Molecular mechanisms of endothelial dysfunction in Kawasaki-disease-associated vasculitis

Abstract

Kawasaki disease (KD) is an acute, inflammation mediated vasculitis, mainly affecting in children under five, which is consider as the most common coronary artery disease in children. The injuries of coronary arteries would result in dilation or thrombus formation, bringing great threaten to patients. Endothelium, located in the inner surface of coronary artery, serves as the interface between the circulating inflammatory cells and vascular media or adventitia, which is the first target of inflammatory attacks during early stage of KD. A series of studies have determined vascular endothelial cells damages and dysfunction in KD patients. However, current therapeutic strategy is still challenging. So that it is critical to underline the mechanisms of endothelium injuries. In this review, the role of endothelial cells in the pathogenesis of KD and the therapeutic methods for endothelial cells were systematically described.

Human Umbilical Cord Mesenchymal Stem Cells Repair Endothelial Injury and Dysfunction by Regulating NLRP3 to Inhibit Endothelial Cell Pyroptosis in Kawasaki Disease

“…Many studies have shown that KD is an acute systemic vasculitis; however, the pathogenesis is still unclear, and immune disorders [4,39,40] and endothelial dysfunction [9,41] are important mechanisms.…”

The Future of Kawasaki Disease Diagnosis: Liquid Biopsy May Hold the Key

“…Vascular endothelial dysfunction involves activation of apoptotic pathways, proliferation and migration, and is central to KD-associated vasculitis. The molecular mechanisms of vascular EC injury and dysfunction in KDassociated vasculitis have been well reviewed elsewhere [84,85]. These include the role of non-coding RNAs (micro-RNAs, long non-coding RNAs), inflammatory cell activation, cytokine production, reactive oxygen species accumulation and lipid oxidation.…”

The role of mitochondria in the pathogenesis of Kawasaki disease

“…Immune and non-immune cells, including macrophage/monocytes, neutrophils, and endothelial cells, express PRRs and are activated in response to PAMPs and DAMPs ( 46 , 47 ). Endothelial cells are critical for the maintenance of vascular homeostatsis ( 46 ) and play a critical role in KD vasculitis and formation of coronary artery aneurysms ( 2 , 48 , 49 ). Upon activation, they recruit circulating inflammatory cells and increase vascular cell wall permeability ( 50 ).…”