Direskeneli H, Keser G, D'Cruz D, Khamashta M A, Akoğlu T, Yazici H, Yurdakul S, Hamuryudan V, Ozgün S, Goral A J
Lupus Arthritis Research Unit, Rayne Institute, St. Thomas' Hospital, London, UK.
Clin Rheumatol. 1995 Jan;14(1):55-61. doi: 10.1007/BF02208085.
Endothelial cell dysfunction has previously been demonstrated in Behçet's disease which has vasculitic features. In this study we investigated anti-endothelial 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.
内皮细胞功能障碍先前已在具有血管炎特征的白塞病中得到证实。在本研究中，我们调查了白塞病患者的抗内皮细胞抗体（AECA）和血管性血友病因子抗原（vWF）水平。还评估了患者血清对内皮细胞增殖的体外作用。70例白塞病患者中有29%存在AECA（结合指数：25±29%，而正常对照组为9±7%，p<0.005）。AECA阳性患者中95%临床活动，而AECA阴性患者为74%（p=0.04）。在特定器官表现中，只有活动性关节炎与AECA阳性相关（7例患者中的6例 vs 63例中的14例，p=0.002）。AECA阳性患者的平均血沉显著更高（37±24mm/h vs 21±17mm/h，p：0.006）。患者的平均vWF水平也显著高于对照组（166±75% vs 84±34%，p<0.0001）。未观察到AECA滴度与vWF水平之间的相关性。研究内皮细胞增殖时，患者与对照组之间未观察到显著差异（增殖指数：1.25±0.28 vs 1.12±0.25，p=0.5）。我们的结果表明，AECA可能与白塞病的疾病活动有关。即使在完全缓解的患者中，vWF的存在可能与内皮细胞释放vWF的内皮损伤以外的因素有关。
