Licciardi Francesco, Covizzi Carlotta, Dellepiane Marta, Olivini Nicole, Mastrolia Maria Vincenza, Lo Vecchio Andrea, Monno Viviana, Tardi Maria, Mauro Angela, Alessio Maria, Filocamo Giovanni, Cattalini Marco, Taddio Andrea, Caorsi Roberta, Marseglia Gian Luigi, La Torre Francesco, Campana Andrea, Simonini Gabriele, Ravelli Angelo, Montin Davide
Division of Pediatric Immunology and Rheumatology, Department of Public Health and Pediatrics, "Regina Margherita" Children Hospital, University of Turin, Turin, Italy.
Pediatrics Unit, University Department of Pediatrics (DEAPG), Bambino Gesù Children's Hospital-IRCCS, Rome, Italy.
Front Pediatr. 2023 Aug 22;11:1137051. doi: 10.3389/fped.2023.1137051. eCollection 2023.
The treatment of multisystem inflammatory syndrome in children unresponsive to first-line therapies (IVIG and/or steroids) is challenging. The effectiveness of IL-1 receptor antagonist, anakinra, is debated.
We conducted an anonymous retrospective multicenter study on MIS-C patients treated with anakinra in Italy from January 2020 to February 2021. Our study outcomes included the percentage of patients who required further therapeutic step-up, the percentage of patients who experienced fever resolution within 24 h and a reduction of CRP by half within 48 h, and the percentage of patients who developed Coronary Artery Anomalies (CAA) during follow-up.
35 cases of MIS-C were treated in 10 hospitals. Of these, 13 patients started anakinra while in the ICU, and 22 patients started anakinra in other wards. 25 patients (71.4%) were treated with corticosteroids at a starting dose 2-30 mg/Kg/day plus IVIG (2 g/Kg), 10 patients (28.6%) received only corticosteroids without IVIG. Anakinra was administered intravenously to all patients in Group A (mean dose 8 mg/Kg/day), and subcutaneously in Group B (mean dose 4 mg/Kg/day). Only two patients required further treatment step-up and no patients developed CAA after receiving anakinra. The most commonly observed side effect was an increase in ALT, occurring in 17.1% of patients.
In this retrospective cohort of severe MIS-C patients treated with anakinra we report favorable clinical outcomes with a low incidence of side effects. The simultaneous use of steroids ± IVIG in these patients hinders definitive conclusions regarding the need of IL-1 inhibition in MIS-C treatment.
对一线治疗(静脉注射免疫球蛋白和/或类固醇)无反应的儿童多系统炎症综合征的治疗具有挑战性。白细胞介素-1受体拮抗剂阿那白滞素的有效性存在争议。
我们对2020年1月至2021年2月在意大利接受阿那白滞素治疗的儿童多系统炎症综合征患者进行了一项匿名回顾性多中心研究。我们的研究结果包括需要进一步加强治疗的患者百分比、24小时内发热消退且48小时内C反应蛋白降低一半的患者百分比,以及随访期间发生冠状动脉异常(CAA)的患者百分比。
10家医院共治疗了35例儿童多系统炎症综合征患者。其中,13例患者在重症监护病房开始使用阿那白滞素,22例患者在其他病房开始使用阿那白滞素。25例患者(71.4%)接受了起始剂量为2 - 30mg/Kg/天的皮质类固醇加静脉注射免疫球蛋白(2g/Kg)治疗,10例患者(28.6%)仅接受皮质类固醇治疗,未使用静脉注射免疫球蛋白。A组所有患者均静脉注射阿那白滞素(平均剂量8mg/Kg/天),B组皮下注射(平均剂量4mg/Kg/天)。只有2例患者需要进一步加强治疗,接受阿那白滞素治疗后没有患者发生冠状动脉异常。最常见的副作用是谷丙转氨酶升高,发生在17.1%的患者中。
在这个接受阿那白滞素治疗的重度儿童多系统炎症综合征患者的回顾性队列中,我们报告了良好的临床结果,副作用发生率较低。这些患者同时使用类固醇±静脉注射免疫球蛋白阻碍了关于在儿童多系统炎症综合征治疗中抑制白细胞介素-1必要性的确切结论。