title: Humoral autoimmunity against endothelium: theory or reality?
abstract: Despite the discovery of anti-endothelial cell antibodies (AECA) in a heterogeneous group of disorders characterized by endothelial damage, their pathogenic role is still debated. Experimental in vitro models indicate that they can either damage endothelial cells or trigger cell signaling by reacting with as yet undefined surface molecules. However, clinical studies suggest that, in addition to AECA, other pathogenic mechanisms are involved in the vasculitic process. Recently, antibodies specific for beta2 glycoprotein I, the phospholipid-binding protein targeted by anti-phospholipid antibodies, have been shown to display anti-endothelial activity. These autoantibodies recognize beta2 glycoprotein I adhered to the endothelium and induce a cell perturbation that might underlie the thrombophilic state of the anti-phospholipid syndrome.
authors.affiliation: Allergy, Clinical Immunology & Rheumatology Unit, IRCCS Istituto Auxologico Italiano, Department of Internal Medicine, University of Milan, Italy. pierluigi.meroni@unimi.it
authors.forename: Pierluigi
authors.initials: P
authors.lastname: Meroni
authors.forename: Nicoletta
authors.initials: N
authors.lastname: Ronda
authors.forename: Elena
authors.initials: E
authors.lastname: Raschi
authors.forename: Maria O
authors.initials: MO
authors.lastname: Borghi
mesh_terms: D000818:Animals; D000917:Antibody Formation / Q000276:immunology*; D000941:Antigens / Q000276:immunology; D016736:Antiphospholipid Syndrome / Q000276:immunology; D001323:Autoantibodies / Q000276:immunology; D015551:Autoimmunity / Q000276:immunology*; D004727:Endothelium / Q000276:immunology*; D006801:Humans
chemical_list: D000941:Antigens; D001323:Autoantibodies; C087589:anti-endothelial cell antibody