Delineating the deranged immune system in the antiphospholipid syndrome

详细信息    查看全文

• 作者：Lucas L. van den Hoogen^a ; ^b ; ^{l.l.vandenhoogen@umcutrecht.nl" class="auth_mail" title="E-mail the corresponding author} ; Joë ; l A.G. van Roon^a ; ^b ; Timothy R.D.J. Radstake^a ; ^b ; Ruth D.E. Fritsch-Stork^a ; Ronald H.W.M. Derksen^a
• 关键词：aCL ; Anticardiolipin antibody ; aPL ; Antiphospholipid antibody ; apoER2 ; Apolipoprotein E receptor 2 ; aPS/PT ; Anti-phosphatidylserine/prothrombin antibody ; APS ; Antiphospholipid syndrome ; CAPS ; Catastrophic antiphospholipid syndrome ; CCP ; Complement control protein ; DC ; Dendritic cell ; ERK ; Extracellular-signal-regulated kinase ; FcγR ; Fc gamma receptor ; GM-CSF ; Granulocyte-macrophage colony-stimulating factor ; GWAS ; Genome wide association study ; HCQ ; Hydroxychloroquine ; IFN ; Interferon ; IL ; Inter
• 刊名：Autoimmunity Reviews
• 出版年：2016
• 出版时间：January 2016
• 年：2016
• 卷：15
• 期：1
• 页码：50-60
• 全文大小：320 K

文摘

The antiphospholipid syndrome (APS) is a systemic autoimmune disease that is characterized serologically by the presence of antiphospholipid antibodies (aPL) and clinically by vascular thrombosis and obstetric complications. The protein β2 glycoprotein I (β2GPI) is identified as the most important autoantigen in this syndrome. Activation of endothelial cells, thrombocytes and placental tissue by anti-β2GPI antibodies relates to the clinical manifestations of APS. This review describes genetic and environmental factors in relation to APS and summarizes the current knowledge on abnormalities in components of both the innate and adaptive immune system in APS. The role of dendritic cells, T-cells, B-cells, monocytes, neutrophils and NK-cells as well as the complement system in APS are discussed. Several gaps in our knowledge on the pathophysiology of APS are identified and a plea is made for future extensive immune cell profiling by a systems medicine approach in order to better unravel the pathogenesis of APS, to gain more insight in the role of the immune system in APS as well as having the potential to reveal biomarkers or novel therapeutic targets.