Anti-phospholipid Syndrome

Question 1

Describe onset of APS

Answer 1

APS with no evidence of any definable associated disease OR APS in association with SLE or other rheumatic or auto-immune disorder

Question 2

How does APS present?

Answer 2

Increased frequency of stroke, MI

In situ thrombosis or embolisation from lesions of libman-sacks (sterile) endocarditis

Recurrent PE
-pulmonary hypertension

Migraine

Livedo reticularis

Question 3

What is catastrophic APS?

Answer 3

Rare, serious and often fatal manifestation characterised by multi organ infarctions over a period of days to weeks

Question 4

What is common in pregnancy?

Answer 4

Late spontaneous fetal loss (second or third trimester); can happen earlier as well

Question 5

What are the investigations?

Answer 5

FBC- thrombocytopenia

Prolongation of APTT

Lupus anticoagulant, anti-cardiolipin antibodies

Anti-beta 2 glycoprotein may be positive

Question 6

How is APS managed in those with thrombosis?

Answer 6

Anti-coagulation

Question 7

How is APS managed in those with recurrent fetal loss?

Answer 7

LMWH during pregnancy

WARFARIN IS TERATOGENIC

Question 8

If a patient has positive autoantibodies but no history of thrombosis what do you do?

Answer 8

Nothing