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川崎病和儿童多系统炎症综合征的趋同与发散:来自 COVASAKI 调查的结果。

Convergence and divergence in Kawasaki disease and multisystem inflammatory syndrome in children: results from the COVASAKI survey.

机构信息

Rheumatology Unit, ERN ReCONNET Center, Meyer Children's Hospital IRCCS, Firenze, and NEUROFARBA Department, University of Florence, Italy.

Paediatric Unit, San Donato Hospital, Arezzo, Italy.

出版信息

Clin Exp Rheumatol. 2024 Apr;42(4):931-936. doi: 10.55563/clinexprheumatol/l64q51. Epub 2024 Apr 29.

Abstract

OBJECTIVES

To compare Kawasaki disease (KD) and multisystem inflammatory syndrome (MIS-C) in children.

METHODS

Prospective collection of demographics, clinical and treatment data. Assessment of type 1 interferon (IFN) score, CXCL9, CXCL10, Interleukin (IL)18, IFNγ, IL6, IL1b at disease onset and at recovery.

RESULTS

87 patients (43 KD, 44 MIS-C) were included. Age was higher in MIS-C compared to KD group (mean 31±23 vs. 94±50 months, p<0.001). Extremities abnormalities (p=0.027), mucosal involvement (p<0.001), irritability (p<0.001), gallbladder hydrops (p=0.01) and lymphadenopathy (p=0.07) were more often recorded in KD. Neurological findings (p=0.002), gastrointestinal symptoms (p=0.013), respiratory involvement (p=0.019) and splenomegaly (p=0.026) were more frequently observed in MIS-C. Cardiac manifestations were higher in MIS-C (p<0.001), although coronary aneurisms were more frequent in KD (p=0.012). In the MIS-C group, the multiple linear regression analysis revealed that a higher IFN score at onset was related to myocardial disfunction (p<0.001), lymphadenopathy (p=<0.001) and need of ventilation (p=0.024). Both CXCL9 and CXCL10 were related to myocardial disfunction (p<0.001 and p=0.029). IL18 was positively associated to PICU admission (0.030) and ventilation (p=004) and negatively associated to lymphadenopathy (0.004). IFNγ values were related to neurological involvement and lymphadenopathy (p<0.001), IL1b to hearth involvement (0.006). A negative correlation has been observed between IL6 values, heart involvement (p=0.013) and PICU admission (p<0.001).

CONCLUSIONS

The demographic and clinical differences between KD e MIS-C cohorts confirm previous reported data. The assessment of biomarkers levels at MIS-C onset could be useful to predict a more severe disease course and the development of cardiac complications.

摘要

目的

比较川崎病(KD)和儿童多系统炎症综合征(MIS-C)。

方法

前瞻性收集人口统计学、临床和治疗数据。评估 1 型干扰素(IFN)评分、CXCL9、CXCL10、白细胞介素(IL)18、IFNγ、IL6、IL1b 在发病时和恢复期的水平。

结果

共纳入 87 例患者(43 例 KD,44 例 MIS-C)。与 KD 组相比,MIS-C 组年龄更大(平均 31±23 岁 vs. 94±50 岁,p<0.001)。KD 组更常出现四肢异常(p=0.027)、黏膜受累(p<0.001)、易激惹(p<0.001)、胆囊水肿(p=0.01)和淋巴结病(p=0.07)。而 MIS-C 组更常出现神经系统表现(p=0.002)、胃肠道症状(p=0.013)、呼吸受累(p=0.019)和脾肿大(p=0.026)。尽管 KD 组更常出现冠状动脉瘤(p=0.012),但 MIS-C 组的心脏表现更高(p<0.001)。在 MIS-C 组中,发病时更高的 IFN 评分与心肌功能障碍(p<0.001)、淋巴结病(p=<0.001)和需要通气(p=0.024)相关。CXCL9 和 CXCL10 均与心肌功能障碍相关(p<0.001 和 p=0.029)。IL18 与 PICU 入院(0.030)和通气(p=004)呈正相关,与淋巴结病呈负相关(0.004)。IFNγ 值与神经系统受累和淋巴结病相关(p<0.001),IL1b 与心脏受累相关(0.006)。观察到 IL6 值与心脏受累(p=0.013)和 PICU 入院(p<0.001)呈负相关。

结论

KD 和 MIS-C 队列的人口统计学和临床差异证实了之前的报告数据。在 MIS-C 发病时评估生物标志物水平可能有助于预测更严重的疾病过程和心脏并发症的发生。

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