Antiphospholipid syndrome

Question 1

define antiphospholipid syndrome

Answer 1

characterised by the presence of antiphospholipid antibodies ( APL) in the plasma, venous and arterial thromboses, recurrent foetal loss and thrombocytopenia

Question 2

what is obstetric APS ?

Answer 2

Female with antiphospholipid antibodies and a history of pregnancy related morbidity but no history of thrombosis-> considered to have obstetric APS

Question 3

summarise the epidemiology of antiphospholipid syndrome?

Answer 3

more common in young females- accounts for 27% of females with more than 2 misscarriages

Question 4

what are the risk factors for antiphospholipid syndrome?

Answer 4

history of SLE
history of other autoimmune rheumatology disorders
history of autoimmune haematological disorders

Question 5

explain the aetiology/ risk factors for antiphospholipid syndrome?

Answer 5

unclear what acc causes development of antibodies in first place

usually occrus as primary disease but 20-30% are associated with SLE

APL directed against plasma proteins bound to anionic phosholipids eg beta 2-GP-I, prothrombin, annexin A5 and phospholipids

APL may develop in susceptible individuals ( eg SLE patients, following exposure to infectious agents

APL is present, a second hit is required for the development of the syndrome

Question 6

what are the presenting symptoms of antiphospholipid syndrome?

Answer 6

• recurrent misscarriages
• history of arterial thromboses- stroke
• history of venous thrombosis- DVT, PE
• history of SLE or other AI rheumatological disorders
• headaches, migraines
• chorea
• epilepsy

Question 7

what are the signs of antiphospholipid syndrome on physical examination?

Answer 7

livedo reticularis - mottled purplish discolouration ( Blanching or non-blanching)

signs of SLE- malar flush, discoid lesion, photosensitivity, arthalgia

signs of valvular disease- caridac murmur

features of thrombocytopen- petechia, mucosal bleeding

Question 8

describe the antibody investigations?

Answer 8

anti-cardolipin positive

lupus anti-coagulant test positive

Question 9

what investigations should you consider?

Answer 9

venous Doppler ultrasound
venography or MRI
MRI of thrombosis
CT angiogram of the chest

Question 10

what is it considered as if a patient has antiphospholipid antibodies but no thrombotic or related obstetric complications?

Answer 10

Do not fit criteria for APS-> considered to have incidental antiphospholipid antibodies

->patients at increased risk of thrombosis but not possible to identify which specific patients are at risk