儿童白塞综合征44例临床特征分析

Analysis of Clinical Features in 44 Pediatric Cases of Behçet’s Syndrome

摘要: 目的：分析总结44例白塞综合征(Behçet’s syndrome, BS)患儿临床特征，提高临床医生对该病的认识及诊治水平。方法：回顾性分析2013年6月至2023年6月青岛大学附属医院儿童肾脏风湿免疫科住院治疗的44例儿童BS病例资料，总结其临床表现、实验室及辅助检查、治疗方案及转归情况。结果：44例BS患儿中，男性24例、女性20例(男女比1.2:1)。发病年龄3~17岁(均值10.1 ± 4.0岁)。42例患儿(95.5%)出现口腔溃疡，30例患儿(68.2%)出现生殖器溃疡，女性患儿的生殖器溃疡发生率显著高于男性(P < 0.001)。16例患儿(36.4%)出现皮疹，10例患儿(22.7%)眼部受累，9例患儿(20.5%)伴有关节症状，2例患儿(4.5%)伴有神经系统症状，1例患儿(2.2%)出现肺栓塞。根据2014年白塞病国际临床评分标准(International Criteria for Behçet’s Disease, ICBD)诊断标准，39例(88.6%)患儿符合该标准，余5例患儿经消化内镜病理活检确诊。44例患儿中38例(86.4%)治疗后症状缓解。27例患儿(61.4%)治疗方案包含沙利度胺，23例患儿症状缓解。9例(20.5%)治疗方案中包含糖皮质激素，6例缓解。5例(11.4%)患儿加用阿达木单抗。4例缓解。结论：儿童BS临床表现以复发性口腔溃疡、生殖器溃疡、葡萄膜炎、皮疹及消化道症状为特征。女性患儿生殖器溃疡发生率高于男性，以沙利度胺和糖皮质激素为基础的个体化综合治疗可显著缓解症状。

Abstract: Objective: To analyze and summarize the clinical characteristics of 44 pediatric patients with Behçet’s syndrome (BS) in order to improve clinicians’ diagnosis and treatment of this disease. Methods: A retrospective analysis was conducted on the clinical data of 44 pediatric BS cases hospitalized in the Department of Pediatric Nephrology, Rheumatology, and Immunology at the Affiliated Hospital of Qingdao University from June 2013 to June 2023. Their clinical manifestations, laboratory and auxiliary examinations, treatment regimens, and outcomes were summarized. Results: Among the 44 pediatric BS patients, there were 24 males and 20 females (male-to-female ratio 1.2:1). The age of onset ranged from 3 to 17 years (mean 10.1 ± 4.0 years). Oral ulcers occurred in 42 patients (95.5%), and genital ulcers in 30 patients (68.2%), with a significantly higher incidence of genital ulcers in female patients than in males (P < 0.001). Skin lesions occurred in 16 patients (36.4%), ocular involvement in 10 (22.7%), joint symptoms in 9 (20.5%), neurological symptoms in 2 (4.5%), and pulmonary embolism in 1 (2.2%). According to the 2014 International Criteria for Behçet’s Disease (ICBD), 39 patients (88.6%) met the diagnostic criteria; the remaining 5 cases were confirmed by gastrointestinal endoscopy and pathological biopsy. Symptom remission was achieved in 38 (86.4%) of the 44 patients after treatment. The treatment regimen included thalidomide for 27 patients (61.4%), with 23 achieving symptom remission. Glucocorticoids were included in the treatment for 9 patients (20.5%), with 6 achieving remission. Adalimumab was added for 5 patients (11.4%), with 4 achieving remission. Conclusion: The clinical manifestations of pediatric BS are characterized by recurrent oral ulcers, genital ulcers, uveitis, skin rashes, and gastrointestinal symptoms. The incidence of genital ulcers is higher in female patients than in males. Individualized comprehensive treatment based on thalidomide and glucocorticoids can significantly alleviate symptoms.