title: Update on the pathogenesis and treatment of the antiphospholipid syndrome.
abstract: OBJECTIVE
  Many advancements in our understanding of the pathogenic mechanisms of the antiphospholipid syndrome (APS) have been accomplished over the recent months. Such progresses are paralleled by the development of innovative pharmacological tools that could provide novel therapeutic windows in APS management. The most recent and innovative findings about the biologic effects of antiphospholipid antibodies (aPLs) and the treatment APS will be hereby critically appraised.
  
  
  RESULTS
  Antibodies against the domain I of β2 glycoprotein I (β2GPI) are increasingly recognized as the main pathogenic subset; pioneer therapeutic options exploiting the pathogenicity of anti-domain I antibodies have been developed. AnnexinA2 and toll-like receptor (TLR)4 have been identified as the main receptors for β2GPI/anti-β2GPI antibodies on target cells; additional co-receptors might include TLR1, TLR2 and TLR6. Upon binding, aPLs engage intracellular mediators as nuclear factor kappa B and mammalian target of rapamycin, which provide potential therapeutic targets. Current innovative treatment options include novel oral anticoagulants and the complement inhibitor eculizumab. The addition to standard treatment of pleiotropic agents such as hydroxychloroquine, statins and vitamin D could allow better disease control.
  
  
  CONCLUSIONS
  The lively and intense research in the APS field opens new frontiers in aPL pathogenic mechanisms, as well as diagnosis and treatment of the syndrome.
  http://links.lww.com/COR/A26.
authors.affiliation: aDepartment of Clinical Sciences and Community Health, University of Milan bExperiment Laboratory of ImmunoRheumatologic Researches, IRCCS Istituto Auxologico Italiano, Milan, Italy.
authors.forename: Cecilia Beatrice
authors.initials: CB
authors.lastname: Chighizola
authors.forename: Elena
authors.initials: E
authors.lastname: Raschi
authors.forename: M Orietta
authors.initials: MO
authors.lastname: Borghi
authors.forename: Pier Luigi
authors.initials: PL
authors.lastname: Meroni
mesh_terms: D017152:Antibodies, Antiphospholipid / Q000276:immunology; D016736:Antiphospholipid Syndrome / Q000276:immunology* / Q000628:therapy; D015551:Autoimmunity; D006801:Humans
chemical_list: D017152:Antibodies, Antiphospholipid