Brisca Giacomo, Consolaro Alessandro, Caorsi Roberta, Pirlo Daniela, Tuo Giulia, Campanello Claudia, Castagnola Elio, Moscatelli Andrea, Gattorno Marco, Ravelli Angelo
Terapia Semintensiva, IRCCS Istituto Giannina Gaslini, Genoa, Italy.
Clinica Pediatrica e Reumatologia, IRCCS Istituto Giannina Gaslini, Genoa, Italy.
Front Pediatr. 2021 Dec 20;9:783745. doi: 10.3389/fped.2021.783745. eCollection 2021.
In this observational study, we report the clinical, therapeutics and outcome features of 23 patients with multisystem inflammatory syndrome (MIS-C) who have been treated in Gaslini Children Hospital (Genoa, Italy) with a multistep antinflammatory treatment protocol, based on disease severity at admission. Patients were initially assigned to four severity classes on admission and treated accordingly. The therapeutic options ranged from IV immunoglobulin alone to a combination of IVIG plus pulses of methylprednisolone plus anakinra for patients with marked cardiac function impairment or signs of macrophage activation syndrome, with rapid treatment escalation in case of inadequate therapeutic response. With the application of this therapeutic strategy, no patient required admission to Intensive Care Unit (ICU) or invasive mechanical ventilation, and no inotropic drugs administration was required. Early aggressive treatment of MIS-C, with therapeutic interventions modulated based on the severity of clinical manifestations may help to prevent the progression of the inflammatory process and to avoid the need of admission to the ICU. A timely intervention with anti-IL-1 blockers can play a pivotal role in very severe patients that are at risk to have an incomplete response to immunoglobulins and steroids.
在这项观察性研究中,我们报告了23例多系统炎症综合征(MIS-C)患者的临床、治疗及预后特征。这些患者在意大利热那亚的加斯利尼儿童医院接受了基于入院时疾病严重程度的多步骤抗炎治疗方案。患者入院时最初被分为四个严重程度等级,并据此进行治疗。治疗选择范围从单独使用静脉注射免疫球蛋白到静脉注射免疫球蛋白加甲基强的松龙脉冲治疗加纳基单抗联合使用,用于有明显心功能损害或巨噬细胞活化综合征迹象的患者,若治疗反应不足则迅速升级治疗。通过应用这种治疗策略,没有患者需要入住重症监护病房(ICU)或进行有创机械通气,也无需使用强心药物。对MIS-C进行早期积极治疗,根据临床表现的严重程度调整治疗干预措施,可能有助于预防炎症过程的进展并避免入住ICU的必要性。对于有对免疫球蛋白和类固醇反应不完全风险的非常严重的患者,及时使用抗IL-1阻滞剂可发挥关键作用。