Department of Pediatric Emergency, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Turkey.
Department of Pediatric Rheumatology, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Turkey.
Rheumatol Int. 2024 Nov;44(11):2569-2575. doi: 10.1007/s00296-023-05351-0. Epub 2023 Jun 6.
The aim of this retrospective study is to evaluate the efficacy of a single-dose anakinra during familial Mediterranean fever (FMF) attacks and its effect on the duration, severity, and frequency of attacks. The patients with FMF who had disease episode and received a single-dose anakinra during disease episode between December 2020 and May 2022 were included. Demographic characteristics, MEFV gene variants detected, concomitant medical conditions, demographics of recent and previous episodes, laboratory findings and length of hospital stay were recorded. A retrospective analysis of medical records revealed 79 attacks from 68 patients who met inclusion criteria. The patients had a median age of 13 (2.5-25) years. All patients reported that the average duration of their previous episodes lasted longer than 24 h. When the recovery time of attacks after subcutaneous anakinra application at the disease attack was examined, it was observed that 4 attacks (5.1%) ended in 10 min; 10 attacks (12.7%) in 10-30 min; 29 attacks (36.7%) in 30-60 min; 28 attacks (35.4%) in 1-4 h; 4 attacks (5.1%) in 24 h; and 4 attacks (5.1%) ended in more than 24 h. There was no patient who did not recover from their attack after a single dose of anakinra. Although the efficacy of a single-dose anakinra administration during FMF attacks in children needs to be confirmed by prospective studies, our results suggest that use of a single-dose anakinra during FMF attacks is effective in reduction of severity and duration of disease attacks.
本回顾性研究旨在评估单次剂量阿那白滞素在家族性地中海热 (FMF) 发作中的疗效及其对发作持续时间、严重程度和频率的影响。该研究纳入了 2020 年 12 月至 2022 年 5 月期间因疾病发作而接受单次剂量阿那白滞素治疗的 FMF 患者。记录了患者的人口统计学特征、检测到的 MEFV 基因突变、合并症、近期和既往发作的人口统计学特征、实验室检查结果和住院时间。对病历进行回顾性分析,共发现 68 例符合纳入标准的患者发生 79 次发作。患者的中位年龄为 13 岁(2.5-25 岁)。所有患者均报告称,其既往发作的平均持续时间超过 24 小时。当分析皮下注射阿那白滞素后疾病发作的恢复时间时,观察到 4 次发作(5.1%)在 10 分钟内结束;10 次发作(12.7%)在 10-30 分钟内结束;29 次发作(36.7%)在 30-60 分钟内结束;28 次发作(35.4%)在 1-4 小时内结束;4 次发作(5.1%)在 24 小时内结束;4 次发作(5.1%)超过 24 小时结束。单次剂量阿那白滞素治疗后,没有患者的发作未得到缓解。虽然单次剂量阿那白滞素在儿童 FMF 发作中的疗效需要前瞻性研究来证实,但我们的结果表明,在 FMF 发作期间使用单次剂量阿那白滞素可有效减轻疾病发作的严重程度和持续时间。