Antiphospholipid Syndrome

Question 1

Define

Answer 1

Characterised by the presence of antiphospholipid antibodies (APL) in the plasma, venous and arterial thrombosis, recurrent foetal loss and thrombocytopaenia

Question 2

Causes

Answer 2

• Antiphospholipid antibodies (APL) are directed against plasma proteins bound to phospholipids
• APL may develop in susceptible individuals following exposure to infectious agents
• Once APL are present, a second event is needed for the syndrome to develop
• APL has effects on a number of coagulation factors (e.g. protein C, annexin V, platelets, fibrinolysis)
• Complement activation by APL is critical for the complications

Cause CLOTS:
Coagulation defect
Livedo reticularis
Obstetric (recurrent miscarriage) Thrombocytopenia ( platelets)

Question 3

Epidemiiiology

Answer 3

More common in YOUNG WOMEN

Accounts for 20% of strokes in < 45 yrs

Accounts for 27% of women with > 2 miscarriages

Question 4

Symptoms

Answer 4

Recurrent miscarriages

• History of arterial thrombosis (stroke)
• History of venous thrombosis (DVT, pulmonary
• embolism)
• Headaches (migraine)
• Chorea
• Epilepsy

Question 5

Signs

Answer 5

• Livedo reticularis (mottled discolouration of the skin)
• Signs of SLE (malar flush, discoid lesions,photosensitivity)
• Signs of valvular heart disease

Question 6

Investigations

Answer 6

FBC - low platelets

ESR - usually normal

U&Es - can get APL nephropathy

Clotting screen - high APTT

Presence of antiphospholipid antibodies may be demonstrated by:

• ELISA testing for anticardiolipin antibodies
• Lupus anticoagulant assays