Antiphospholipid Syndrome (APS) is an autoimmune condition that produces antibodies against phospholipids (fat molecules)
Presentation: recurrent miscarriages, DVTs and arterial clots
Antibodies: lupus anticoagulant, beta glycoprotein and anti-cardiolipin
Management: warfarin or LWMH in pregnancy
Antiphospholipid antibodies target phospholipid binding proteins, resulting in arterial or venous clots. APS can be primary of secondary to Systemic Lupus Erythematous (40%).
Incidence: 1/ 100,000.
History
Miscarriage
Examination
Livedo reticularis
Cognitive or renal impairment (from clots)
Investigations
Antibodies: lupus anticoagulant, beta glycoprotein and anti-cardiolipin
Long APTT (don't correct with mixing of normal plasma)
Diagnosis: clinical, based upon:
Antibodies- repeated 12 weeks later
Lupus anti-coagulant
Beta glycprotein
Anti-cardiolipin
2. Clinical features
Clots- VTE or arterial
Pregnancy loss
Differentials: thrombophilias
Classification: aetiology
Primary/ idiopathic
Secondary
Acute & long term: warfarin
Pregnancy: LMWH +- aspirin
Long Term Management
Unprovoked DVTs in the context of APS have a high rate of re-occurrence, therefore anticoagulation is usually lifelong. Warfarin (or heparin during pregnancy) is standard.
Hughes Syndrome
Old name for APS
Page written in 2025.