白塞氏综合征 (BS) 是一种以免疫失调为特征的血管炎。生物标志物对于评估临床非典型发病机制很有价值。我们的目的是在一项大规模的真实世界研究中调查不同生物标志物的分布及其对 BS 患者临床特征的影响。这是一项回顾性、单中心研究。总共有 502 名诊断为 BS 的患者参加了这项研究。我们分析了该队列的临床特征，并将患者的症状分为六类，包括皮肤粘膜、关节、神经、胃肠道、血管和眼部受累。对患者的 HLA-B51 细胞、自身抗体和免疫细胞亚群进行了测试。 Pearson相关性、Wilcoxon秩和检验和多元逻辑回归用于数据分析。 40.8%的BS患者血清中检测到多种自身抗体。抗内皮细胞抗体（AECA）的阳性率是自身抗体中最高的，在BS患者中检出率为23.5%（118/502）。 BS患者HLA-B51阳性率为27.1%。产生肿瘤坏死因子(TNF)-α、IL-2和IL-4的CD4 T细胞与胃肠道BS呈正相关。 IL-4 CD4 T 细胞增加是胃肠道 BS 的危险因素（P = 0.006，总率 [OR] = 2.491，95% 置信区间 [CI]：[1.317，5.100]）。 BS 患者中可检测到多种自身抗体。 HLA-B51 和 AECA 是最常见的生物标志物。 IL-4 CD4 T 细胞增加是 BS 胃肠道受累的危险因素。© 2024。作者。

Behcet's syndrome (BS) is a vasculitis characterized by immune dysregulation. Biomarkers are valuable for assessing clinically atypical pathogenesis. We aimed to investigate the distribution of different biomarkers and their effects on the clinical features of patients with BS in a large-scale, real-world study. This is a retrospective, single-center study. In total, 502 patients diagnosed with BS were enrolled in this study. We analyzed the clinical features of this cohort and divided patients' symptoms into six categories, including mucocutaneous, articular, neurological, gastrointestinal, vascular, and ocular involvements. HLA-B51 cells, autoantibodies, and subsets of immune cells from the patients were tested. Pearson's correlation, Wilcoxon rank sum test and multivariate logistic regression were used for data analysis. Various autoantibodies were detected in the serum of 40.8% of patients with BS. The positivity rate of anti-endothelial cell antibodies (AECA) was the highest among autoantibodies and was found in 23.5% (118/502) of patients with BS. The positivity rate of HLA-B51 in patients with BS was 27.1%. Tumor necrosis factor (TNF)-α, IL-2, and IL-4 producing CD4+ T cells were positively correlated with the gastrointestinal BS. Increased IL-4+CD4+ T cell was a risk factor for gastrointestinal BS (P = 0.006, Overall rate [OR] = 2.491, 95% Confidence interval [CI]: [1.317, 5.100]). Various autoantibodies can be detected in patients with BS. HLA-B51 and AECA are the most common biomarkers. Increased IL-4+ CD4+ T cell was a risk factor for gastrointestinal involvement in BS.© 2024. The Author(s).