目的:斯蒂尔病是一种罕见的自身炎症性疾病,以全身性炎症为特征,发烧,皮疹,和关节炎。术语“斯蒂尔病”涵盖小儿亚型系统性幼年特发性关节炎(sJIA)和成人发作斯蒂尔病(AOSD),影响成年人。生物药物,包括抗白细胞介素-1药物阿纳金拉,canakinumab,rilonacept,白细胞介素-6拮抗剂托珠单抗,用于斯蒂尔病的管理。
方法:我们对随机对照试验进行了系统评价和网络荟萃分析,研究方案在PROSPERO(CRD42023450442)中注册。MEDLINE,EMBASE,和CENTRAL从开始到2023年9月17日进行筛查。我们纳入了接受安慰剂或生物药物的斯蒂尔氏病患者:anakinra,canakinumab,rilonacept,或者托珠单抗.主要疗效和安全性结果是ACR50反应和严重不良事件的发生。分别。使用rankographs和SUCRA值对干预措施进行排名。
结果:纳入了430例患者的9项试验。与安慰剂相比,所有生物药物的ACR50反应几率更高。生物药物与严重不良事件之间没有统计学上的显著关联。多变量荟萃分析发现生物药物之间没有差异。根据SUCRA排名,就ACR50应答和严重不良事件的发生而言,anakinra是最有效和安全的选择.
结论:这是首次系统评价和荟萃分析,以评估生物药物在儿童和成人Still病患者中的疗效和安全性。生物药物在实现ACR反应方面是有效的,并且在斯蒂尔病的治疗中表现出了较低的不良事件。
OBJECTIVE: Still\'s disease is a rare autoinflammatory disorder characterized by systemic inflammation, fever, rash, and arthritis. The term \"Still\'s disease\" covers the pediatric subtype systemic Juvenile Idiopathic Arthritis (sJIA) and adult-onset Still\'s disease (AOSD), which affects adults. Biological drugs, including anti-interleukin-1 agents anakinra,
canakinumab, rilonacept, and the interleukin-6 antagonist tocilizumab, are used in the management of Still\'s disease.
METHODS: We conducted a systematic
review and network meta-analysis of randomized controlled trials, and the study protocol was registered in PROSPERO (CRD42023450442). MEDLINE, EMBASE, and CENTRAL were screened from inception until September 17, 2023. We included patients with Still\'s disease who received placebo or biological drugs: anakinra,
canakinumab, rilonacept, or tocilizumab. The primary efficacy and safety outcomes were achievement of ACR50 response and occurrence of serious adverse events, respectively. The interventions were ranked using rankograms and SUCRA values.
RESULTS: Nine trials with 430 patients were included. All biological drugs were associated with greater odds of ACR50 response compared with placebo. There was no statistically significant association between biological drugs and serious adverse events. The multivariate meta-analysis found no difference between biological drugs. As per SUCRA rankings, anakinra was the most effective and safe option with respect to ACR50 response and occurrence of serious adverse events.
CONCLUSIONS: This is the first systematic
review and meta-analysis to assess the efficacy and safety of biological drugs in pediatric and adult patients with Still\'s disease. Biological drugs were effective in achieving ACR response and demonstrated a low adverse event profile in the management of Still\'s disease.