APL antibody test results >12 weeks apart

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8
Q

Interpretation of lupus anticoagulant testing; necessity of testing prior to anticoagulant tx

A

Present/Absent; ideally performed before anticoagulant tx

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9
Q

Interpretation of anticardiolipin antibody testing

A

IgG and/or IgM present in medium-high titer (>40 GPL or MPL, or >99th %ile)

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10
Q

Interpretation of anti-B2-glycoprotein I testing

A

IgG and/or IgM present in titer >99th %ile for normal population

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11
Q

Is lupus anticoagulant only present in pts w/ SLE?

A

No, it is present in many pts w/o SLE

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12
Q

Is lupus anticoagulant associated w/ anticoagulation?

A

No, it is associated w/ thrombosis

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13
Q

What testing is usually used for initial lab screening of lupus anticoagulant?

A

Combo of sensitive clotting assays, ie lupus anticoagulant-sensitive aPTT and dilute Russell’s viper venom time

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14
Q

How do lupus anticoagulants function in clotting assays?

A

They paradoxically block phospholipid-dependent clotting assays by interfering w/ assembly of prothrombin complex

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15
Q

How does presence of lupus anticoagulant affect clotting time in assay testing?

A

Clotting time remains prolonged despite addition of normal plasma

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16
Q

What does second confirmatory testing for lupus anticoagulant consist of?

A

Addition/Removal of phospholipid from assay

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17
Q

Can lupus anticoagulant be quantified?

A

No

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18
Q

Most common lab method of detecting anticardiolipin antibodies; which anticardiolipin antibodies should be measured?

A

ELISA; IgG and IgM

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19
Q

Units for measuring anticardiolipin IgG and IgM antibodies

A

GPL and MPL

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20
Q

Most common lab method of detecting anti-B2-glycoprotein I antibodies; which anti-B2-glycoprotein I antibodies should be measured?

A

ELISA; IgG and IgM

21
Q

Units for measuring anti-B2-glycoprotein I IgG and IgM antibodies

22
Q

Most common serious complications associated w/ APLS

A

Venous/Arterial thrombosis

23
Q

Percentage of thrombotic events that are venous in APLS pts

24
Q

Percentage of all pts w/ venous thrombosis who will test positive for lupus anticoagulant antibodies

25
Most frequent site of venous thrombosis
Lower extremity
26
Percentage of asymptomatic nonpregnant adults incidentally found to have APL antibodies that will eventually develop thromboses
<1% each year
27
Percentage of thrombotic events in pts w/ APLS that occur during pregnancy/PP period
Up to 25%
28
Percent risk of thrombosis during pregnancy/PP period in pts w/ APLS
5-12%
29
Possible atypical sites of arterial thromboses in pts w/ APLS (4)
Retinal, subclavian, digital, brachial arteries
30
Most common consequence of arterial occlusion; most frequently involved vessel in these situations
Stroke; MCA
31
Percentage of o/w healthy pts w/ stroke who are <50 y/o in whom APL antibodies are present
4-6%
32
Percentage of pts w/ APLS who have autoimmune thrombocytopenia
40-50%
33
Miscellaneous conditions associated w/ APL antibodies (9)
TIAs, amaurosis fugax, coronary occlusions, autoimmune hemolytic anemia, livedo reticularis, cutaneous ulcers, chorea gravidarum, multi-infarct dementia, transverse myelitis
34
Type of early pregnancy loss associated w/ APLS; type of early pregnancy loss NOT associated w/ APLS
Fetal loss (>10wga), recurrent embryonic/fetal loss; sporadic embryonic loss
35
Percentage of pts w/ RPL who have APL antibodies
5-20%
36
Percentage of pts w/ pre-E who will test positive for APL antibodies
11-17%
37
Percentages of pregnancies c/b FGR in pts w/ APLS
15-30%
38
Thromboembolic indications for APL antibody testing (3)
Prior unexplained arterial/venous thromboembolism, new arterial/venous thromboembolism during pregnancy, hx of VTE w/o previous testing
39
OB indications for APL antibody testing (3)
Hx of 1+ fetal loss (at >10wga) of morphologically normal fetus, hx of 1+ premature births of morphologically normal neonate at <34wga 2/2 eclampsia or severe pre-E (or features c/w placental insufficiency), and/or 3+ unexplained consecutive spontaneous pregnancy losses (<10wga) w/ maternal anatomic/hormonal abnormalities and parental chromosomal causes excluded
40
Lab testing for APLS (3)
Lupus anticoagulant, anticardiolipin antibodies (IgG and IgM), anti-B2-glycoprotein I antibodies (IgG and IgM)
41
What should be done if an initial positive APL test returns?
Confirm after 12 weeks, and persistence of positive results on repeat testing is confirmatory
42
Meds to start pts on w/ APLS and hx of thrombotic event (2)
Ppx heparin, LDA
43
Duration of ppx heparin in pregnant pts w/ APLS and hx of thrombotic event
Throughout pregnancy and through 6 weeks PP
44
Benefit of ppx heparin and LDA in pts w/ APLS and RPL
50% reduction in pregnancy loss
45
OB surveillance in pts w/ APLS
Serial growth US in 3rd tri
46
Percentage of pts w/ APLS who develop thromboses during 3-10 years of f/u (even w/o hx of thrombosis); percentage of pts w/ APLS who develop SLE
50%; 10%
47
Longterm management specialists for pts w/ APLS (3)
IM, heme, rheum
48
Contraception considerations for pts w/ APLS
COCs are contraindicated, but progestin-only forms of contraception are appropriate