Summary
Endothelial cell dysfunction has previously been demonstrated in Behçet's disease which has vasculitic features. In this study we investigated antiendothelial cell antibodies (AECA) and von Willebrand factor antigen (vWF) levels in patients with Behçet's disease. In vitro effects of patient sera on endothelial cell proliferation were also evaluated. AECAs were present in 29% of 70 Behçet's disease patients (Binding Index:25±29% vs 9±7% in normal controls, p<0.005). 95% of AECA positive patients were clinically active compared to 74% of AECA negative patients (p=0.04). Among specific organ manifestations only active arthritis correlated with AECA positivity (6 of 7 patients vs 14 of 63, p=0.002). AECA positive patients had a significantly higher mean ESR (37±24 mm/h vs 21±17 mm/h, p:0.006). Mean vWF levels were also significantly higher in patients compared to controls (166±75% vs 84±34%, p.<0.0001). No correlations were observed between AECA titres and vWF levels. No significant differences were observed between patients and controls when endothelial cell proliferation was studied (Proliferation Index: 1.25±0.28 vs 1.12±0.25, p=0.5). Our results suggest that AECA may be related to disease activity in Behçet's disease. The presence of vWF, even in patients in complete remission, might be related to factors other than endothelial damage for vWF release from endothelial cells.
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References
Müftüoğlu, A.U., Yurdakul, S., Yazici, H. et al. Vascular involvement in Behçet's disease—a review of 129 cases. In: Recent Advances in Behçet's Disease, Eds.: Lehner, T., Barnes, C.G., London, Royal Society of Medicine Services, 1986, 255–260.
Koç, Y., Güllü, I., Akpek, G. et al. Vascular involvement in Behçet's Disease. J Rheumatol 1992, 19, 402–410.
Kansu, E., Sahin, G., Sahin, F., Sivri, B., Sayek, I., Batman, F. Impaired prostacyclin synthesis by vessel walls in Behçet's Disease. Lancet 1986, ii, 1154.
Cines, D.B., Lyss, A.P., Reeber, M., Bina, M., DeHoratius, R.J. Presence of complement fixing anti-endothelial cell antibodies in systemic lupus erythematosus. J Clin Invest 1984, 73, 611–625.
Rosenbaum, J., Pottinger, B.E., Woo, P. et al. Measurement and characterization of circulating anti-endothelial cell IgG in connective tissue diseases. Clin Exp Immunol 1988, 72, 450–456.
Ferraro, G., Meroni, P.L., Tincani, A., et al. Anti-endothelial cell antibodies in patients with Wegener's granulomatosis and micropolyarteritis. Clin Exp Immunol 1990, 79, 47–53.
Savage, C.O.S., Pottinger, B.E., Gaskin, G., Lockwood, C.M., Pusey, C.D., Pearson, J.D. Vascular damage in Wegener's granulomatosis and microscopic polyarteritis: presence of anti-endothelial cell antibodies and their relation to anti-neutrophil cytoplasm antibodies. Clin Exp Immunol 1991, 85, 14–19.
D'Cruz, D.P., Houssiau, F.A., Ramirez, G., et al. Antibodies to endothelial cells in systemic lupus erythematosus: a potential marker for nephritis and vasculitis. Clin Exp Immunol 1991, 85, 254–261.
Del, Papa, N., Meroni, P.L., Barcellini, W. et al. Antibodies to endothelial cells in primary vasculitides mediate in vitro endothelial cytotoxicity in the presence of normal peripheral blood mononuclear cells. Clin Immunol Immunopathol 1992, 63, 267–274.
Frampton, G., Jayne, D.R.W., Perry, G.J., Lockwood, C.M., Cameron, J.S. Autoantibodies to endothelial cells and neutrophil cytoplasmic antigens in systemic vasculitis. Clin Exp Immunol 1990, 82, 227–232.
International study group for Behçet's disease. Criteria for diagnosis of Behçet's disease. Lancet 1990, 335, 1078–1080.
Hunder, G.G., Arend, W.P., Bloch, D.A. et al. The American College of Rheumatology 1990 Criteria for the classification of vasculitis. Arthritis Rheum 1990, 33, 1065–1108.
