Antiphospholipid syndrome

Question 1

what is the definition of antiphospholipid syndrome?

Answer 1

Antiphospholipid syndrome (APS), also known as antiphospholipid antibody syndrome, is the association of persistent antiphospholipid antibodies (lupus anticoagulant, anticardiolipin antibody, and/or anti-beta2-glycoprotein I) with a variety of clinical features characterised by thromboses and pregnancy-related morbidity.

Question 2

what are the risk factors for APS?

Answer 2

history of systemic lupus erythematosus
history of other autoimmune rheumatological disorders
history of other autoimmune diseases
history of autoimmune haematological disorders

Question 3

what are the key presentations for APS?

Answer 3

High index of suspicion for antiphospholipid syndrome in any young patient (<50 years) presenting with arterial thrombosis in any vessel.
history or current diagnosis of vascular thrombosis
history of pregnancy loss
history of pregnancy-associated morbidity

Question 4

what are the signs of APS?

Answer 4

history of systemic lupus erythematosus
features of thrombocytopenia
arthralgia/arthritis
livedo reticularis

Question 5

what are the first line and gold standard investigations for APS?

Answer 5

lupus anticoagulant
anticardiolipin antibodies
anti-beta2-glycoprotein I antibodies
antinuclear antibody, double-stranded DNA, and extractable nuclear antigen antibodies

Question 6

what are the differential diagnoses for APS?

Answer 6

Sneddon’s syndrome

Inherited thrombophilia

Question 7

how is APS managed?

Answer 7

Main treatment goals are management of acute thrombosis and prevention of thrombosis recurrence and pregnancy morbidity.
Non-pregnant:
Heparin, warfarin, management of risk factors
Pregnant:
Heparin, foetal monitoring, warfarin, risk factor management, aspirin, warfarin post delivery, venous thromboembolism thromboprophylaxis