Department of Paediatric Rheumatology, CEREMAI, Bicêtre University Hospital, Paris SUD University of Medicine, Le Kremlin Bicêtre, France.
Rheumatology (Oxford). 2011 Jan;50(1):184-8. doi: 10.1093/rheumatology/keq324. Epub 2010 Oct 29.
To set-up an international cohort of patients suspected with Behçet's disease (BD). The cohort is aimed at defining an algorithm for definition of the disease in children.
International experts have defined the inclusion criteria as follows: recurrent oral aphthosis (ROA) plus one of following-genital ulceration, erythema nodosum, folliculitis, pustulous/acneiform lesions, positive pathergy test, uveitis, venous/arterial thrombosis and family history of BD. Onset of disease is <16 years, disease duration is ≤3 years, future follow-up duration is ≥4 years and informed consent is obtained. The expert committee has classified the included patients into: definite paediatric BD (PED-BD), probable PED-BD and no PED-BD. Statistical analysis is performed to compare the three groups of patients. Centres document their patients into a single database.
At January 2010, 110 patients (56 males/54 females) have been included. Mean age at first symptom: 8.1 years (median 8.2 years). At inclusion, 38% had only one symptom associated with ROA, 31% had two and 31% had three or more symptoms. A total of 106 first evaluations have been done. Seventeen patients underwent the first-year evaluation, and 36 had no new symptoms, 12 had one and 9 had two. Experts have examined 48 files and classified 30 as definite and 18 as probable. Twenty-six patients classified as definite fulfilled the International Study Group criteria. Seventeen patients classified as probable did not meet the international criteria.
The expert committee has classified the majority of patients in the BD group although they presented with few symptoms independently of BD classification criteria.
建立一个国际 Behçet 病（BD）疑似患者队列。该队列旨在制定儿童 BD 定义的算法。
国际专家定义了以下纳入标准：复发性口腔溃疡（ROA）加以下一种表现：生殖器溃疡、结节性红斑、毛囊炎、脓疱/痤疮样病变、阳性 PATHERGY 试验、葡萄膜炎、静脉/动脉血栓形成和 BD 家族史。疾病发作年龄<16 岁，疾病持续时间≤3 年，未来随访时间≥4 年，获得知情同意。专家委员会将纳入患者分为：明确的儿科 BD（PED-BD）、可能的 PED-BD 和无 PED-BD。对三组患者进行统计学分析。各中心将患者的病历记录在一个单一的数据库中。
截至 2010 年 1 月，共纳入 110 例患者（56 例男性/54 例女性）。首次出现症状时的平均年龄为 8.1 岁（中位数为 8.2 岁）。纳入时，38%的患者仅有与 ROA 相关的一种症状，31%的患者有两种症状，31%的患者有三种或更多症状。共进行了 106 次首次评估。17 例患者进行了第一年的评估，36 例无新症状，12 例有 1 个新症状，9 例有 2 个新症状。专家检查了 48 份病历，其中 30 份被归类为明确，18 份被归类为可能。26 例被归类为明确的患者符合国际研究组标准。17 例被归类为可能的患者不符合国际标准。
尽管这些患者的症状较少，但专家委员会将大多数患者归类为 BD 组，而不论其是否符合 BD 分类标准。
