Department of Medicine, School of Medicine, Nazarbayev University, Kerei-Zhanibek Str. 5/1, Astana, 010000, Kazakhstan.
Department of Pediatric Rheumatology, National Research Center of Mother and Child Health, University Medical Center, Astana, Kazakhstan.
Rheumatol Int. 2019 Jun;39(6):1107-1112. doi: 10.1007/s00296-019-04300-0. Epub 2019 Apr 11.
Behçet's disease (BD) is a systemic vasculitis affecting prominently the veins, which is usually diagnosed in adulthood, but can occur in children younger than 16 years in about 4-26% of cases. The therapy is based on several immune-suppressive drugs; in case of inadequate control and/or complications, the biologic therapy with anti-TNF drugs has been successfully used in adults. Here, we reported one pediatric case of BD with systemic (persistent/recurrent high fever), skin and mucosal manifestations (recurrent aphthous stomatitis, anal/penile ulcers, erythema nodosum and papulo-pustules), that were unresponsive to the conventional treatment with steroids and colchicine; however, he was successfully treated with adalimumab. Compared to adult patients, the experience with adalimumab in the treatment of pediatric BD is very limited. Indeed, through a systematic search in the medical literature, we retrieved 4 case reports and 2 case series, describing BD pediatric patients treated with adalimumab, in addition to three clinical studies including some BD children. The analysis and discussion of these available clinical experiences may indicate adalimumab as an effective and safe option to treat several forms of BD, in addition to BD-related chronic uveitis.
贝赫切特病（BD）是一种累及静脉为主的系统性血管炎，通常在成年后发病，但在约 4-26%的病例中，儿童在 16 岁以下也可能发病。治疗基于多种免疫抑制药物；在控制不佳和/或出现并发症的情况下，抗 TNF 药物的生物治疗已在成人中成功应用。在此，我们报告了一例儿科贝赫切特病病例，其表现为全身（持续性/复发性高热）、皮肤和黏膜（复发性口腔溃疡、肛门/阴茎溃疡、结节性红斑和丘疹脓疱）表现，对常规治疗（皮质类固醇和秋水仙碱）无效；然而，他成功地接受了阿达木单抗治疗。与成年患者相比，阿达木单抗在儿科贝赫切特病治疗中的经验非常有限。实际上，通过对医学文献进行系统检索，我们共检索到 4 例病例报告和 2 例病例系列，描述了接受阿达木单抗治疗的儿科贝赫切特病患者，此外还有 3 项包括一些贝赫切特病儿童的临床研究。对这些现有临床经验的分析和讨论表明，阿达木单抗可能是治疗多种形式贝赫切特病（除了与贝赫切特病相关的慢性葡萄膜炎）的有效和安全选择。
