title: Antiphospholipid syndrome: an update.
abstract: BACKGROUND
  Antiphospholipid syndrome (APS) or 'Hughes syndrome' is a prothrombotic disease characterized by thrombosis and pregnancy morbidity in the presence of antiphospholipid antibodies (aPL). More than three decades have passed, and experts are still uncovering new pieces of this disease complex pathogenesis and management.
  
  
  METHODS
  We searched in literature using MEDLINE and PubMed databases focusing on the latest development on disease pathogenesis, risk assessment of thrombosis and treatment of APS.
  
  
  RESULTS
  The phosphatidylinositol 3-kinase (PI3K)-AKT-mTORC pathway was most recently identified to have a crucial role in activating inflammation among endothelial vessel wall causing vascular lesions in APS. Additionally, new variables are being implemented to assess the risk of thrombosis in patients with APS. Global APS Score (GAPSS) utilizes cardiovascular risk factors and new autoimmune antibodies as part of the score assessment and is the most valid so far. It can be a promising tool in the future for prediction of thrombosis. Anticoagulation remains the cornerstone in APS; however, many new potential therapeutic agents are developing and are currently under investigation.
  
  
  CONCLUSIONS
  The most recent advances in pathogenesis, risk stratification and treatment provide a platform for high yield studies with the ultimate goal of providing the optimal management to patients with APS.
authors: Division of Rheumatology, Faculty of Medicine, The Royal London Hospital, London, UK.
authors: Mira
authors: M
authors: Merashli
authors: Faculty of Medicine, Lebanese American University Medical Center-Rizk Hospital, Beirut, Lebanon.
authors: Mohammad Hassan A
authors: MH
authors: Noureldine
authors: Division of Rheumatology, Faculty of Medicine, American University of Beirut, Beirut, Lebanon.
authors: Imad
authors: I
authors: Uthman
authors: Graham Hughes Lupus Research Laboratory, Division of Women's Health King's College London, The Rayne Institute, St Thomas' Hospital, London, UK.
authors: Munther
authors: M
authors: Khamashta
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000818:Animals; D017306:Annexin A2 / Q000037:antagonists & inhibitors; D000925:Anticoagulants / Q000627:therapeutic use; D016736:Antiphospholipid Syndrome / Q000209:etiology / Q000628:therapy*; D015507:Drugs, Investigational; D005260:Female; D006801:Humans; D006886:Hydroxychloroquine / Q000627:therapeutic use; D019161:Hydroxymethylglutaryl-CoA Reductase Inhibitors / Q000627:therapeutic use; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D020935:MAP Kinase Signaling System / Q000502:physiology; D051379:Mice; D019869:Phosphatidylinositol 3-Kinases / Q000378:metabolism; D010956:Plasmapheresis; D011247:Pregnancy; D011249:Pregnancy Complications, Cardiovascular / Q000209:etiology* / Q000517:prevention & control; D018570:Risk Assessment; D000069283:Rituximab; D055502:Secondary Prevention; D058570:TOR Serine-Threonine Kinases / Q000378:metabolism; D013925:Thromboplastin / Q000037:antagonists & inhibitors; D013927:Thrombosis / Q000209:etiology* / Q000517:prevention & control
chemical_list: D000305:Adrenal Cortex Hormones; D017306:Annexin A2; D000925:Anticoagulants; D015507:Drugs, Investigational; D006886:Hydroxychloroquine; D019161:Hydroxymethylglutaryl-CoA Reductase Inhibitors; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D019869:Phosphatidylinositol 3-Kinases; D000069283:Rituximab; D058570:TOR Serine-Threonine Kinases; D013925:Thromboplastin; C472822:ANXA2 protein, human; C546842:MTOR protein, human
keywords: Antiphospholipid antibodies; antiphospholipid syndrome; management; pathogenesis; risk stratification