Department of Paediatric Rheumatology, London, UK.
Infection, Inflammation and Rheumatology Section, London, UK.
Rheumatology (Oxford). 2021 Feb 1;60(2):911-917. doi: 10.1093/rheumatology/keaa652.
The objectives of this study were (i) to describe the clinical presentation, treatment and outcome of paediatric inflammatory multisystem syndrome temporally related to Sars-CoV-2 (PIMS-TS) in children; (ii) to propose a framework to guide multidisciplinary team (MDT) management; and (iii) to highlight the role of the paediatric rheumatologist in this context.
This study involved a retrospective case notes review of patients referred to a single specialist paediatric centre with suspected PIMS-TS, with a focus on clinical presentation, laboratory parameters, treatment, and outcome in the context of an MDT framework.
Nineteen children of median age 9.1 years fulfilled the definition of PIMS-TS and were managed within an MDT framework: 5/19 were female; 14/19 were of Black, Asian or minority ethnicity; 9/19 also fulfilled diagnostic criteria for complete or incomplete Kawasaki disease (KD). Severe systemic inflammation, shock, and abdominal pain were ubiquitous. Treatment was stratified within an MDT framework and included CSs in all; i.v. immunoglobulin in all; anakinra in 4/19; infliximab in 1/19; and antiviral (aciclovir) in 4/19.
We observed significant diagnostic equipoise using a current definition of PIMS-TS, overlapping with KD. Outside of clinical trials, an MDT approach is vital. The role of the paediatric rheumatologist is to consider differential diagnoses of hyperinflammation in the young, to advise on empiric immunomodulatory therapy, to set realistic therapeutic targets, to gauge therapeutic success, to oversee timely step-down of immunomodulation, and to contribute to the longer-term MDT follow-up of any late inflammatory sequelae.
本研究的目的是(i)描述与 SARS-CoV-2 相关的儿童炎症性多系统综合征（PIMS-TS）的临床表现、治疗和结局；(ii)提出一个指导多学科团队（MDT）管理的框架；(iii)强调儿科风湿病学家在这种情况下的作用。
本研究回顾性分析了一家专门的儿科中心疑似 PIMS-TS 患儿的病历，重点关注 MDT 框架下的临床表现、实验室参数、治疗和结局。
19 名年龄中位数为 9.1 岁的儿童符合 PIMS-TS 的定义，并在 MDT 框架内得到管理：5/19 为女性；14/19 为黑人、亚洲人或少数族裔；9/19 还符合完全或不完全川崎病（KD）的诊断标准。严重的全身炎症、休克和腹痛普遍存在。治疗是在 MDT 框架内分层的，包括所有患儿均使用皮质类固醇（CSs）；所有患儿均使用静脉注射免疫球蛋白；4/19 例使用阿那白滞素；1/19 例使用英夫利昔单抗；4/19 例使用抗病毒药物（阿昔洛韦）。
使用当前的 PIMS-TS 定义，我们观察到显著的诊断平衡，与 KD 重叠。在临床试验之外，MDT 方法至关重要。儿科风湿病学家的作用是考虑年轻患者炎症过度活跃的鉴别诊断，建议经验性免疫调节治疗，设定现实的治疗目标，评估治疗效果，监督免疫调节的及时下调，并为任何迟发性炎症后遗症的长期 MDT 随访做出贡献。
