title: Anti-endothelial cell antibodies in vasculitis: A systematic review.
abstract: Anti-endothelial cell antibodies (AECAs) are those that can bind to endothelial cells (ECs) via variable region-specific interactions. The identification and quantification of AECAs varies depending on the technique used. The best approach would be to combine at least two different methods. Thus, AECA measurement cannot be considered a diagnostic tool, but the detection and titers of AECAs are associated with disease activity in various systemic vasculitis diseases. AECAs have been described in almost all primary systemic vasculitis diseases but also in many secondary vasculitis diseases, with the identification of various antigens. AECAs may play a pathogenic role in vasculitis, both in vitro and in vivo, mainly via EC activation and induction of apoptosis. We used a systematic review of the literature to better define the prevalence, clinical association, targeted antigens, possible pathophysiologic role and clinical usefulness of AECAs in various types of vasculitis.
authors.affiliation: INSERM U1016, Institut Cochin, Paris, France; CNRS UMR 8104, Paris, France; Université Paris Descartes, Sorbonne Paris Cité, Paris, France; LABEX Inflamex, Université Sorbonne Paris Cité, 75013 Paris, France; Service de Médecine Interne, Centre de Référence Maladies Systémiques Autoimmunes Rares, Vascularites nécrosantes et sclérodermie systémique, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Paris, France.
authors.forename: Paul
authors.initials: P
authors.lastname: Legendre
authors.affiliation: INSERM U1016, Institut Cochin, Paris, France; CNRS UMR 8104, Paris, France; Université Paris Descartes, Sorbonne Paris Cité, Paris, France; LABEX Inflamex, Université Sorbonne Paris Cité, 75013 Paris, France; Service de Médecine Interne, Centre de Référence Maladies Systémiques Autoimmunes Rares, Vascularites nécrosantes et sclérodermie systémique, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Paris, France.
authors.forename: Alexis
authors.initials: A
authors.lastname: Régent
authors.affiliation: INSERM U1016, Institut Cochin, Paris, France; CNRS UMR 8104, Paris, France; Université Paris Descartes, Sorbonne Paris Cité, Paris, France; LABEX Inflamex, Université Sorbonne Paris Cité, 75013 Paris, France; Service de Médecine Interne, Centre de Référence Maladies Systémiques Autoimmunes Rares, Vascularites nécrosantes et sclérodermie systémique, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Paris, France.
authors.forename: Mathilde
authors.initials: M
authors.lastname: Thiebault
authors.affiliation: INSERM U1016, Institut Cochin, Paris, France; CNRS UMR 8104, Paris, France; Université Paris Descartes, Sorbonne Paris Cité, Paris, France; LABEX Inflamex, Université Sorbonne Paris Cité, 75013 Paris, France; Service de Médecine Interne, Centre de Référence Maladies Systémiques Autoimmunes Rares, Vascularites nécrosantes et sclérodermie systémique, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Paris, France. Electronic address: luc.mouthon@cch.aphp.fr.
authors.forename: Luc
authors.initials: L
authors.lastname: Mouthon
mesh_terms: D001323:Autoantibodies / Q000276:immunology*; D013700:Giant Cell Arteritis / Q000276:immunology*; D006801:Humans; D014657:Vasculitis / Q000276:immunology*
chemical_list: D001323:Autoantibodies; C087589:anti-endothelial cell antibody
keywords: ANCA; Anti-endothelial cell antibodies; Giant cell arteritis; Vasculitis