Pathogenetic Aspects of Autoantibodies to Endothelial Cells in Systemic Vasculitis

  • Geoffrey Frampton
  • Daniel Chan
  • Nadia Khouri
  • J. Stewart Cameron
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 336)


The vascular endothelium is no longer considered to be little more than a passive, semipermeable, thrombo-resistant membrane which lines blood vessels. It is now clearly apparent because of the major advances in endothelial cell culture techniques (1) that the endothelium constitutes a dynamic interface between the blood and the rest of the body. Autoantibodies that recognise antigens on endothelial cells (AECA) have been described in many disorders with obvious vascular pathology (2–7). AECA have been detected by their ability to bind to cultured human umbilical vein endothelial cells (HUVEC) growing on plastic ELISA plates, or detected using functional assays, in which the end point is monocyte, neutrophil, or complement-dependent cytolysis. Recently, several groups (8–10), including are own (11), have reported the presence of AECA in ANCA positive Wegener’s granulomatosis (WG) and microscopic polyarteritis (MPA). However, little is known of the target autoantigens in systemic vasculitis or whether these antibodies are of pathogenetic significance. Therefore the aim of this study was to determine in more detail the relationship between AECA, ANCA and disease activity in patients with WG or MPA.


Human Umbilical Vein Endothelial Cell Kawasaki Disease Systemic Vasculitis Mixed Connective Tissue Disease Line Blood Vessel 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    E.A. Jaffe, R.L. Nachman, C.G. Becker, and C.R. Minick, Culture of human endothelial cells derived from umbilical veins, J. Clin. Invest. 52: 2745 (1973).PubMedCrossRefGoogle Scholar
  2. 2.
    D.B. Cines, A.P. Lyss, M. Reeber, R.J. DeHoratious, Presence of complement-fixing anti-endothelial antibodies in sytemic lupus erythematosus, J. Clin. Invest. 73: 61 (1984).Google Scholar
  3. 3.
    C.M. Holt, N. Lindsey, J. Moult, et al. Antibody-dependent cellular cytotoxicity of vascular endothelium: characterisation and pathogenic associations in systemic sclerosis, Chit. Exp. Immunol. 78: 358 (1989).Google Scholar
  4. 4.
    D.Y.M. Leung, T. Collins, L.A. Lapierre, R.S. Geha, and J.S Pober, Immunoglobulin M antibodies present in the acute phase of Kawasaki syndrome lyse cultured vascular endothelial cells stimulated by gamma-interferon, J. Clin. Invest.77:1428 (1986).Google Scholar
  5. 5.
    D.Y.M. Leung, J.L. Moake, P.L. Havens, M. Kim, and J.S. Pober. Lytic antiendothelial cell antibodies in haemolytic-uraemic syndrome, Lancet. iî: 183 (1988).Google Scholar
  6. 6.
    D.B. Cines, A. Tomaski, and S. Tannenbaum, Immune endothelial cell injury in heparin-associated thrombocytopenia, N. Engl. J. Med 316: 581 (1987).PubMedCrossRefGoogle Scholar
  7. 7.
    A.M.M. Miltenberg, M.E. Meijer-Paape, J.J. Weening, M.R. Daha, L.A. van Es, and F.J. Van der Woude. Induction of antibody-dependent cellular cytotoxicity against endothelial cells by renal tranplantation, Transplantation. 48: 681 (1989).Google Scholar
  8. 8.
    L. Brasille, J.M. Kremer, J.L. Clarke, and J. Cerilli, Identification of an autoantibody to vascular endothelial cell-specific antigens in patients with systemic vasculitis, Am. J. Med 87: 74 (1989).CrossRefGoogle Scholar
  9. 9.
    G. Ferraro, P.L. Meroni, A. Tincani, et al. Anti-endothelial cell antibodies in patients with Wegener’s granulomatosis and micropolyarteritis, Clin. Exp. Immunol. 79: 47 (1990).PubMedCrossRefGoogle Scholar
  10. 10.
    C.O.S Savage, B.E Pottinger, G. Gaskin, et al. Vascular damage in Wegener’s granulomatosis and microscopic polyarteritis: presence of anti-endothelial cell antibodies and their relationship to anti-neutrophil antibodies, Clin. Exp. Immuno185:14 (1991).Google Scholar
  11. 11.
    G. Frampton, D.R.W. Jayne, G.J. Perry, C.M. Lockwood, and J.S. Cameron, Autoantibodies to endothelial cells and and neutrophil cytoplasmic antigens in systemic vasculitis, Clin. Exp. Invnunol. 82: 227 (1990).CrossRefGoogle Scholar
  12. 12.
    D.P. DeBono, D.E. Macintyre, D.J.G. White, and J.L. Gordon, Endothelial adenine uptake as an assay for cell-or complement-mediated cytotoxicity, Immunol 32: 221 (1977).Google Scholar
  13. 13.
    P.E. Short, C.E. Williams, A.M. Picken, and F.G.H. Hill, Factor VIII related antigen: an improved enzyme immunoassay, Med. Lab. Sci. 39: 357 (1982).Google Scholar
  14. 14.
    T.M. Chan, G. Frampton, D.R.W. Jayne, et al. Clinical significance of anti-endothelial cell antibodies in systemic vasculitis: a longitudinal study comparing AECA and ANCA. Submitted for publication.Google Scholar
  15. 15.
    P.L. Meroni, N.D. Papa, A. Sinico, et al. Antiendothelial cell antibodies in primary vasculitides, Contr. Nephrol 94: 89 (1991).Google Scholar
  16. 16.
    R.J. Falk, R.S. Terrell, L.A. Charles, and J.C. Jennette, Antineutrophil cytoplasmic autoantibodies induce neutrophils to degranulate and produce oxygen radicals in vitro, Proc. Natl. Acad Sci. USA. 87: 4115 (1990).PubMedCrossRefGoogle Scholar
  17. 17.
    T.M. Chan, G. Frampton, N.A. Staines et al. Different mechanisms by which anti-DNA MoAbs bind to human endothelial cells and glomerular mesangial cells, Clin. Exp. Immunol 88: 68 (1992).PubMedCrossRefGoogle Scholar
  18. 18.
    T.M. Chan, G. Frampton, G.J. Perry et al. Binding of anti-DNA antibodies to endothelial cells influences the expression of adhesion molecules, Lupus. 1 (Suppl 1 ) (1992) (Abstract).Google Scholar
  19. 19.
    G.J. Perry, N. Gregson, T. M. Chan, J.S. Cameron, and G. Frampton, Western blot analysis of membrane epitopes recognised by anti-endothelial cell antibodies in SLE. In press (1992).Google Scholar

Copyright information

© Springer Science+Business Media New York 1993

Authors and Affiliations

  • Geoffrey Frampton
    • 1
  • Daniel Chan
    • 1
  • Nadia Khouri
    • 1
  • J. Stewart Cameron
    • 1
  1. 1.Renal Laboratory Clinical Science Laboratories UMDSGuy’s HospitalLondonUK

Personalised recommendations