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Antiendothelial Cells Autoantibodies in Vasculitis-Associated Systemic Diseases

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Abstract

Antiendothelial cell antibodies (AECA) have been detected in healthy individuals, as well as in autoimmune and systemic inflammatory diseases, including systemic vasculitides. AECA have been reported in large vessel vasculitides such as giant cell arteritis and Takayasu arteritis; medium-sized vessel vasculitides, such as polyarteritis nodosa related to hepatitis B virus infection and Kawasaki disease; and small-sized vessel vasculitides, such as Wegener’s granulomatosis, microscopic polyangiitis, and Henoch–Schonlein purpura. In Takayasu arteritis and antineutrophil cytoplasm antibody-positive vasculitides, AECA have been reported to correlate with disease activity. A cell-based enzyme-linked immunosorbent assay (ELISA) using cultured human umbilical vein endothelial cells (HUVEC) represent one of the reference techniques for AECA detection, although flow cytometry and immunobloting have also been proposed. AECA might contribute to the pathogenesis of systemic vasculitides and vasculitis-associated diseases through (1) activation of endothelial cells (EC), (2) direct cytotoxic effect due to complement-dependent cytotoxicity or indirect cytotoxic effect secondary to antibody-dependent cytotoxicity, (3) induction of coagulation, (4) induction of apoptosis through the binding of phospholipids or heat-shock protein 60, and (5) induction of EC activation. None of the identified target antigens of AECA is specific for EC, and EC-specific target antigens of AECA remain to be identified in systemic vasculitides.

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Acknowledgements

Philippe Guilpain is on a grant from the Fondation pour la Recherche Médicale (FRM).

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  1. Faculty of Medicine, Paris Descartes University, UPRES EA 4058, Paris, France

    Philippe Guilpain & Luc Mouthon

  2. Faculty of Medicine, Department of Internal Medicine and Reference Center for Vasculitides and Systemic Sclerosis, Cochin Hospital, Assistance Publique Hôpitaux de Paris, Paris Descartes University, Paris, France

    Philippe Guilpain & Luc Mouthon

  3. Laboratoire d’Immunologie, Paris Descartes University, UPRES EA 4058, Pavillon Gustave Roussy, 4e étage, 8 rue Méchain, 75014, Paris, France

    Luc Mouthon

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Guilpain, P., Mouthon, L. Antiendothelial Cells Autoantibodies in Vasculitis-Associated Systemic Diseases. Clinic Rev Allerg Immunol 35, 59–65 (2008). https://doi.org/10.1007/s12016-007-8069-3

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