Antiphospholipid syndrome

  • A Correction to this article was published on 25 January 2018

Abstract

Antiphospholipid syndrome (APS) is an autoimmune disease characterized by the presence of antiphospholipid antibodies, such as lupus anticoagulant, anticardiolipin antibodies and anti-β2-glycoprotein 1 antibodies. APS can present with a variety of clinical phenotypes, including thrombosis in the veins, arteries and microvasculature as well as obstetrical complications. The pathophysiological hallmark is thrombosis, but other factors such as complement activation might be important. Prevention of thrombotic manifestations associated with APS includes lifestyle changes and, in individuals at high risk, low-dose aspirin. Prevention and treatment of thrombotic events are dependent mainly on the use of vitamin K antagonists. Immunosuppression and anticomplement therapy have been used anecdotally but have not been adequately tested. Pregnancy morbidity includes unexplained recurrent early miscarriage, fetal death and late obstetrical manifestation such as pre-eclampsia, premature birth or fetal growth restriction associated with placental insufficiency. Current treatment to prevent obstetrical morbidity is based on low-dose aspirin and/or low-molecular-weight heparin and has improved pregnancy outcomes to achieve successful live birth in >70% of pregnancies. Although hydroxychloroquine and pravastatin might further improve pregnancy outcomes, prospective clinical trials are required to confirm these findings.

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Figure 1: Pathophysiology of antiphospholipid antibody-associated thrombosis.
Figure 2: Effect of antiphospholipid antibodies on trophoblasts.
Figure 3: Clinical manifestation of antiphospholipid syndrome.

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Acknowledgements

The authors thank the Guy's and St Thomas’ Charity for their support of K.S.

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Introduction (G.R.-I.); Epidemiology (S.S.); Mechanisms/pathophysiology (J.E.S. and P.G.d.G.); Diagnosis, screening and prevention (K.D., Y.S. and O.S.); Management (K.S. and B.J.H.); Quality of life (K.S. and S.S.); Outlook (S.J.); Overview of Primer (K.S. and B.J.H.).

Corresponding author

Correspondence to Beverley J. Hunt.

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Competing interests

J.E.S. has received an investigator-initiated grant from UCB. The other authors declare no competing interests.

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Schreiber, K., Sciascia, S., de Groot, P. et al. Antiphospholipid syndrome. Nat Rev Dis Primers 4, 17103 (2018). https://doi.org/10.1038/nrdp.2017.103

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