Antiphospholipid syndrome

Karen Schreiber, Savino Sciascia, Philip G. De Groot, Katrien Devreese, Soren Jacobsen, Guillermo Ruiz-Irastroza, Jane E. Salmon, Yehuda Shoenfeld, Ora Shovman, Beverley J. Hunt*

*Corresponding author for this work

Research output: Contribution to journalJournal articleResearchpeer-review


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.

Original languageEnglish
Article number17103
JournalNature Reviews Disease Primers
Publication statusPublished - 11. Jan 2018
Externally publishedYes


  • Adult
  • Anti-Inflammatory Agents, Non-Steroidal/therapeutic use
  • Antibodies, Antiphospholipid/adverse effects
  • Anticoagulants/therapeutic use
  • Antiphospholipid Syndrome/diagnosis
  • Aspirin/therapeutic use
  • Female
  • Humans
  • Pregnancy
  • Pregnancy Complications
  • Quality of Life
  • Risk Assessment/methods
  • Thrombosis/etiology


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