Department of Rheumatology and Immunology, Huadong Hospital Affiliated To Fudan University, #221 yan'an west Road, Shanghai, 200040, People's Republic of China.
Orphanet J Rare Dis. 2021 Mar 17;16(1):132. doi: 10.1186/s13023-021-01772-x.
Intestinal Behçet's syndrome (BS) has high morbidity and mortality rates with serious complications. The purpose of this study was to investigate the clinical characteristics and laboratory parameters of intestinal and mucocutaneous BS patients and analyze the risk factors of intestinal involvement in BS patients.
A retrospective analysis was used to collect the demographic data and laboratory parameters from 97 intestinal and 154 mucocutaneous BS patients. Univariate and multivariate logistic regression analyses were used to investigate the risk factors of intestinal involvement in BS patients.
The most common clinical manifestations of first onset in intestinal BS patients were oral ulceration (100.00%), followed by genital ulcers (62.89%) and erythema nodule (28.87%), gastrointestinal lesions (28.87%), pseudofolliculitis (25.77%), fever (17.53%), arthritis (16.49%), ocular involvement (5.15%), while the least common were vascular involvement (2.06%) and hematologic involvement involvement (2.06%). The most common intestinal segment involved in intestinal BS patients was terminal ileum (30.9%), followed by ileocecal (18.6%), colon (15.5%). By univariate logistic regression analysis, gender, age at hospitalization, age of disease onset, BDCAF, T-SPOT, fever, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), leukocyte, erythrocyte, hemoglobin (HGB), neutrophil-to-lymphocyte ratio, serum amyloid A, complement 3, albumin, total cholesterol, high-density lipoprotein and interleukin 6 (IL-6) were found all risk factors of intestinal involvement in BS patients (P < 0.05 or P = 0.00). Moreover, gender (male), BDCAF (≥ 2), ESR (≥ 15 mm/H), CRP (> 10 mg/L), HGB (< 130 g/L) and IL-6 (> 7 pg/ml) were found the independent risk factors of intestinal involvement in BS patients (all P < 0.05).
More attention shall be paid to gender, BDCAF, ESR, CRP, HGB and IL-6 in BS patients. When gender (male), BDCAF (≥ 2), ESR (≥ 15 mm/H), CRP (> 10 mg/L), HGB (< 130 g/L) and IL-6 (> 7 pg/ml) being observed, it may reminds that the presence of intestinal involvement in BS patients.
肠型贝赫切特综合征（BS）发病率和死亡率高，且并发症严重。本研究旨在探讨肠型和黏膜皮肤型 BS 患者的临床特征和实验室参数，并分析 BS 患者肠道受累的危险因素。
采用回顾性分析方法，收集 97 例肠型和 154 例黏膜皮肤型 BS 患者的人口统计学数据和实验室参数。采用单因素和多因素 logistic 回归分析方法，探讨 BS 患者肠道受累的危险因素。
肠型 BS 患者首发的最常见临床表现为口腔溃疡（100.00%），其次为生殖器溃疡（62.89%）、结节红斑（28.87%）、胃肠道病变（28.87%）、假性毛囊炎（25.77%）、发热（17.53%）、关节炎（16.49%）、眼部受累（5.15%），而血管受累（2.06%）和血液系统受累（2.06%）最少见。肠型 BS 患者最常见的受累肠段为回肠末端（30.9%），其次为回盲部（18.6%）、结肠（15.5%）。单因素 logistic 回归分析发现，性别、住院时年龄、发病年龄、BDCAF、T-SPOT、发热、红细胞沉降率（ESR）、C 反应蛋白（CRP）、白细胞、红细胞、血红蛋白（HGB）、中性粒细胞与淋巴细胞比值、血清淀粉样蛋白 A、补体 3、白蛋白、总胆固醇、高密度脂蛋白和白细胞介素 6（IL-6）均为 BS 患者肠道受累的危险因素（P<0.05 或 P=0.00）。此外，性别（男性）、BDCAF（≥2）、ESR（≥15mm/H）、CRP（>10mg/L）、HGB（<130g/L）和 IL-6（>7pg/ml）为 BS 患者肠道受累的独立危险因素（均 P<0.05）。
BS 患者应注意性别、BDCAF、ESR、CRP、HGB 和 IL-6。当观察到性别（男性）、BDCAF（≥2）、ESR（≥15mm/H）、CRP（>10mg/L）、HGB（<130g/L）和 IL-6（>7pg/ml）时，可能提示 BS 患者存在肠道受累。
