Department of Pediatrics, Division of Rheumatology, Faculty of Medicine, Hacettepe University, 06100, Ankara, Turkey.
Clin Rheumatol. 2018 Dec;37(12):3329-3335. doi: 10.1007/s10067-018-4095-1. Epub 2018 Apr 16.
Our aim was to report our experiences of pediatric macrophage activation syndrome (MAS) patients treated with anakinra and to review previous studies reporting anakinra treatment in pediatric MAS patients associated with systemic juvenile idiopathic arthritis (sJIA) or autoinflammatory diseases (AIDs). The study group consisted of pediatric MAS patients due to sJIA or AIDs, followed up in the Pediatric Rheumatology Unit of Hacettepe University between January 2015 and January 2017 and treated with anakinra (anti-IL1). We conducted a systematic review of the published literature involving pediatric MAS patients associated with sJIA or AIDs, treated with anakinra. Thirteen sJIA patients and two AIDs patients were included the study. Nineteen MAS episodes were observed in 15 patients. Anakinra (2 mg/kg/day) was started in with a median 1 day after admission. Clinical symptoms resolved, and laboratory findings normalized within median (minimum-maximum) 2 (1-4) and 6 (4-9) days, respectively after the introduction of anakinra. Steroid treatment was stopped in a median of 10 (4-13) weeks after the initiation of anakinra treatment. Patients were followed up for a median of 13 (6-24) months. Two patients developed recurrent MAS episodes when the anakinra dose was reduced, while the other patients achieved remission. In the literature review, we identified nine articles, describing 35 pediatric MAS patients associated with sJIA or AIDs and treated with anakinra. Except for two, all the patients reached remission. Our study and systematic literature review may help to improve the knowledge on the role of anakinra treatment in the management of MAS.
我们的目的是报告我们用阿那白滞素治疗儿科巨噬细胞活化综合征（MAS）患者的经验，并回顾以前报道的用阿那白滞素治疗与全身幼年特发性关节炎（sJIA）或自身炎症性疾病（AIDs）相关的儿科 MAS 患者的研究。研究组由因 sJIA 或 AIDs 而接受阿那白滞素（抗 IL1）治疗的儿科 MAS 患者组成，在哈塞泰佩大学儿科风湿病科于 2015 年 1 月至 2017 年 1 月期间接受随访。我们对涉及用阿那白滞素治疗与 sJIA 或 AIDs 相关的儿科 MAS 患者的已发表文献进行了系统回顾。13 例 sJIA 患者和 2 例 AIDs 患者纳入研究。15 例患者共观察到 19 次 MAS 发作。阿那白滞素（2mg/kg/天）于入院后中位数 1 天开始使用。在引入阿那白滞素后，中位数（最小-最大）分别为 2（1-4）天和 6（4-9）天，临床症状得到缓解，实验室检查结果恢复正常。阿那白滞素治疗开始后，中位数 10（4-13）周停用类固醇。中位数随访 13（6-24）个月。2 例患者在减少阿那白滞素剂量时发生复发性 MAS 发作，而其他患者则达到缓解。在文献复习中，我们确定了 9 篇文章，描述了 35 例与 sJIA 或 AIDs 相关并接受阿那白滞素治疗的儿科 MAS 患者。除了 2 例，所有患者都达到了缓解。我们的研究和系统文献回顾可能有助于提高对阿那白滞素治疗在 MAS 管理中的作用的认识。
