Abstract：
OBJECTIVE: 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.
METHODS: 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.
RESULTS: 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.
CONCLUSIONS: Anti-IL-1 agents were effective and demonstrated a low adverse event profile in paediatric and adult FMF patients.
METHODS: 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.
RESULTS: 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.
CONCLUSIONS: Anti-IL-1 agents were effective and demonstrated a low adverse event profile in paediatric and adult FMF patients.
摘要：
目的：家族性地中海热（FMF）是最常见的遗传性单基因发热综合征，表现为反复发作的发热和多发性浆膜炎。秋水仙碱是目前推荐的FMF的一线治疗方法。然而,一小部分FMF患者对秋水仙碱无反应或不耐受。抗白细胞介素-1（抗IL-1）药物是秋水仙碱耐药或不耐受的FMF患者的替代治疗选择。本系统综述和荟萃分析旨在为成人和儿童FMF患者抗IL-1药物的疗效和安全性提供定性和定量证据。
方法：MEDLINE，EMBASE,中部,和WebofScience从开始到2023年5月进行了筛选。我们纳入了成人和儿童FMF患者，他们接受了至少一种抗IL-1药物的连续治疗：anakinra，canakinumab,还有rilonacept.主要疗效结果是达到完全缓解的患者比例，主要安全性结果是在治疗期间经历至少一次不良事件的患者比例。对定量合成进行了随机效应荟萃分析。
结果：纳入了由1399例FMF患者组成的44例报告。60%（95%CI：49%，72％）的成人患者和81％（95％CI：72％，89%)的儿科患者达到完全缓解。抗IL-1剂显著降低炎症标记物的水平。在25%(95%CI:13%,37%）的成人患者和12%（95%CI：3%，21%)的儿科患者。
结论：在儿童和成人FMF患者中，抗IL-1药物是有效的，并显示出低不良事件。
方法：MEDLINE，EMBASE,中部,和WebofScience从开始到2023年5月进行了筛选。我们纳入了成人和儿童FMF患者，他们接受了至少一种抗IL-1药物的连续治疗：anakinra，canakinumab,还有rilonacept.主要疗效结果是达到完全缓解的患者比例，主要安全性结果是在治疗期间经历至少一次不良事件的患者比例。对定量合成进行了随机效应荟萃分析。
结果：纳入了由1399例FMF患者组成的44例报告。60%（95%CI：49%，72％）的成人患者和81％（95％CI：72％，89%)的儿科患者达到完全缓解。抗IL-1剂显著降低炎症标记物的水平。在25%(95%CI:13%,37%）的成人患者和12%（95%CI：3%，21%)的儿科患者。
结论：在儿童和成人FMF患者中，抗IL-1药物是有效的，并显示出低不良事件。
