CTD- Anti-phospholipid syndrome

Question 1

What is Anti-phospholipid syndrome (APS)

Answer 1

a disorder that manifests clinically as recurrent venous or arterial thrombosis and/or fetal loss.

Question 2

assoc with APS (3)

Answer 2

SLE or another rheumatic or autoimmune disorder.

Question 3

presentation/ common findings (6)

Answer 3

increased frequency of stroke or MI, especially in younger.

Pulmonary emboli/ thrombosis can lead to life-threat pulmonary hypertension

Migraine

Livedo reticularis

Late spontaneous fetal loss (second or third trimester)

Recurrent early fetal loss (< 10 weeks’ gestation)

Question 4

Catastrophic APS (CAPS)

Answer 4

rare, serious, and often fatal manifestation (mortality rate of approximately 50%) characterized by multiorgan infarctions over a period of days to weeks.

Question 5

Investigations (4)

Answer 5

There may be thrombocytopenia and prolongation of APTT.

Lupus anticoagulant

anti-cardiolipin antibodies

anti-beta 2 glycoprotein

Question 6

Management (3)

Answer 6

anti-coagulation for those with an episode of thrombosis

with recurrent pregnancy loss low molecular weight heparin is used during pregnancy (warfarin is teratogenic).

positive antibodies but who have never had had an episode of thrombosis do not require anti-coagulation.