白细胞介素‑18结合蛋白调控NLRP3炎症小体在川崎病冠状动脉病变中的机制研究进展

许雯; 王诗; 李田华

doi:10.7499/j.issn.1008-8830.2605032

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中国当代儿科杂志 ›› 2026, Vol. 28 ›› Issue (10) : 1312-1317. DOI: 10.7499/j.issn.1008-8830.2605032

综述

白细胞介素‑18结合蛋白调控NLRP3炎症小体在川崎病冠状动脉病变中的机制研究进展

许雯 ¹ ,
王诗 ¹ (综述) ,
李田华 ² (审校)

作者信息 +

Mechanisms by which interleukin-18 binding protein regulates the NLRP3 inflammasome in Kawasaki disease-related coronary artery lesions

Wen XU ¹ ,
Shi WANG ¹ ,
Tian-Hua LI ²

Author information +

文章历史 +

摘要

川崎病（Kawasaki disease, KD）是一种以全身血管炎为特征的急性发热性疾病，冠状动脉病变（coronary artery lesion, CAL）是其最严重的并发症。NOD样受体热蛋白结构域相关蛋白3（NOD‑like receptor family pyrin domain containing 3, NLRP3）炎症小体在KD‑CAL中扮演枢纽角色，其活化后切割并释放成熟的白细胞介素‑18（interleukin‑18, IL‑18）等促炎因子，驱动炎症瀑布反应，IL‑18结合蛋白（IL‑18 binding protein, IL‑18BP）是IL‑18的内源性高亲和力拮抗剂。研究显示，IL‑18/IL‑18BP平衡失调及NLRP3炎症小体异常激活共同参与了KD冠状动脉损伤的发生与发展。该文旨在阐明IL‑18与NLRP3炎症小体在KD‑CAL中的正反馈放大机制，以及IL‑18BP通过阻断该正反馈环路调控NLRP3炎症小体的作用途径，为KD的治疗提供新的理论依据和潜在干预靶点。

Abstract

Kawasaki disease (KD) is an acute febrile illness characterized by systemic vasculitis, with coronary artery lesion (CAL) representing its most serious complication. The NOD-like receptor family pyrin domain containing 3 (NLRP3) inflammasome plays a pivotal role in KD-associated CAL. Upon activation, it cleaves and releases mature interleukin-18 (IL-18) and other proinflammatory cytokines, which propagates downstream inflammatory cascades. IL‑18 binding protein (IL‑18BP) is an endogenous high-affinity antagonist. Studies have shown that dysregulation of the IL‑18/IL‑18BP axis, together with aberrant NLRP3 inflammasome activation, contributes to the initiation and progression of coronary artery injury in KD. This review aims to elucidate the positive feedback amplification between IL-18 and the NLRP3 inflammasome in KD-CAL, and the pathways through which IL-18BP regulates the NLRP3 inflammasome by blocking this positive feedback loop, thereby providing a new theoretical basis and potential interventional targets for the treatment of KD.

导出引用

许雯, 王诗, 李田华. 白细胞介素‑18结合蛋白调控NLRP3炎症小体在川崎病冠状动脉病变中的机制研究进展[J]. 中国当代儿科杂志. 2026, 28(10): 1312-1317 https://doi.org/10.7499/j.issn.1008-8830.2605032

Wen XU, Shi WANG, Tian-Hua LI. Mechanisms by which interleukin-18 binding protein regulates the NLRP3 inflammasome in Kawasaki disease-related coronary artery lesions[J]. Chinese Journal of Contemporary Pediatrics. 2026, 28(10): 1312-1317 https://doi.org/10.7499/j.issn.1008-8830.2605032

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