What is the treatment for Antiphospholipid syndrome (APS)?

Treatment of Antiphospholipid Syndrome (APS)

For patients with antiphospholipid syndrome (APS), warfarin with a target INR of 2.0-3.0 is the first-line treatment for thrombotic APS, while direct oral anticoagulants (DOACs) should be avoided, especially in triple-positive patients. 1

Treatment Based on APS Classification

Thrombotic APS

• Initial treatment:

  • Parenteral anticoagulation (LMWH or unfractionated heparin) overlapping with warfarin initiation 1
  • Transition to warfarin with target INR 2.0-3.0 for long-term management 1

• For patients with ischemic stroke/TIA meeting APS criteria:

  • Warfarin anticoagulation is recommended to reduce recurrent stroke/TIA risk 2
  • Avoid rivaroxaban in patients with triple-positive antibodies (lupus anticoagulant, anticardiolipin, and anti-β2 glycoprotein-I) due to excess thrombotic events compared to warfarin 2, 1

• For high-risk APS patients (recurrent thrombosis despite therapeutic INR):

  • Consider increasing INR target range to 3.0-4.0 1
  • Consider adding low-dose aspirin (75-100 mg/day) 1

Obstetric APS

• For pregnant women with obstetric APS:

  • Low-dose aspirin plus prophylactic-dose LMWH is strongly recommended 2, 1
  • Avoid warfarin during pregnancy due to teratogenic effects 1
  • Consider adding hydroxychloroquine to standard therapy for primary APS 2

• For pregnant women with thrombotic APS:

  • Low-dose aspirin and therapeutic-dose heparin (usually LMWH) throughout pregnancy and postpartum 2

Catastrophic APS

• Requires triple therapy approach:
  • Therapeutic anticoagulation
  • High-dose glucocorticoids
  • Plasma exchange and/or intravenous immunoglobulins 1

Special Populations

Asymptomatic aPL-Positive Patients

• For pregnant women with positive aPL who don't meet criteria for APS:
  • Prophylactic aspirin (81-100 mg daily) during pregnancy for preeclampsia prophylaxis 2
  • Treatment should begin early (before 16 weeks) and continue through delivery 2

Patients with Isolated aPL (Not Meeting Full APS Criteria)

• Antiplatelet therapy alone is recommended 1

Treatment Duration

• Long-term (indefinite) anticoagulation is recommended while antiphospholipid antibodies persist 1, 3
• Short-term anticoagulation (6 months) appears less beneficial 3
• Regular monitoring of INR is essential, with attention to potential effects of antiphospholipid antibodies on coagulation tests 1

Emerging Therapies

• For refractory cases, especially catastrophic APS, biologics such as rituximab and eculizumab may be considered 4
• Hydroxychloroquine may be beneficial for APS patients with concomitant Systemic Lupus Erythematosus to reduce thrombosis risk 1

Important Caveats

• DOACs (apixaban, dabigatran, edoxaban, rivaroxaban) are not recommended for APS patients, especially those with triple-positive antibodies or arterial thrombosis 2, 1
• Control of vascular risk factors is essential in all APS patients 1
• Combination therapy of warfarin and aspirin does not clearly offer additional benefit over warfarin alone for venous thrombosis 3
• Treatment discontinuation might be considered in select low-risk primary APS patients whose antibodies become persistently negative, but this requires careful monitoring 5