Department of Pediatrics C, Schneider Children's Medical Center of Israel, Petach Tikva, Israel; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
Pediatric Rheumatology Unit, Schneider Children's Medical Center of Israel, Petach Tikva, Israel; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
Semin Arthritis Rheum. 2018 Jun;47(6):897-899. doi: 10.1016/j.semarthrit.2017.10.008. Epub 2017 Oct 12.
This study describes our 5-year experience treating protracted febrile myalgia syndrome (PFMS) with pulsed doses of corticosteroids.
Eight patients with PFMS who received pulse corticosteroid therapy were identified from the electronic database of a tertiary pediatric medical center (2011-2016). Their clinical and laboratory data were collected. Differences in continuous variables between hospital admission and discharge were analyzed using Wilcoxon's matched pairs test.
There were 6 female and 2 male patients of median age 10.45 years (range 6.2-17.1) Six patients were found to be homozygous for the M694V mutation. In 4 patients, PFMS was the first-ever manifestation of familial Mediterranean fever. Pulse corticosteroid therapy was administered at a dose of 10mg/kg for 3 days. Pain was alleviated (visual analog scale score, 0) within hours of initiation of therapy, although pain flare-ups lasting for minutes to hours were still observed during hospitalization. At discharge, all patients were prescribed continuous oral corticosteroids (1-2mg/kg) with gradual tapering down over 6 weeks.
Pulse corticosteroid therapy is effective in alleviating PFMS pain; however, it does not completely abort a PFMS episode.
本研究描述了我们使用脉冲剂量皮质类固醇治疗迁延性发热性肌痛综合征(PFMS)的 5 年经验。
从一家三级儿科医学中心的电子数据库中确定了 8 例接受脉冲皮质类固醇治疗的 PFMS 患者(2011-2016 年)。收集了他们的临床和实验室数据。采用 Wilcoxon 配对检验分析入院和出院时连续变量的差异。
中位年龄为 10.45 岁(范围 6.2-17.1)的 6 名女性和 2 名男性患者,6 名患者为 M694V 突变纯合子。4 例患者的 PFMS 是家族性地中海热的首次表现。脉冲皮质类固醇治疗的剂量为 10mg/kg,持续 3 天。治疗开始后数小时内疼痛得到缓解(视觉模拟评分,0),但在住院期间仍观察到持续数分钟至数小时的疼痛发作。出院时,所有患者均给予连续口服皮质类固醇(1-2mg/kg),在 6 周内逐渐减量。
脉冲皮质类固醇治疗可有效缓解 PFMS 疼痛,但不能完全终止 PFMS 发作。