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  • Review Article
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Current treatment of antiphospholipid syndrome: lights and shadows

Nature Reviews Rheumatology volume 11, pages 586–596 (2015)Cite this article

Subjects

Key Points

  • For patients with definite antiphospholipid syndrome (APS) who experience a first venous thrombotic event, the recommended treatment is prolonged use of oral anticoagulation therapy to maintain a target international normalized ratio (INR) of 2.0–3.0

  • For patients with definite APS and arterial thrombosis, several therapies have been proposed: anticoagulation therapy with a target INR 3.0–4.0; antiplatelet therapy alone; anticoagulation therapy with a target INR 2.0–3.0; or the combination of antiplatelet and anticoagulation therapy (target INR 2.0–3.0)

  • Although combined therapy with low-dose aspirin and low-molecular-weight heparin is the mainstay of treatment for women with obstetric APS, the strength of the evidence for the efficacy of this approach is still a matter of debate

  • The treatment of seronegative APS (as well as cases where antiphospholipid antibodies 'disappear' over time), thrombotic and obstetric manifestations refractory to conventional treatment, and manifestations not included in APS classification criteria is still uncertain

  • For areas of APS treatment for which published evidence is scarce or nonexistent, recommendations are based on clinical judgement and physician experience

Abstract

For patients with antiphospholipid syndrome (APS), the consensus is to treat those who develop thrombosis with long-term oral anticoagulation therapy and to prevent obstetric manifestations by use of aspirin and heparin. These recommendations are based on data from randomized controlled trials and observational studies. Despite this body of knowledge, areas of uncertainty regarding the management of APS exist where evidence is scarce or nonexistent. In other words, for a subset of patients the course of management is unclear. Some examples are patients with 'seronegative' APS, those who do not fulfil the formal (clinical or serological) classification criteria for definite APS, and those with recurrent thrombotic events despite optimal anticoagulation. Other challenges include the treatment of clinical manifestations not included in the classification criteria, such as haematologic manifestations (thrombocytopenia and haemolytic anaemia), neurologic manifestations (chorea, myelitis and multiple sclerosis-like lesions), and nephropathy and heart valve disease associated with antiphospholipid antibodies (aPL), as well as the possible withdrawal of anticoagulation treatment in selected cases of thrombotic APS in which assays for aPL become persistently negative. This Review focuses on the current recommendations for thrombotic and obstetric manifestations of APS, as well as the management of difficult cases. Some aspects of treatment, such as secondary prophylaxis of venous thrombosis, are based on strong evidence—the 'lights' of APS treatment. Conversely, other areas, such as the treatment of non-criteria manifestations of APS, are based only on expert consensus or common sense and remain the 'shadows' of APS therapy.

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Authors and Affiliations

  1. Department of Autoimmune Diseases, Hospital Clinic, Villarroel 170, Barcelona, 08036, Catalonia, Spain

    Gerard Espinosa & Ricard Cervera

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  1. Gerard Espinosa
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  2. Ricard Cervera
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G.E. researched data for the article and wrote the manuscript. Both authors provided a substantial contribution to discussions of the content and to review and/or editing of the manuscript before submission.

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Correspondence to Gerard Espinosa.

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The authors declare no competing financial interests.

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Espinosa, G., Cervera, R. Current treatment of antiphospholipid syndrome: lights and shadows. Nat Rev Rheumatol 11, 586–596 (2015). https://doi.org/10.1038/nrrheum.2015.88

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