What is the treatment for antiphospholipid antibody syndrome?

Treatment for Antiphospholipid Antibody Syndrome

For patients with antiphospholipid syndrome (APS) with previous thrombosis, warfarin anticoagulation with a target INR of 2.0-3.0 is the recommended first-line therapy. 1

Diagnosis and Classification

APS is characterized by:

• Arterial and/or venous thrombosis
• Pregnancy morbidity
• Persistent presence of antiphospholipid antibodies (tested 12 weeks apart)
  • Lupus anticoagulant
  • Anti-cardiolipin antibodies
  • Anti-β2 glycoprotein-I antibodies

Treatment Algorithm Based on Clinical Presentation

1. Confirmed APS with History of Thrombosis

• First-line therapy: Warfarin with target INR 2.0-3.0 2, 1
• Duration: Indefinite anticoagulation 1, 3
• Monitoring:
  • Weekly INR monitoring during initiation
  • Monthly when stable 1

2. Isolated Antiphospholipid Antibody (without full APS criteria)

• Recommended therapy: Antiplatelet therapy (aspirin) 2
• This applies to patients with ischemic stroke or TIA who have an isolated antiphospholipid antibody but don't fulfill criteria for APS 2

3. APS with Arterial Thrombosis (including stroke)

• Recommended therapy: Warfarin with target INR 2.0-3.0 2, 1
• For patients with ischemic stroke or TIA who meet the criteria for APS, anticoagulation with warfarin is reasonable 2

4. APS in Pregnancy

• Warfarin is contraindicated in pregnancy
• Recommended therapy: Therapeutic-dose low-molecular-weight heparin (LMWH) 1

Special Considerations

Triple-Positive Antibody Patients

• Patients with all three antibodies (lupus anticoagulant, anticardiolipin, and anti-β2 glycoprotein-I) are at higher risk
• Important warning: DOACs, particularly rivaroxaban, are contraindicated in these patients due to excess thrombotic events compared to warfarin 2, 1

Refractory APS (Recurrent Thrombosis Despite Anticoagulation)

1. Verify medication compliance and proper INR levels
2. Consider:
   • Increasing LMWH dose by 25-30% if on therapeutic LMWH 1
   • Adding hydroxychloroquine 1
   • Adding intravenous immunoglobulin 1

APS with Neuropsychiatric Manifestations

• Antiplatelet and/or anticoagulation therapy is recommended for NPSLE (neuropsychiatric systemic lupus erythematosus) related to antiphospholipid antibodies 2
• Anticoagulation may be superior to antiplatelet therapy for secondary prevention of arterial events 2

Common Pitfalls and Caveats

1. Avoid DOACs in APS patients: Multiple guidelines recommend against routine use of DOACs in APS patients, particularly those with triple-positive antibodies 2, 1

2. Target INR range: While some older studies suggested higher INR targets (>3.0), current evidence supports a target INR of 2.0-3.0 to effectively balance thrombosis prevention against bleeding risk 2, 1

3. Testing timing: Testing for antiphospholipid antibodies should be repeated at least 12 weeks apart to confirm diagnosis 2

4. Primary prevention: For patients with persistently positive antiphospholipid antibodies but no prior thrombosis, data support potential benefit from antiplatelet agents 2

5. Pregnancy management: Requires specialized approach with LMWH rather than warfarin 1

The treatment of APS remains primarily focused on anticoagulation and antiplatelet therapy despite its autoimmune nature 4. Research into novel treatments including anti-B cell monoclonal antibodies and complement cascade inhibitors shows promise but has not yet changed standard clinical practice 5.