Antiphospholipid syndrome (275/1700)

A 32yo female with 3 prv 1st trimester miscarriages is dx with antiphospholipid syndrome. Anticardiolipin antibodies +ve. She is now 18wks pregnant. What would be the most appropriate
management?

a. Aspirin
b. Aspirin & warfarin
c. Aspirin & heparin
d. Heparin only
e. Warfarin only






























answer: C
Definition
• multi-system vasculopathy manifested by recurrent thromboembolic events, spontaneous abortions, and thrombocytopenia
• often presents with migraine-type headaches
• circulating antiphospholipid autoantibodies interfere with coagulation cascade

• primary APLA: occurs in the absence of other disease
• secondary APLA: occurs in the setting of a connective tissue disease (including SLE), malignancy, drugs (hydralazine, procainamide, phenytoin, interferon, quinidine), and infections (HIV, TB, hepatitis C, infectious mononucleosis)
• catastrophic APLA: development within 1 wk of small vessel thrombotic occlusion in ≥3 organ systems with positive antiphospholipid Ab (high mortality)


CLINICAL

• Vascular thrombosis Arterial: stroke/TIA, multi-infarct dementia, MI, valvular incompetence, limb ischemia
• Venous: DVT, PE, renal and retinal vein thrombosis
• Pregnancy: Fetal death (>10 wk GA), recurrent spontaneous abortions (<10 wk GA) or premature birth (<34 wk GA)
• livedo reticularis, Raynaud’s phenomenon, purpura, leg ulcers, and gangrene

LAB: AB must be positive on 2 occasions, at least 12 wk apart

• SLE anticoagulant
• Anti-cardiolipin Ab IgG and/or IgM
• Anti-β2 glycoprotein-I Ab IgG and/or IgM 
• thrombocytopenia, hemolytic anemia, neutropenia

Treatment

• Thrombosis

lifelong anti-coagulation with warfarin, target INR 2.0-3.0 for first venous event, >3.0 for recurrent and/or arterial event

•  Recurrent fetal loss

heparin/low molecular weight heparin ± Aspirin during pregnancy