title: Delineating the deranged immune system in the antiphospholipid syndrome.
abstract: 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.
authors: Department of Rheumatology and Clinical Immunology, University Medical Centre Utrecht, Heidelberglaan 100, 3584 CX Utrecht, The Netherlands; Laboratory of Translational Immunology, University Medical Centre Utrecht, Heidelberglaan 100, 3584 CX Utrecht, The Netherlands. Electronic address: l.l.vandenhoogen@umcutrecht.nl.
authors: Lucas L
authors: LL
authors: van den Hoogen
authors: Department of Rheumatology and Clinical Immunology, University Medical Centre Utrecht, Heidelberglaan 100, 3584 CX Utrecht, The Netherlands; Laboratory of Translational Immunology, University Medical Centre Utrecht, Heidelberglaan 100, 3584 CX Utrecht, The Netherlands.
authors: Joël A G
authors: JA
authors: van Roon
authors: Department of Rheumatology and Clinical Immunology, University Medical Centre Utrecht, Heidelberglaan 100, 3584 CX Utrecht, The Netherlands; Laboratory of Translational Immunology, University Medical Centre Utrecht, Heidelberglaan 100, 3584 CX Utrecht, The Netherlands.
authors: Timothy R D J
authors: TR
authors: Radstake
authors: Department of Rheumatology and Clinical Immunology, University Medical Centre Utrecht, Heidelberglaan 100, 3584 CX Utrecht, The Netherlands.
authors: Ruth D E
authors: RD
authors: Fritsch-Stork
authors: Department of Rheumatology and Clinical Immunology, University Medical Centre Utrecht, Heidelberglaan 100, 3584 CX Utrecht, The Netherlands.
authors: Ronald H W M
authors: RH
authors: Derksen
mesh_terms: D000818:Animals; D017951:Antigen Presentation; D016736:Antiphospholipid Syndrome / Q000235:genetics / Q000276:immunology*; D001402:B-Lymphocytes / Q000276:immunology; D003713:Dendritic Cells / Q000276:immunology; D006801:Humans; D013601:T-Lymphocytes / Q000276:immunology
keywords: Anti-β2 glycoprotein I antibody; Antiphospholipid syndrome; Autoreactive T-cell; Dendritic cell; Monocyte; Neutrophil