Division of Rheumatology, Department of Pediatrics, Hacettepe Üniversitesi İhsan Doğramacı Çocuk Hastanesi, Çocuk Romatoloji Bölümü, Kat: 3 Sıhhiye, 06100, Ankara, Turkey.
Division of Rheumatology, Department of Pediatrics, Istanbul University Istanbul Faculty of Medicine, Istanbul, Turkey.
BioDrugs. 2023 Nov;37(6):813-828. doi: 10.1007/s40259-023-00613-6. Epub 2023 Jun 29.
Behçet's disease (BD) is a variable vessel vasculitis. Biologic drugs are increasingly used in the treatment of BD. We aimed to analyze biologic drug use in the treatment of pediatric BD.
MEDLINE/PubMed and Scopus databases were searched from the inception of these databases until 15 November 2022, according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Only reports presenting data of pediatric patients with BD (BD diagnosis < 18 years of age) treated with biologic drugs were included. The demographic features, clinical characteristics, and data on treatment were extracted from the included papers.
We included 87 articles including 187 pediatric patients with BD treated with biologic drugs (215 biologic treatments). Tumor necrosis factor (TNF)-α inhibitors (176 treatments) were the most frequently used biologic drugs followed by interferons (21 treatments). Other reported biologic treatments were anti-interleukin-1 agents (n = 11), tocilizumab (n = 4), daclizumab (n = 2), and rituximab (n = 1). The most common indication for biologic drug use was ocular involvement (93 treatments) followed by multisystem active disease (29 treatments). Monoclonal TNF-α inhibitors, adalimumab and infliximab, were preferred over etanercept in ocular and gastrointestinal BD. The improvement rates with any TNF-α inhibitor, adalimumab, infliximab, etanercept, and interferons were 78.5%, 86.1%, 63.4%, 87.5%, and 70%; respectively. The organ-specific improvement rate with TNF-α inhibitors was 76.7% and 70% for ocular and gastrointestinal system involvement. Adverse events have been reported for TNF-α inhibitors, interferons, and rituximab. Six of these were severe [TNF-α inhibitors (n = 4); interferons (n = 2)].
The presented systematic literature search revealed that TNF-α inhibitors followed by interferons were the most frequently used biologic drugs in pediatric BD. Both group of biologic treatments appeared to be effective and have an acceptable safety profile in pediatric BD. However, controlled studies are required for analyzing indications for biologic treatments in pediatric BD.
贝切特病（BD）是一种可变血管炎。生物制剂在 BD 的治疗中越来越多地被应用。我们旨在分析生物制剂在儿科 BD 治疗中的应用。
根据系统评价和荟萃分析的首选报告项目（PRISMA）指南，从这些数据库的创建到 2022 年 11 月 15 日，检索 MEDLINE/PubMed 和 Scopus 数据库。仅纳入报告了接受生物制剂治疗的儿科 BD 患者（BD 诊断年龄<18 岁）数据的研究。从纳入的文献中提取人口统计学特征、临床特征和治疗数据。
我们纳入了 87 篇文章，共包括 187 例接受生物制剂治疗的儿科 BD 患者（215 例生物制剂治疗）。肿瘤坏死因子（TNF）-α 抑制剂（176 种治疗）是最常用的生物制剂，其次是干扰素（21 种治疗）。其他报告的生物治疗包括抗白细胞介素-1 制剂（n=11）、托珠单抗（n=4）、达利珠单抗（n=2）和利妥昔单抗（n=1）。生物制剂应用最常见的适应证是眼部受累（93 种治疗），其次是多系统活动疾病（29 种治疗）。在眼部和胃肠道 BD 中，单克隆 TNF-α 抑制剂，阿达木单抗和英夫利昔单抗，优于依那西普。任何 TNF-α 抑制剂、阿达木单抗、英夫利昔单抗、依那西普和干扰素的改善率分别为 78.5%、86.1%、63.4%、87.5%和 70%。TNF-α 抑制剂对眼部和胃肠道系统受累的器官特异性改善率分别为 76.7%和 70%。TNF-α 抑制剂、干扰素和利妥昔单抗均有不良反应报告。其中 6 例为严重不良反应（TNF-α 抑制剂[n=4]；干扰素[n=2]）。
本系统文献检索显示，TNF-α 抑制剂其次是干扰素是儿科 BD 中最常用的生物制剂。两组生物制剂治疗在儿科 BD 中均显示出有效性和可接受的安全性。然而，需要进行对照研究来分析儿科 BD 中生物制剂治疗的适应证。
