Department of Pediatric Pulmonology, Infectious Diseases and Immunology, Ghent University Hospital, Ghent, Belgium.
Primary Immunodeficiency Research Lab, Center for Primary Immunodeficiency Ghent, Jeffrey Modell Diagnosis and Research Center, Ghent University Hospital, Ghent, Belgium.
Eur J Pediatr. 2021 Jul;180(7):2019-2034. doi: 10.1007/s00431-021-03993-5. Epub 2021 Feb 18.
An association between a novel pediatric hyperinflammatory condition and SARS-CoV-2 was recently published and termed pediatric inflammatory multisystem syndrome, temporally associated with SARS-CoV-2 (PIMS-TS) or multisystem inflammatory syndrome (in children) (MIS(-C)). We performed a systematic review and describe the epidemiological, clinical, and prognostic characteristics of 953 PIMS-TS/MIS(-C) cases in 68 records. Additionally, we studied the sensitivity of different case definitions that are currently applied. PIMS-TS/MIS(-C) presents at a median age of 8 years. Epidemiological enrichment for males (58.9%) and ethnic minorities (37.0% Black) is present. Apart from obesity (25.3%), comorbidities are rare. PIMS-TS/MIS(-C) is characterized by fever (99.4%), gastrointestinal (85.6%) and cardiocirculatory manifestations (79.3%), and increased inflammatory biomarkers. Nevertheless, 50.3% present respiratory symptoms as well. Over half of patients (56.3%) present with shock. The majority of the patients (73.3%) need intensive care treatment, including extracorporal membrane oxygenation (ECMO) in 3.8%. Despite severe disease, mortality is rather low (1.9%). Of the currently used case definitions, the WHO definition is preferred, as it is more precise, while encompassing most cases.Conclusion: PIMS-TS/MIS(-C) is a severe, heterogeneous disease with epidemiological enrichment for males, adolescents, and racial and ethnic minorities. However, mortality rate is low and short-term outcome favorable. Long-term follow-up of chronic complications and additional clinical research to elucidate the underlying pathogenesis is crucial. What is Known: • A novel pediatric inflammatory syndrome with multisystem involvement has been described in association with SARS-CoV-2. • To date, the scattered reporting of cases and use of different case definitions provides insufficient insight in the full clinical spectrum, epidemiological and immunological features, and prognosis. What is New: • This systematic review illustrates the heterogeneous spectrum of PIMS-TS/MIS(-C) and its epidemiological enrichment for males, adolescents, and racial and ethnic minorities. • Despite its severe presentation, overall short-term outcome is good. • The WHO MIS definition is preferred, as it is more precise, while encompassing most cases.
一种新型儿科高炎症状态与 SARS-CoV-2 相关，最近已被发表，并被命名为 SARS-CoV-2 相关儿童多系统炎症综合征（pediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2，PIMS-TS）或儿童多系统炎症综合征（multisystem inflammatory syndrome in children，MIS-C）。我们进行了一项系统性回顾，共描述了 68 项研究中的 953 例 PIMS-TS/MIS-C 病例的流行病学、临床和预后特征。此外，我们还研究了目前应用的不同病例定义的敏感性。PIMS-TS/MIS-C 的中位发病年龄为 8 岁，男性（58.9%）和少数民族（37.0%为黑人）的发病率较高。除肥胖（25.3%）外，合并症罕见。PIMS-TS/MIS-C 的特征是发热（99.4%）、胃肠道（85.6%）和心血管表现（79.3%）以及炎症标志物升高。然而，50.3%的患者也有呼吸道症状。超过一半的患者（56.3%）出现休克。大多数患者（73.3%）需要重症监护治疗，包括体外膜肺氧合（ECMO），占 3.8%。尽管疾病严重，但死亡率相当低（1.9%）。在目前使用的病例定义中，世界卫生组织（WHO）的定义更为准确，同时也包含了大多数病例。结论：PIMS-TS/MIS-C 是一种严重的、异质性疾病，男性、青少年以及种族和民族少数民族发病率较高。然而，死亡率较低，短期预后较好。长期随访慢性并发症和进一步的临床研究，以阐明潜在的发病机制至关重要。已知：• 一种新型与 SARS-CoV-2 相关的儿科炎症综合征伴有多系统受累已被描述。• 迄今为止，病例的分散报告和不同病例定义的使用提供了对全面临床谱、流行病学和免疫学特征以及预后的了解不足。新发现：• 这项系统性综述说明了 PIMS-TS/MIS-C 的异质性谱及其在男性、青少年以及种族和民族少数民族中的流行病学富集。• 尽管其表现严重，但总体短期预后良好。• 世界卫生组织（WHO）的 MIS 定义更为准确，同时也包含了大多数病例，因此更为优选。
