anti-phospholipid syndrome

Question 1

what is it?

Answer 1

autoimmune disorder

Question 2

how does it manifest?

Answer 2

recurrent venous or arterial thrombosis and/or foetal loss

Question 3

what are causes?

Answer 3

HLA-DR7 + environmental triggers
primary = young women
secondary = association with SLE

Question 4

what is pathophysiology?

Answer 4

antiphospholipid antibodies react against phospholipids on plasma membranes

Question 5

what does the presence of anti-phospholipid antibodies lead to?

Answer 5

hypercoaguable state

Question 6

how does is clinically present - venous?

Answer 6

DVT/PE
livedo reticularis

Question 7

what is livedo reticularis?

Answer 7

blood clots in capillaries lead to swelling of venules resulting in purplish, net-like discolouration of skin

Question 8

how does it clinically present - arterial?

Answer 8

increased frequency of stroke or MI

Question 9

what investigations are used?

Answer 9

bloods

Question 10

what will bloods show?

Answer 10

thrombocytopenia
prolongation of aPTT

Question 11

how is it diagnosed?

Answer 11

1 clinical + 1 lab diagnosis

Question 12

what antibodies will be positive?

Answer 12

antiphospholipid antibody
lupus anticoagulant
anti cardiolipin
anti-beta 2 glycoprotein

Question 13

what is management?

Answer 13

lifelong anti-coagulant

Question 14

what is 1st line anticoagulant?

Answer 14

LMWH then warfarin or aspirin prophylaxis

Question 15

what is the management for pregnancy?

Answer 15

LMWH and aspirin

Question 16

what is the exception for specific patients for anti-coagulation?

Answer 16

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

Question 17

what are the complications?

Answer 17

recurrent miscarriage