目的:这项研究的目的是建立一个国际多中心注册中心,以收集儿童多系统炎症综合征(MIS-C)患者的数据,为了突出临床表现之间的关系,发病年龄和地理分布对临床结局的影响。
方法:多中心回顾性研究,涉及不同的国际罕见免疫疾病协会。我们收集了2022年3月至9月间从48个中心和22个国家诊断为MIS-C的1009例患者。五个年龄组(<1,1-4,5-11,12-16,>16岁)和四个地理宏观区域,西欧,中东欧,拉丁美洲,亚非资源有限国家(LRC),已确定。
结果:LRC的转诊时间明显延长。强化抗炎治疗,包括生物制品,呼吸支持和机械通气在年龄较大的儿童和欧洲国家更常用.幼儿(<1岁)的死亡率较高,老年患者(>16岁)和LRC。多变量分析确定了LRC的住宅,存在严重的心脏受累,肾性高血压,淋巴细胞减少和不使用肝素预防,作为与不利结果最密切相关的因素。
结论:按年龄和地理宏观区域对患者进行分层提供了对临床表现的见解,MIS-C的治疗和结果死亡率和后遗症率与年龄和地理区域相关。在LRC入院和治疗的患者表现出更严重的结果,可能是由于住院延误以及获得生物药物和重症监护设施的机会有限。
OBJECTIVE: The aim of the study was to establish an international multicenter registry to collect data on patients with Multisystem Inflammatory Syndrome in Children (MIS-C), in order to highlight a relationship between clinical presentation, age of onset and geographical distribution on the clinical outcome.
METHODS: Multicenter retrospective study involving different international societies for rare immunological disorders.1009 patients diagnosed with MIS-C between March and September 2022, from 48 centers and 22 countries were collected. Five age groups (<1, 1-4, 5-11, 12-16, >16 years) and four geographic macro-areas, Western Europe, Central-Eastern Europe, Latin America, Asian-African resource-limited countries (LRC), were identified.
RESULTS: Time to referral was significantly higher in LRC. Intensive anti-inflammatory treatment, including biologics, respiratory support and mechanic ventilation were more frequently used in older children and in European countries. The mortality rate was higher in very young children (<1 year), in older patients (>16 years of age) and in LRC. Multivariate analysis identified the residence in LRC, presence of severe cardiac involvement, renal hypertension, lymphopenia and non-use of heparin prophylaxis, as the factors most strongly associated with unfavorable outcomes.
CONCLUSIONS: The stratification of patients by age and geographic macro-area provided insights into the clinical presentation, treatment and outcome of MIS-C. The mortality and sequelae rates exhibited a correlation with the age and geographical areas. Patients admitted and treated in LRC displayed more severe outcomes, possibly due to delays in hospital admission and limited access to biologic drugs and to intensive care facilities.