Kawasaki disease: an ongoing enigma with parallels to COVID-19-related multisystem inflammatory syndrome in children

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Abstract
Kawasaki disease (KD) is a rare pediatric illness of unknown origin that causes high fever, acute inflammation of the blood vessels, and acquired heart conditions. Although multiple aspects of KD remain largely elusive, recent literature has detected an increasing number of KD-like cases among pediatric populations following severe acute respiratory syndrome 2 (SARS-CoV-2) infection, termed “multisystem inflammatory syndrome in children” (MIS-C). While similarities in certain clinical manifestations (fever, mucocutaneous disturbances, cardiac dysfunction, and skin rashes) have caused difficulties in distinguishing the two diseases, key differences in pathophysiology, etiology and epidemiology indicate they are distinct entities triggered by unique infectious agents. The two share commonalities in pathophysiology, characterized by hyperinflammation via cytokine storm. However, KD is notably associated with pathological changes in the coronary arteries, whereas MIS-C presents as a more exaggerated inflammatory syndrome causing myocarditis and ventricular dysfunction. Differences in age range, genetic predispositions, and ethnic distribution of patients has also been identified. Treatment strategies, namely intravenous immunoglobulin therapy (IVIG), have proven effective in both diseases, however MIS-C patients are reportedly less responsive to IVIG and often require additional intervention methods. Distinguishing key characteristics in pathophysiology, etiology, and epidemiology not only helps in understanding differences in clinical manifestations, but also provides valuable insight into potentially unique diagnostic and therapeutic approaches to KD and MIS-C.  
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2024
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