Jaffe, E.A., Nachman, R.L., Becker, C.G., Minick, C.R. Culture of human endothelial cells derived from umbilical cord veins. Identification by morphologic and immunologic criteria. J Clin Invest 1973, 52, 2745–2758.
Kahaleh, M.B., Sherer, G.K., LeRoy, E.C. Endothelial injury in scleroderma. J Exp Med 1979, 149, 1326–1335.
Short, P.E., Williams, C.E., Picken, A.M., Hill, F.G. Factor VIII related antigen: an improved enzyme immunoassay. Med Lab Sci 1982, 39, 351–355.
Aydintuğ, A.O., Tokgöz, G., D'Cruz, D.P. et al. Antibodies to endothelial cells in patients with Behçet's disease. Clin Immunol Immunopathol 1993, 67, 157–162.
Pivetti-Pezzi, P., Priori, R., Catarinelli, C. et al. Markers of vascular injury in Behçet's disease associated with retinal vasculitis. Ann Ophthalmol 1992, 24, 411–414.
Edelsten, C., D'Cruz, D., Hughes, G.R.V., Graham, E.M. Antiendothelial cell antibodies in retinal vasculitis. Curr Eye Res 1992, 11, 203–208.
Müftüoğlu, A.U., Yazici, H., Yurdakul, S. et al. Behçet's Disease. Relation of serum C-reactive protein and erythrocyte sedimentation rates to disease activity. Int J Dermatol 1986, 25, 235–239.
Meroni, P.L., Del Papa, N., Sinico, A. et al. Anti-endothelial cell antibodies in primary vasculitides. Contrib Nephrol Basel, Karger, 1991, 94, 89–97.
Niwa, Y., Miyake, S., Sakane, T., Shingu, M., Yokoyama, M. Autooxidative damage in Behçet's disease-endothelial cell damage following the elevated oxygen radicals generated by stimulated neutrophils. Clin Exp Immunol 1982, 49, 247–255.
Schmitz-Huebner, U., Knop, J. Evidence for an endothelial cell dysfunction in association with Behçet's Disease. Thromb Res 1984, 34, 277–285.
Hampton, K.K., Chamberlain A., Menon, D.K., Davies, J.A. Coagulation and fibrinolytic activity in Behçet's disease. Thromb Haemostas 1991, 66, 292–294.
Yazici, H., Hekim, N., Özbakir, F. et al. Von Willebrand factor in Behçet's syndrome. J Rheumatol 1987, 14, 305–306.
Woolf, A.D., Wakerley, G., Wallington, T.B., Scott, D.G.I., Dieppe, P.A. Factor VIII related antigen in the assessment of vasculitis. Ann Rheum Dis 1987, 46, 441–447.
Schorer, A.E., Moldow, C.F., Rick, M.E. Interleukin-1 or endotoxin increases the release of von Willebrand factor from human endothelial cells. Br J Haematol 1987, 67, 193–197.
Nawroth, P.P., Stern, D.M. Modulation of endothelial cell haemostatic properties by tumor necrosis factor. J Exp Med 1986, 163, 740–745.
Pottinger, B.E., Read, R.C., Paleolog, E.M., Higgins, P.G., Pearson, J.D. Von Willebrand Factor is an acute phase reactant in man. Thromb Res 1989, 53, 387–394.
Memişoğlu, H.R., Aksu, H.S.Z., Erken, E., Göcük, M. Serum IL-1 levels in Behçet's disease. In: Behçet's Disease. Basic and Clinical Aspects. Eds.: O'Duffy, J.D., Kökmen, E., New York, Marcel Dekker Inc, 1991, 387–391.
Akoğlu, T., Direskeneli, H., Yazici, H. et al. TNF-α, soluble IL-2R and soluble CD-8 levels in patients with Behçet's disease. J Rheumatol 1990, 17, 1107–1108.
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Direskeneli, H., Keser, G., D'Cruz, D. et al. Anti-endothelial cell antibodies, endothelial proliferation and von Willebrand factor antigen in Behçet's disease. Clin Rheumatol 14, 55–61 (1995). https://doi.org/10.1007/BF02208085
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DOI: https://doi.org/10.1007/BF02208085