Paediatrics, Matsuyama Red Cross Hospital, Matsuyama, Japan
Department of Paediatric Acute Diseases, Okayama University Academic Field of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama University Hospital, Okayama, Japan.
BMJ Case Rep. 2022 Jun 9;15(6):e250375. doi: 10.1136/bcr-2022-250375.
Multisystem inflammatory syndrome in children (MIS-C) is a newly described syndrome related to the COVID-19, resembling other known aetiologies, including Kawasaki disease. Cardiovascular involvement is common; left ventricle dysfunction and coronary artery aneurysm (CAA) are also observed. Many treatment guidelines recommend using intravenous immunoglobulin (IVIG) alone or with glucocorticoids as the first-line therapy. Biological agents, such as anakinra, are recommended for refractory cases, but the evidence is still accumulating. Moreover, the use of other treatment agents can be beneficial, especially when anakinra is unavailable. Here, we report the case of a 9-year-old girl who presented with MIS-C with CAAs. She received cyclosporine because two rounds of IVIG treatment were ineffective and the use of anakinra is not approved in Japan. Her cytokine profile showed that cyclosporine prevented exacerbation. The case highlights that cyclosporine therapy can be an option for the treatment of refractory MIS-C with CAA.
儿童多系统炎症综合征(MIS-C)是一种与 COVID-19 相关的新描述综合征,类似于其他已知病因,包括川崎病。心血管受累很常见;也观察到左心室功能障碍和冠状动脉瘤(CAA)。许多治疗指南建议将静脉注射免疫球蛋白(IVIG)单独或与糖皮质激素作为一线治疗。对于难治性病例,建议使用生物制剂,如阿那白滞素,但证据仍在不断积累。此外,其他治疗药物的使用也可能有益,特别是在无法使用阿那白滞素的情况下。在这里,我们报告了一例患有 CAA 的 MIS-C 患儿的病例。她接受了环孢素治疗,因为两轮 IVIG 治疗均无效,而且阿那白滞素在日本尚未获得批准。她的细胞因子谱表明环孢素可预防病情恶化。该病例强调了环孢素治疗可能是治疗难治性伴有 CAA 的 MIS-C 的一种选择。