antiphospholipid syndrome Flashcards
define antiphospholipid syndrome
An autoimmune disorder characterised by inflammation, thromboembolism, and high levels of antiphospholipid antibodies.
risk factors for antiphospholipid syndrome
- Diabetes
- HTN
- Obesity
- Female
- Smoking
- Oestrogen therapy (Menopause)
pathophysiology of antiphospholipid syndrome
- Those with APS make abnormal proteins called antiphospholipid autoantibodies in the blood.
- These antibodies bind to the phospholipids on the surface of cells, causing inflammation and increasing the risk of thrombosis.
- This causes blood to flow improperly and can lead to dangerous clotting in arteries and veins
- problems for a developing fetus and pregnancy miscarriage.
what are the specific antiphospholipid antibodies are:
- Lupus anticoagulant
- Anticardiolipin antibodies
- Anti-beta-2 glycoprotein I antibodies
what causes antiphospholipid syndrome
can occur on its own
or
2˚ to AI disorder (esp. SLE)
what is Catastrophic Antiphospholipid Syndrome
a rare complication, with rapid thrombosis in multiple organs within a few days.
This has a high mortality rate.
main features of antiphospholipid syndrome
CLOT:
Clots – usually venous thromboembolism
Livedo reticularis – mottled, lace-like appearance of the skin on lower limbs
Obstetric loss – recurrent miscarriages, pre-eclampsia, and premature births
Thrombocytopenia
other features of antiphospholipid syndrome
- Valve disease (non-bacterial growths)
- Aortic and mitral regurgitation/stenosis
- increased risk of arterial and venous thrombosis
how to diagnose antiphospholipd syndrome
- FBCs to show thrombocytopenia
- One or more of the following are needed on 2 separate occasions, 12 weeks apart, to confirm the diagnosis:
1. Anticardiolipin antibodies
2. Anti-beta-2 glycoprotein I antibodies
3. Positive lupus anticoagulant assay
what can cause a false positive for antiphospholipid syndrome and how to avoid this
Syphilis can cause false-positive tests, so should be excluded using treponemal serology (test for syphilis).
how to treat antiphospholipid syndrom
Long-term warfarin with a target INR of 2-3 to prevent thrombosis
how to treat antiphospholipid syndrome in pregnancy
Low molecular weight heparin and aspirin are used in pregnancy (warfarin in teratogenic)