From the Guidelines
Antiphospholipid Syndrome (APS) diagnosis is dependent on the accurate detection and interpretation of antiphospholipid antibodies (aPL).
Workup for APS
- The laboratory criteria for APS include persistent lupus anticoagulant (LA), anticardiolipin (aCL), and anti-β2-glycoprotein I (aβ2GPI) antibodies 1.
- Moderate and high titer thresholds are set at 40 Unit (U) and 80 U levels, respectively 1.
- ELISA is the recommended method for measuring aCL and aβ2GPI 1.
- Interpretation of aPL results should always be related to clinical symptoms 1.
Management of APS
- Combined low-dose aspirin and prophylactic-dose heparin (usually LMWH) is recommended for patients meeting criteria for obstetric APS 1.
- Low-dose aspirin and therapeutic-dose heparin (usually LMWH) is recommended for patients with thrombotic APS 1.
- The addition of hydroxychloroquine (HCQ) to prophylactic-dose heparin or LMWH and low-dose aspirin therapy may be considered for patients with primary APS 1.
Clinical Considerations
- Classification criteria are distinct from the detection and interpretation of aPL in the day-to-day clinical setting 1.
- Patients with lower-titer aCL and/or anti-β2 GPI who do not meet laboratory classification criteria may still have some degree of risk 1.
- Decisions regarding therapy should be made on a case-by-case basis, taking into account additional relevant risk factors 1.
From the Research
Diagnosis and Management of Antiphospholipid Syndrome (APS)
- The diagnosis of APS requires a combination of clinical manifestations (thrombotic events and/or pregnancy morbidity) and the presence of antiphospholipid antibodies (aPL) 2.
- The clinical spectrum of APS encompasses various manifestations, including venous and arterial thrombosis, and pregnancy morbidity, which cannot be explained exclusively by a prothrombotic state 3.
- Risk stratification is essential, with "triple positive" patients having the highest risk of recurrent thrombosis 2.
Treatment of APS
- The primary goal of treatment is to reduce the risk of thrombotic events and pregnancy morbidity 4.
- Treatment with vitamin K antagonists (VKA) with a target international normalized ratio (INR) of 2-3 is recommended for patients with APS and first unprovoked venous thrombosis 2, 5.
- For patients with APS and first arterial thrombosis, treatment with VKA with INR 2-3 or INR 3-4 is recommended, considering the individual's bleeding/thrombosis risk 5.
- Low-dose aspirin (LDA) is recommended for asymptomatic aPL carriers, patients with systemic lupus erythematosus without prior thrombotic or obstetric APS, and non-pregnant women with a history of obstetric APS only, all with high-risk aPL profiles 5.
Management of Refractory or Difficult Cases
- Refractory forms of APS could benefit from adding hydroxychloroquine and/or intravenous immunoglobulin to anticoagulation therapy 3.
- Increasing the anticoagulation intensity of VKA, switching to low-molecular-weight heparin, or adding antiplatelet treatment may be considered for patients with recurrent thrombosis despite adequate treatment 6.
- Promising novel treatments include anti-B cell monoclonal antibodies, new-generation anticoagulants, and complement cascade inhibitors 3.
Pregnancy Management
- Combination treatment with LDA and prophylactic dosage heparin during pregnancy is recommended for women with prior obstetric APS 5.
- Increase of heparin to therapeutic dose, addition of hydroxychloroquine, or addition of low-dose prednisolone in the first trimester may be considered for patients with recurrent pregnancy complications 5.