Department of Rheumatology and Immunology, Huadong Hospital affiliated to Fudan University, #221 Yan'an West Road, Shanghai, 200040, People's Republic of China.
Arthritis Res Ther. 2022 Apr 29;24(1):98. doi: 10.1186/s13075-022-02782-1.
Behçet's syndrome (BS) is a rare variant vasculitis which can involve the eyes and gastrointestinal systems. However, ocular involvement rarely overlaps with intestinal lesions. This study aimed to compare the clinical characteristics and laboratory parameters of ocular BS and intestinal BS patients in China and analyze the differences between two key phenotypes to verify the heterogeneous conditions in BS patients.
A retrospective analysis was used to collect the demographic data, clinical characteristics, endoscopic findings, and laboratory parameters from 135 ocular BS and 174 intestinal BS patients. The Mann-Whitney U test and Pearson chi-square or continuity correction was used to analyze the differences between two groups.
Among 916 BS patients enrolled in this study, ocular BS and intestinal BS accounted for 14.74% (135 cases) and 19.00% (174 cases), respectively. Ocular and intestinal involvements overlapped in only 7 cases (0.76%). Male gender (74.8% vs. 51.1%, P=0.00), erythema nodosum (45.9% vs. 32.2%, P=0.01), and vascular involvement (6.7% vs. 1.7%, P=0.03) were more frequent in the ocular BS group compared with the intestinal BS group. On the contrary, hematologic involvement (7.5% vs. 0.0%, P=0.00) and fever (17.8% vs. 4.4%, P=0.00) were more frequent in the intestinal BS group compared with the ocular BS group. Additionally, the inflammation markers including ESR [26.5 (16.0-41.5) vs. 9.0 (5.0-15.0) mm/H, P=0.00], CRP [14.8 (4.8-33.0) vs. 4.1 (1.6-8.3) mg/L, P=0.00], serum amyloid A [27.4 (10.8-92.3) vs. 11.3 (6.0-24.0) mg/L, P=0.00], and interleukin 6 [8.4 (1.7-18.7) vs. 1.7 (1.5-3.2) pg/mL, P=0.00] were higher in the intestinal BS group than those in the ocular BS group, respectively.
Ocular BS was more prevalent in male patients and more likely to manifest with erythema nodosum and vascular involvement, while intestinal BS tends to have fever and hematologic disorders with higher inflammation markers. Ocular BS and intestinal BS are two distinct clinical phenotypes and very rarely overlapped.
贝赫切特综合征（BS）是一种罕见的血管炎变异体，可累及眼睛和胃肠道系统。然而，眼部受累很少与肠道病变重叠。本研究旨在比较中国眼部 BS 和肠道 BS 患者的临床特征和实验室参数，并分析两种关键表型之间的差异，以验证 BS 患者的异质性。
采用回顾性分析收集了 135 例眼部 BS 和 174 例肠道 BS 患者的人口统计学数据、临床特征、内镜检查结果和实验室参数。采用 Mann-Whitney U 检验和 Pearson chi-square 或连续性校正检验分析两组间的差异。
在本研究纳入的 916 例 BS 患者中，眼部 BS 和肠道 BS 分别占 14.74%（135 例）和 19.00%（174 例）。仅有 7 例（0.76%）出现眼部和肠道重叠受累。与肠道 BS 组相比，眼部 BS 组男性（74.8% vs. 51.1%，P=0.00）、结节性红斑（45.9% vs. 32.2%，P=0.01）和血管受累（6.7% vs. 1.7%，P=0.03）更为常见。相反，与肠道 BS 组相比，血液学受累（7.5% vs. 0.0%，P=0.00）和发热（17.8% vs. 4.4%，P=0.00）更为常见。此外，炎症标志物包括 ESR[26.5（16.0-41.5）vs. 9.0（5.0-15.0）mm/H，P=0.00]、CRP[14.8（4.8-33.0）vs. 4.1（1.6-8.3）mg/L，P=0.00]、血清淀粉样蛋白 A[27.4（10.8-92.3）vs. 11.3（6.0-24.0）mg/L，P=0.00]和白细胞介素 6[8.4（1.7-18.7）vs. 1.7（1.5-3.2）pg/mL，P=0.00]在肠道 BS 组中分别高于眼部 BS 组。
眼部 BS 更常见于男性患者，更可能表现为结节性红斑和血管受累，而肠道 BS 则倾向于发热和血液系统疾病，伴有更高的炎症标志物。眼部 BS 和肠道 BS 是两种不同的临床表型，很少重叠。
