The Potential Role of Endothelial Cells in the Pathophysiology, Diagnosis, and Management of Acute Kawasaki Disease

The vital role of the endothelium in Kawasaki disease (KD) has been clear for over 20 years. Endothelial cells (ECs) play a critical role in the pathophysiology of the disease, being the target of several molecular pathways resulting in EC damage, dysfunction, and potential detachment or transition to another cell phenotype. The alteration of endothelium health is crucial for the development of coronary artery damage. In children with KD, molecular components or cells are released from injured endothelium and can be measured in peripheral blood samples, suggesting several potential diagnostic and therapeutic implications. Novel diagnostic and therapeutic strategies focusing on ECs and on the pathways that result in endothelial damage have been investigated, but to date, the evidence is not yet strong enough to change clinical practice. Nonetheless, results are promising and provide a reliable basis to guide the design of translational research, as seen with statins, which have confirmed their beneficial effects on vascular integrity in later stages of KD, including in clinical studies. The purpose of this narrative review is to provide a comprehensive overview of the current knowledge on the role of ECs in the pathophysiology, diagnosis, and management of KD, and to synthesize the available evidence to guide further research aimed at confirming the viability of these new strategies.