Antiphospholipid syndrome

Question 1

What is antiphospholipid syndrome?

Answer 1

a disorder associated with antiphospholipid antibodies where the blood becomes prone to clotting.

hyper-coagulable state

main associations are with thrombosis and complications in pregnancy, particularly recurrent miscarriage

Question 2

When does antiphospholipid syndrome arise?

Answer 2

can occur on its own or secondary to an autoimmune condition, particular systemic lupus erythematosus

Question 3

Association with antiphospholipid antibodies?

Answer 3

It is associated with antiphospholipid antibodies including:

Lupus anticoagulant
Anticardiolipin antibodies
Anti-beta-2 glycoprotein I antibodies

These antibodies interfere with coagulation and create a hypercoagulable state where the blood is more prone to clotting.

Question 4

Medical condition associations of antiphospholipid syndrome?

Answer 4

Venous thromboembolism: DVT , PE

Arterial thrombosis: Stroke, MI, renal thrombosis

Pregnancy: recurrent miscarriage, stillbirth, preeclampsia

Question 5

Other less common associations?

Answer 5

Livedo reticularis is a purple lace like rash that gives a mottled appearance to the skin.

Libmann-Sacks endocarditis is a type of non-bacterial endocarditis where there are growths (vegetations) on the valves of the heart. The mitral valve is most commonly affected. It is associated with SLE and antiphospholipid syndrome.

Thrombocytopenia (low platelets) is common in antiphospholipid syndrome.

Question 6

Diagnosis of antiphospholipid syndrome?

Answer 6

Diagnosis is made when there is a history of thrombosis or pregnancy complication plus persistent antibodies:

Lupus anticoagulant
Anticardiolipin antibodies
Anti-beta-2 glycoprotein I antibodies

Question 7

Management of antiphospholipid syndrome?

Answer 7

Patients are usually managed jointly between rheumatology, haematology and obstetrics (if pregnant).

Long term warfarin with an INR range of 2-3 is used to prevent thrombosis (3-4 may be used with recurrent thrombosis).

Pregnant women are started on low molecular weight heparin (e.g. enoxaparin) plus aspirin to reduce the risk of pregnancy complications. Warfarin is contraindicated in pregnancy.