From the Guidelines
For patients with Antiphospholipid Syndrome (APS) who have experienced a venous thrombotic event, long-term anticoagulation with adjusted dose Vitamin K Antagonists (VKA) is recommended, with a target INR of 2.5, over direct oral anticoagulant (DOAC) therapy during the treatment phase. This recommendation is based on the most recent and highest quality study available, which suggests that VKA therapy is preferred over DOAC therapy in patients with APS 1.
Key Considerations for APS Management
- For patients with APS, anticoagulation therapy is crucial to prevent thrombotic events.
- The choice of anticoagulant and target INR may vary depending on the individual patient's risk factors and medical history.
- Regular monitoring of platelet count, liver function, and renal function is essential for patients on anticoagulation therapy.
- Hydroxychloroquine may be beneficial as adjunctive therapy, particularly in patients with concomitant lupus.
Special Considerations for Pregnant Women with APS
- Pregnant women with APS should receive prophylactic low molecular weight heparin (such as enoxaparin 40 mg daily or equivalent) plus low-dose aspirin throughout pregnancy and for 6 weeks postpartum.
- Women with recurrent pregnancy losses should receive the same regimen.
- Patients with catastrophic APS require aggressive treatment with therapeutic anticoagulation, high-dose corticosteroids, intravenous immunoglobulin, and sometimes plasma exchange.
General Principles of APS Management
- APS management focuses on preventing thrombotic events through anticoagulation therapy.
- The goal of treatment is to prevent the hypercoagulable state caused by autoantibodies against phospholipid-binding proteins that interfere with coagulation pathways and promote thrombosis.
- Treatment decisions should be individualized based on the patient's specific risk factors and medical history, and should take into account the latest available evidence and guidelines 2, 3, 4.
From the Research
Guidelines for Antiphospholipid Syndrome (APS) Management
- The standard anticoagulant treatment for thrombotic APS is life-long warfarin or an alternative vitamin K antagonist 5.
- Treatment with vitamin K-antagonists (VKA) with INR 2.0-3.0 is first-line treatment for a first or recurrent APS-related venous thrombotic event 6.
- Patients with first arterial thrombosis should be treated with clopidogrel or VKA with target INR 2.0-3.0 6.
- Low-dose aspirin is the first option for primary thromboprophylaxis in asymptomatic aPL carriers, and also plays a role as combination therapy with VKAs in arterial thrombosis 7.
- Adjuvant therapies such as hydroxychloroquine and statins may be useful in complex settings such as thrombotic recurrences or high risk of bleeding 7, 8.
Special Considerations
- In APS patients, lupus anticoagulant can affect phospholipid-dependent coagulation monitoring tests, so that they may not reflect true anticoagulation intensity 5.
- Anticoagulant dosing and monitoring in thrombotic APS patients also require consideration in anticoagulant-refractory APS and during pregnancy 5, 9.
- Patients with catastrophic APS, recurrent thrombotic events or recurrent pregnancy morbidity should be referred to an expert centre 6.
Treatment Options
- Other anticoagulant options include low molecular weight heparin, unfractionated heparin, and fondaparinux 5.
- Treatment with direct oral anticoagulants is not recommended for patients with arterial thrombosis or triple positive aPL 6, 7.
- Promising novel treatments include anti-B cell monoclonal antibodies, new-generation anticoagulants, and complement cascade inhibitors 8.