目的:评价Janus激酶抑制剂(JAKi)治疗成人斯蒂尔病(AOSD)的疗效和安全性。
方法:我们搜索了Embase,PubMed,Cochrane中央控制试验登记册(CENTRAL),和中国国家知识基础设施(CNKI)从成立到2023年10月22日。对结果进行了向后搜索相关出版物的补充。两位作者独立选择试验。对现有研究进行了全面回顾和分析。
结果:本综述共纳入9项研究,共35例患者。在这些病人中,17例(48.6%)患者接受了托法替尼治疗,14(40%)与巴利替尼,4(11.4%)与鲁索替尼和1(2.9%)与upadacitinib。用JAKI治疗后,17例(48.6%)患者完全缓解,12例(34.3%)患者部分缓解,7例(20%)患者出现疗效丧失或复发.在患有巨噬细胞活化综合征(MAS)的AOSD患者中,使用ruxolitinib的缓解率为100%。报告的不良事件(AE)的发生率总体上是轻度和罕见的。大多数不良事件是异常的血脂参数(9.7%),细菌性肺炎(3.2%),有组织肺炎(3.2%),腹泻(3.2%),心率增加(3.2%),月经过多(3.2%)和白细胞减少(3.2%)。一名患者死于细菌性肺炎。
结论:JAKI治疗可能是AOSD患者的一种选择,特别是对于耐火AOSD。对于AOSD合并MAS的患者,ruxolitinib似乎是比其他JAKI药物更好的选择。尽管我们的研究表明JAKI在AOSD患者中具有良好的耐受性,我们仍然需要警惕致命的感染。
OBJECTIVE: To evaluate the efficacy and safety of Janus kinases inhibitors (JAKi) for adult-onset Still\'s disease (AOSD) patients.
METHODS: We searched the Embase, PubMed, the Cochrane Central Register of Controlled Trials (CENTRAL), and the China National Knowledge Infrastructure (CNKI) from inception up to 22 October 2023. The results were supplemented by a backward search of relevant publications. Two authors independently selected trials. The available studies were comprehensively reviewed and analysed.
RESULTS: A total of 9 studies with a total of 35 patients were included in the review. Of these patients, 17 (48.6%) patients were treated with tofacitinib, 14 (40%) with baricitinib, 4 (11.4%) with ruxolitinib and 1 (2.9%) with upadacitinib. After treatment with JAKi, 17 (48.6%) patients showed complete remission, 12 (34.3%) patients showed partial remission, and 7 (20%) patients showed loss of efficacy or relapse. The use of ruxolitinib showed a remission rate of 100% in AOSD patients with macrophage activation syndrome (MAS). The incidence of adverse events (AEs) reported were mild and rare overall. Most AEs were abnormal lipid parameters (9.7%), bacterial pneumonia (3.2%), organised pneumonia (3.2%), diarrhoea (3.2%), increased heart rate (3.2%), menometrorrhagia (3.2%) and leukopenia (3.2%). One patient died from bacterial pneumonia.
CONCLUSIONS: JAKi therapy may be an option for patients with AOSD, especially for refractory AOSD. For patients with AOSD complicated by MAS, ruxolitinib seems to be a better choice than other JAKi agents. Although our study shows that JAKi are well tolerated in AOSD patients, we still need to be on the lookout for fatal infections.