Research Center of Systemic Auto Inflammatory Diseases, Behçet's Disease and Rheumatology-Ophthalmology Collaborative Uveitis Center, Rheumatology Unit, Policlinico "Le Scotte", Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Viale Bracci 16, 53100, Siena, Italy.
Department of Life Sciences and Public Health, Fondazione Policlinico A. Gemelli IRCCS, Rome, Italy.
Intern Emerg Med. 2021 Nov;16(8):2163-2171. doi: 10.1007/s11739-021-02725-9. Epub 2021 Apr 9.
Behçet's syndrome (BS) represents an understudied topic in pediatrics: the main aims of our study were to characterize demographic and clinical features of a cohort of BS patients with juvenile-onset managed in three tertiary referral centers in Italy, evaluate their evolution in the long-term, and detect any potential differences with BS patients having an adult-onset. Medical records of 64 juvenile-onset and 332 adult-onset BS followed-up over a 2-year period were retrospectively analyzed and compared. Mean age ± SD of first symptom-appearance was 10.92 ± 4.34 years with a female-to-male ratio of 1.06:1. Mucocutaneous signs were the most frequent initial manifestations, followed by uveitis. Throughout the disease course, genital aphthae (76.56%) and pseudofolliculitis (40.63%) prevailed among the mucocutaneous signs, while major organ involvement was represented by gastrointestinal and ocular involvement (43.75 and 34.38%, respectively). No significant differences emerged for both mucocutaneous signs and specific major organ involvement between juvenile-onset and adult BS patients. After excluding nonspecific abdominal pain, juvenile-onset BS patients were less frequently characterized by the development of major organ involvement (p = 0.027). Logistic regression detected the juvenile-onset as a variable associated with reduced risk of long-term major organ involvement (OR 0.495 [0.263-0.932], p = 0.029). In our cohort, juvenile-onset BS resembled the clinical spectrum of adult-onset patients. Pediatric patients with a full-blown disease at onset showed a more frequent mucocutaneous involvement. In addition, patients with juvenile-onset seemed to develop less frequently major organ involvement and had an overall less severe disease course.
贝切特综合征（BS）是儿科领域中一个研究较少的课题：我们的主要研究目的是对在意大利三家三级转诊中心接受治疗的 BS 幼年发病患者的人群特征和临床表现进行描述，评估他们的长期演变情况，并发现任何与成人发病 BS 患者之间存在的潜在差异。回顾性分析了 64 例幼年发病和 332 例成年发病 BS 患者的医疗记录，并对其进行了为期 2 年的随访。首发症状的平均年龄为 10.92±4.34 岁，女性与男性的比例为 1.06:1。黏膜皮肤表现是最常见的首发症状，其次是葡萄膜炎。在整个疾病过程中，黏膜皮肤表现中生殖器溃疡（76.56%）和假性毛囊炎（40.63%）最为常见，而主要器官受累则以胃肠道和眼部受累为主（分别为 43.75%和 34.38%）。在黏膜皮肤表现和特定的主要器官受累方面，幼年发病与成年发病 BS 患者之间无显著差异。排除非特异性腹痛后，幼年发病 BS 患者的主要器官受累发生率较低（p=0.027）。Logistic 回归分析发现，幼年发病是与长期主要器官受累风险降低相关的变量（OR 0.495 [0.263-0.932]，p=0.029）。在我们的队列中，幼年发病 BS 与成年发病患者的临床表现相似。在发病时即表现为全面疾病的儿科患者，黏膜皮肤受累更为频繁。此外，幼年发病患者似乎较少发生主要器官受累，且总体疾病病程较轻。
