Division of Rheumatology and Immunology, Shanghai Key Laboratory of Clinical Geriatric Medicine, Research Center on Aging and Medicine, Huadong Hospital, Fudan University, 200040, Shanghai, People's Republic of China.
Department of Biostatistics, School of Public Health, Fudan University, Shanghai, People's Republic of China.
Arthritis Res Ther. 2021 Jan 30;23(1):45. doi: 10.1186/s13075-021-02429-7.
Behcet's syndrome (BS) is a complex, heterogeneous disorder. However, classification of its subgroups is still debated. The purpose of this study was to investigate the clinical features and aggregation of patients with BS in China, based on manifestations and organ involvements.
This was a cross-sectional study of BS patients in Huadong Hospital of Fudan University between September 2012 and January 2020. We calculated relative risks (RRs) of clinical variables according to sex. Moreover, we conducted a hierarchical cluster analysis applied according to eighteen variables to determine subgroups of patients.
A total of 860 BS patients were included. Male sex was associated with ocular involvement (RR 2.32, 95% CI 1.67, 3.22, P < 0.0001), vascular involvement (RR 2.00, 95% CI 1.23, 3.23, P = 0.004), cardiac lesion (RR 5.46, 95% CI 2.33, 12.77, P < 0.0001), and central nervous system involvement (RR 2.95, 95% CI 1.07, 6.78, P = 0.007) and was negatively associated with genital ulcers (RR 0.84, 95% CI 0.79, 0.91, P < 0.0001). Five clusters (C1-C5) were observed. C1 (n = 307) showed the skin and mucosa type. In C2 (n = 124), all had articular involvement, barely having major organ involvement except for 18 cases with intestinal lesions. In C3 (n = 156), the gastrointestinal type, 144 patients presented with intestinal involvement, and 36 patients with esophageal ulcers. In C4 (n = 142), all subjects presented with uveitis. C5 (n = 131) consisted of 44 patients with cardiac lesions, 58 with vascular involvement, and 26 cases having central nervous system involvement.
Our analysis confirmed sex differences in phenotypes of BS. Cluster analysis identified gastrointestinal, uveitis, and cardiovascular involvement cluster separately in different subsets, which represents the most commonly involved organs. Further research is required to replicate and clarify the patterns of phenotype in BS.
贝赫切特综合征（BS）是一种复杂、异质性的疾病。然而，其亚组的分类仍存在争议。本研究旨在基于表现和器官受累情况，探讨中国 BS 患者的临床特征和聚集情况。
这是一项横断面研究，纳入 2012 年 9 月至 2020 年 1 月期间在复旦大学华山医院就诊的 BS 患者。我们根据性别计算了临床变量的相对风险（RR）。此外，我们根据 18 个变量进行层次聚类分析，以确定患者亚组。
共纳入 860 例 BS 患者。男性与眼部受累（RR 2.32，95%CI 1.67，3.22，P<0.0001）、血管受累（RR 2.00，95%CI 1.23，3.23，P=0.004）、心脏病变（RR 5.46，95%CI 2.33，12.77，P<0.0001）和中枢神经系统受累（RR 2.95，95%CI 1.07，6.78，P=0.007）相关，与生殖器溃疡（RR 0.84，95%CI 0.79，0.91，P<0.0001）呈负相关。观察到 5 个聚类（C1-C5）。C1（n=307）表现为皮肤黏膜型。C2（n=124）中所有患者均有关节受累，除 18 例肠道病变外，几乎无主要器官受累。C3（n=156）为胃肠道型，144 例患者表现为肠道受累，36 例患者表现为食管溃疡。C4（n=142）所有患者均有葡萄膜炎。C5（n=131）包括 44 例心脏病变、58 例血管受累和 26 例中枢神经系统受累。
本分析证实了 BS 表型存在性别差异。聚类分析分别在不同亚组中确定了胃肠道、葡萄膜炎和心血管受累聚类，这代表了最常受累的器官。需要进一步研究来复制和阐明 BS 的表型模式。
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