Antiphospholipid syndrome

Question 1

What are the main 2 characteristics of APS

What autoantibodies are found in APS

Answer 1

Thrombosis and pregnancy morbidity

Anticardiolipin AB
Lupus anticoagulant
AntiB2 glycoprotein 1 AB

Question 2

What is the diagnostic criteria for APS

-3 points

Answer 2

1+ arterial/venous/small vessel thrombosis

10wk+ => 1+ fetal death
<34wk => 1+ premature birth
<10wks => 3+ spontaneous abortions

aCL, b2GP1, LA +ve at least 2x 12wk+ apart

Question 3

What are the risk factors with APS and thrombosis

Answer 3

surgery/immobilisation/long distance travel

CV risk factors

• HTN
• hyperlipidemia
• obesity/smoking

Pregancy/estrogens/puerperium

COX2 inhibitors => increased thromboxane, platelet aggregation

Question 4

What are the symptoms and signs of cerebral APS

-what is the relationship between APS, cerebral APS, cerebral lupus and SLE

Answer 4

Headache
Amaurosis fugax (painless loss of vision for minutes

Ischemic stroke/TIAs/cerebral venous thrombosis
Acute ischemic encephalopathy

Seizures/chorea

Transverse myelitis (inflammation across the spinal cord)

APS => cerebral APS <=> cerebral lupus
SLE => cerebral lupus <=> cerebral APS
APS does not lead to SLE

Question 5

What are the symptoms and signs of cerebral lupus

-how would you manage them

Answer 5

Focal disease => strokes, TIA => anticoagulation

Diffuse disease => organic brain syndrome => immunosuppresion

Question 6

Describe the pathophysiology behind the pregnancy complications that can arise

Answer 6

If unsuccessful
-uteroplacental insufficiency, IUGR => stillbirth, fetal death

If successful
-preeclampsia, abruption, premature delivery, maternal thrombosis

Question 7

How would you manage pregnancy in APS

• no thrombosis, pregnancy loss
• past maternal thrombosis
• repeated embryonic loss <10wks
• late fetal loss/IUGR/severe PET
• postpartum

Answer 7

no thrombosis, pregnancy loss => aspirin, close monitoring

past maternal thrombosis, repeated embryonic loss/fetal death/IUGR/PET => heparin + aspirin

postpartum => 6wks heparin

Question 8

How would you manage a patient with

• venous thrombosis
• arterial thrombosis

Answer 8

Long term treatment even if APS ABs become negative

Venous (PE/DVT) => target INR 2-3
Arterial (Stroke/MI/recurrent events) => target INR 3-4

Question 9

How is APS related to SLE

Answer 9

APS can be secondary to SLE

-having both increases organ damage and mortality