Department of Internal Medicine, Cerrahpasa Medical Faculty, Istanbul University-Cerrahpasa, Istanbul, Turkey.
Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical Faculty, Istanbul University-Cerrahpasa, Istanbul, Turkey.
Rheumatology (Oxford). 2024 Apr 2;63(4):925-935. doi: 10.1093/rheumatology/kead514.
FMF is the most common hereditary monogenic fever syndrome marked by recurrent attacks of fever and polyserositis. Colchicine is the current recommended first-line treatment for FMF. However, a small portion of FMF patients are unresponsive or intolerant to colchicine. Anti-IL-1 agents are alternative treatment options for colchicine-resistant or -intolerant FMF patients. This systematic review and meta-analysis aimed to provide qualitative and quantitative evidence for the efficacy and safety of anti-IL-1 agents in adult and paediatric FMF patients.
MEDLINE, EMBASE, CENTRAL and Web of Science were screened from inception to May 2023. We included adult and paediatric FMF patients who received continuous treatment with at least one of the anti-IL-1 drugs: anakinra, canakinumab and rilonacept. The primary efficacy outcome was the proportion of patients who achieved complete remission of attacks and the primary safety outcome was the proportion of patients who experienced at least one adverse event during treatment. A random-effects meta-analysis was performed for the quantitative synthesis.
Fourty-four reports consisting of 1399 FMF patients were included. Sixty percent (95% CI 49%, 72%) of the adult patients and 81% (95% CI 72%, 89%) of the paediatric patients achieved complete remission. Anti-IL-1 agents significantly decreased levels of inflammatory markers. At least one adverse event was observed in 25% (95% CI 13%, 37%) of the adult patients and 12% (95% CI 3%, 21%) of the paediatric patients.
Anti-IL-1 agents were effective and demonstrated a low adverse event profile in paediatric and adult FMF patients.
FMF 是最常见的遗传性单基因发热综合征，其特征为反复发作的发热和多浆膜炎。秋水仙碱是目前推荐的 FMF 一线治疗药物。然而，一小部分 FMF 患者对秋水仙碱无反应或不耐受。抗 IL-1 药物是对秋水仙碱耐药或不耐受的 FMF 患者的替代治疗选择。本系统评价和荟萃分析旨在为抗 IL-1 药物在成人和儿科 FMF 患者中的疗效和安全性提供定性和定量证据。
从建库到 2023 年 5 月，我们对 MEDLINE、EMBASE、CENTRAL 和 Web of Science 进行了筛选。我们纳入了接受至少一种抗 IL-1 药物（阿那白滞素、卡那单抗和利纳西普）连续治疗的成人和儿科 FMF 患者。主要疗效结局是达到完全缓解发作的患者比例，主要安全性结局是治疗期间至少发生 1 次不良事件的患者比例。采用随机效应荟萃分析进行定量综合分析。
共纳入 44 项研究，包含 1399 例 FMF 患者。60%（95%CI 49%，72%）的成年患者和 81%（95%CI 72%，89%）的儿科患者达到完全缓解。抗 IL-1 药物显著降低了炎症标志物水平。25%（95%CI 13%，37%）的成年患者和 12%（95%CI 3%，21%）的儿科患者观察到至少 1 次不良事件。
抗 IL-1 药物在儿科和成年 FMF 患者中有效，且具有较低的不良事件谱。
