Division of Rheumatology, Children's National Hospital, Washington, DC, USA.
Rheumatology Fellowship and Training Branch, The National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, MD, USA.
Pediatr Rheumatol Online J. 2023 Jul 31;21(1):76. doi: 10.1186/s12969-023-00858-z.
Evidence for the treatment of multisystem inflammatory syndrome in children (MIS-C) is lacking. Anakinra, which targets IL-1-mediated inflammation, is reserved for refractory cases of MIS-C; however, its use in the treatment of MIS-C is not clearly established.
To examine a role for anakinra in MIS-C, we performed a single center observational cohort study of all MIS-C patients diagnosed at our children's hospital from May 15 to November 15, 2020. Demographics, clinical features, diagnostic testing, and cardiac function parameters were compared between MIS-C patients treated with intravenous immunoglobulin (IVIG) monotherapy and IVIG with anakinra (IVIG + anakinra).
Among 46 patients with confirmed MIS-C, 32 (70%) were in the IVIG + anakinra group, of which 9 (28%) were also given corticosteroids (CS). No patients were treated with anakinra alone. MIS-C patients in the IVIG + anakinra group were enriched in a CV shock phenotype (p = 0.02), and those with CV shock were treated with higher doses of anakinra for a longer duration. Furthermore, MIS-C patients in the IVIG + anakinra group exhibited improvements in fever and cardiac function with or without CS. No significant adverse events were observed, and no differences in IL-1β levels were found among MIS-C patients in the IVIG + anakinra group.
Anakinra treatment, which was co-administered with IVIG primarily in patients with severe MIS-C, was associated with improvements in fever and cardiac function, and demonstrated a favorable side-effect profile. These findings suggest a role for adjunctive anakinra in the treatment of severe MIS-C.
目前针对儿童多系统炎症综合征(MIS-C)的治疗方法证据有限。阿那白滞素是一种针对白细胞介素-1(IL-1)介导的炎症反应的药物,仅用于治疗难治性 MIS-C 病例;然而,其在 MIS-C 治疗中的应用尚未明确。
为了研究阿那白滞素在 MIS-C 中的作用,我们对 2020 年 5 月 15 日至 11 月 15 日在我院确诊的所有 MIS-C 患者进行了一项单中心观察性队列研究。比较了接受静脉注射免疫球蛋白(IVIG)单药治疗和 IVIG 联合阿那白滞素(IVIG+阿那白滞素)治疗的 MIS-C 患者的人口统计学特征、临床特征、诊断检测和心脏功能参数。
在 46 例确诊为 MIS-C 的患者中,32 例(70%)接受了 IVIG+阿那白滞素治疗,其中 9 例(28%)还接受了皮质类固醇(CS)治疗。没有患者单独接受阿那白滞素治疗。IVIG+阿那白滞素治疗组的 MIS-C 患者中,心血管休克表型更为丰富(p=0.02),且存在心血管休克的患者接受了更高剂量和更长时间的阿那白滞素治疗。此外,IVIG+阿那白滞素治疗组的 MIS-C 患者在接受或不接受 CS 治疗后,发热和心脏功能均有所改善。未观察到明显的不良反应,且 IVIG+阿那白滞素治疗组的 MIS-C 患者的白细胞介素-1β水平无差异。
阿那白滞素与 IVIG 联合治疗主要用于重症 MIS-C 患者,可改善发热和心脏功能,并具有良好的副作用特征。这些发现提示阿那白滞素在重症 MIS-C 治疗中的作用。