Thrombosis and Anti-phospholipid Syndrome: a 5-Year Update on Treatment
Purpose of Review
The aim of this review is to provide an update of the therapeutic tools for thrombotic anti-phospholipid syndrome (APS), focusing on the last 5 years.
Early studies appointed anticoagulation at moderate intensity as the mainstay of treatment of thrombotic APS; in the last 5 years, the strategy has not much mutated. Some uncertainties regarding the role of direct oral anticoagulants and the optimal regimen for arterial thrombotic APS still persist: high-intensity anticoagulation, anticoagulation plus anti-platelet agent, and double anti-platelet agents being the possible alternatives. Several drugs have been proposed as effective additional tools for the management of thrombotic APS: hydroxychloroquine, statins, vitamin D, and sirolimus might be beneficial when added on the top of anticoagulation. Pregnant women with thrombotic APS should be switched to low-dose aspirin plus low molecular weight heparin at therapeutic dose. Despite adequate treatment, APS patients display a significant rate of recurrences; rituximab, eculizumab, and intravenous immunoglobulins are among the options to be considered for these patients.
From 2013 to date, the kaleidoscope of therapeutic options in thrombotic APS has been enriched, but tangible improvements in the management of patients are still awaited.
KeywordsAnti-phospholipid antibodies Anti-phospholipid syndrome Thrombosis Treatment Update
Compliance with Ethical Standards
Conflict of Interest
The authors declare that they have no conflict of interest.
Human and Animal Rights and Informed Consent
This article does not contain any studies with human or animal subjects performed by any of the authors.
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- 7.•• Bala MM, Celinska-Lowenhoff M, Szot W, et al. Antiplatelet and anticoagulant agents for secondary prevention of stroke and other thromboembolic events in people with antiphospholipid syndrome. Cochrane Database Syst Rev. 2017;10:doi: https://doi.org/10.1002/14651858. This is a 2017 Cochrane review assessing the beneficial effects of anti-platelet agents and anticoagulation in anti-phospholipid syndrome.
- 11.• Sciascia S, Coloma-Bazán E, Radin M, et al. Can we withdraw anticoagulation in patients with antiphospholipid syndrome after seroconvertion? Autoimmun Rev. 2017;16:1109–14. This recent review discusses the available evidence on the potential withdrawal of anticoagulation in patients who turn negative for anti-phospholipid antibodies. CrossRefPubMedGoogle Scholar
- 14.Coloma Bazán E, Donate López C, Moreno Lozano P, Cervera R, Espinosa G. Discontinuation of anticoagulation or antiaggregation treatment may be safe in patients with primary antiphospholipid syndrome when antiphospholipid antibodies became persistently negative. Immunol Res. 2013;56:358–61.CrossRefPubMedGoogle Scholar
- 16.•• Chighizola CB, Andreoli L, Gerosa M, Tincani A, Ruffatti A, Meroni PL. The treatment of anti-phospholipid syndrome: a comprehensive clinical approach. J Autoimmun. 2018; https://doi.org/10.1016/j.jaut.2018.02.003. This is a recently published review that extensively reports all the possible treatment strategies for anti-phospholipid syndrome, critically appointed by experts of the field.
- 20.Jackson WG, Oromendia C, Unlu O, Erkan D, DeSancho M, Antiphospholipid Syndrome Alliance for Clinical Trials and International Networking. Recurrent thrombosis in patients with antiphospholipid antibodies and arterial thrombosis on antithrombotic therapy. Blood Adv. 2017;1:2320–4.CrossRefPubMedPubMedCentralGoogle Scholar
- 24.Riancho-Zarrabeitia L, Cubería M, Muñoz P, López-Hoyos M, García-Canale S, García-Unzueta M, Hernández JL, Martínez-Taboada VM Vitamin D and antiphospholipid syndrome: a retrospective cohort study and meta-analysis. Semin Arthritis Rheum 2017; https://doi.org/10.1016/j.semarthrit.2017.10.007.
- 30.Chighizola CB, Raimondo MG, Meroni PL. Management of thrombotic antiphospholipid syndrome. Semin Thromb Hemost 2017; https://doi.org/10.1055/s-0036-1597282.
- 37.Andreoli L, Bertsias GK, Agmon-Levin N, Brown S, Cervera R, Costedoat-Chalumeau N, et al. EULAR recommendations for women’s health and the management of family planning, assisted reproduction, pregnancy and menopause in patients with systemic lupus erythematosus and/or antiphospholipid syndrome. Ann Rheum Dis. 2017;76:476–85.CrossRefPubMedPubMedCentralGoogle Scholar