Potential roles of NLRP3 inflammasome in the pathogenesis of Kawasaki disease

• Published in: Journal of cellular physiology Vol. 238; no. 3; pp. 513 - 532
• Main Authors: Shahi, Abbas, Afzali, Shima, Firoozi, Zahra, Mohaghegh, Poopak, Moravej, Ali, Hosseinipour, Ali, Bahmanyar, Maryam, Mansoori, Yaser
• Format: Journal Article
• Language: English
• Published: United States Wiley Subscription Services, Inc 01.03.2023
• Subjects: Apoptosis; Arteries; Blood flow; Blood vessels; Caspase; Cell death; Endothelial cells; Etiology; Humans; Inflammasomes; Inflammasomes - metabolism; Inflammation; interleukin‐1β; Ischemia; Kawasaki disease; Mucocutaneous lymph node syndrome; Mucocutaneous Lymph Node Syndrome - etiology; Mucocutaneous Lymph Node Syndrome - genetics; Mucocutaneous Lymph Node Syndrome - pathology; Mucocutaneous Lymph Node Syndrome - physiopathology; NLR Family, Pyrin Domain-Containing 3 Protein - metabolism; NLRP3; Pathogenesis; Proteins; Pyroptosis; Systemic vasculitis; pyroptosis; NLRP3; Kawasaki disease; inflammasomes; inflammation; interleukin-1β
• ISSN: 0021-9541; 1097-4652
• DOI: 10.1002/jcp.30948

There is a heterogeneous group of rare illnesses that fall into the vasculitis category and are characterized mostly by blood vessel inflammation. Ischemia and disrupted blood flow will cause harm to the organs whose blood arteries become inflamed. Kawasaki disease (KD) is the most prevalent kind of vasculitis in children aged 5 years or younger. Because KD's cardiovascular problems might persist into adulthood, it is no longer thought of as a self‐limiting disease. KD is a systemic vasculitis with unknown initiating factors. Numerous factors, such as genetic predisposition and infectious pathogens, are implicated in the etiology of KD. As endothelial cell damage and inflammation can lead to coronary endothelial dysfunction in KD, some studies hypothesized the crucial role of pyroptosis in the pathogenesis of KD. Additionally, pyroptosis‐related proteins like caspase‐1, apoptosis‐associated speck‐like protein containing a CARD (ASC), proinflammatory cytokines like IL‐1 and IL‐18, lactic dehydrogenase, and Gasdermin D (GSDMD) have been found to be overexpressed in KD patients when compared to healthy controls. These occurrences may point to an involvement of inflammasomes and pyroptotic cell death in the etiology of KD and suggest potential treatment targets. Based on these shreds of evidence, in this review, we aim to focus on one of the well‐defined inflammasomes, NLRP3, and its role in the pathophysiology of KD.