Abstract

Objectives

Behcet Disease (BD) is a systemic vasculitis, first described with a triad of oral aphthous ulcers, genital ulcers, and uveitis. The authors aimed to share the clinical properties and utilities of three distinct classification criteria for BD in this study.

Methods

This case-control study was conducted in pediatric BD patients, diagnosed between January 2012 and July 2019. The control group included 53 children with other rheumatic disorders. Sensitivity and specificity for International Study Group(ISG), International Criteria for BD(ICBD), and pediatric criteria for BD(PEDBD) criteria were tested.

Results

The mean age at symptom onset and diagnosis of the 16 BD patients (6 females, 10 males) were 11.2±3.6 and 13±3.1 years, respectively. The most frequent symptoms were oral aphthous(93.8%), joint involvement(75%), genital ulcers(50%), erythema nodosum(50%) and uveitis(37.5%) among BD patients. The sensitivity and specificity of ISG criteria were 87.5% and 100%. Furthermore, ICBD criteria had a sensitivity and specificity of 93.7% and 98.1%, whereas the authors found sensitivity and specificity as 93.7% and 96.2% for PEDBD.

Conclusion

ICBD and PEDBD reached higher sensitivity for pediatric BD diagnosis and ICBD had the highest specificity. The authors speculate that the utilization of ICBD may provide early diagnosis of BD in childhood, prevent related morbidities and misdiagnosis.

中文翻译：

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白塞病儿童不同分类标准的评价：来自单一转诊中心的结果

摘要

目标

白塞病 (BD) 是一种全身性血管炎，首先被描述为具有口腔阿弗他溃疡、生殖器溃疡和葡萄膜炎的三联征。作者旨在分享本研究中三种不同的 BD 分类标准的临床特性和效用。

方法

该病例对照研究是在 2012 年 1 月至 2019 年 7 月间诊断出的儿童 BD 患者中进行的。对照组包括 53 名患有其他风湿性疾病的儿童。测试了国际研究组 (ISG)、国际 BD 标准 (ICBD) 和儿科 BD 标准 (PEDBD) 的敏感性和特异性。

结果

16 名 BD 患者（6 名女性，10 名男性）在症状出现和诊断时的平均年龄分别为 11.2±3.6 和 13±3.1 岁。BD患者最常见的症状是口腔口疮（93.8%）、关节受累（75%）、生殖器溃疡（50%）、结节性红斑（50%）和葡萄膜炎（37.5%）。ISG 标准的敏感性和特异性分别为 87.5% 和 100%。此外，ICBD 标准的敏感性和特异性分别为 93.7% 和 98.1%，而作者发现 PEDBD 的敏感性和特异性分别为 93.7% 和 96.2%。

结论

ICBD 和 PEDBD 对儿科 BD 诊断的敏感性更高，ICBD 的特异性最高。作者推测，利用 ICBD 可能会在儿童期提供 BD 的早期诊断，防止相关的发病率和误诊。