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WIREs Syst Biol Med
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Can a systems biology approach unlock the mysteries of Kawasaki disease?

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Abstract Kawasaki disease (KD) is a systemic inflammatory illness of childhood that particularly affects the coronary arteries. It can lead to coronary artery aneurysms, myocardial infarction, and sudden death. Clinical and epidemiologic data support an infectious cause, and the etiology remains unknown, but recent data support infection with a ‘new’ virus. Genetic factors influence KD susceptibility; the incidence is 10‐fold higher in children of Asian when compared with Caucasian ethnicity. Recent research has identified genes affecting immune response that are associated with KD susceptibility and outcome. A re‐examination of the pathologic features of KD has yielded a three process model of KD vasculopathy, providing a framework for understanding the KD arterial immune response and the damage it inflicts and for identifying new therapeutic targets for KD patients with coronary artery abnormalities. The researcher is faced with many challenges in determining the pathogenesis of KD. A systems biology approach incorporating genomics, proteomics, transcriptomics, and microbial bioinformatics analysis of high‐throughput sequence data from KD tissues could provide the keys to unlocking the mysteries of this potentially fatal illness of childhood. WIREs Syst Biol Med 2013, 5:221–229. doi: 10.1002/wsbm.1202 This article is categorized under: Physiology > Mammalian Physiology in Health and Disease Translational, Genomic, and Systems Medicine > Translational Medicine

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Kawasaki disease: the process. KD is manifested by clinical symptoms of fever, exanthem, conjunctival injection, enanthem, swelling and redness of the hands and feet, and cervical adenopathy. Pathologic features include, most notably, inflammation of the coronary arteries, but also inflammation of other medium‐sized arteries and other non‐vascular tissues. Intravenous immunoglobulin (IVIG) appears to abort both clinical symptoms and the progression of pathologic events.

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Kawasaki disease: a model of pathogenesis indicating the potential of genomics, transcriptomics, and proteomics to inform this serious illness of childhood.

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