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Cytokine Storm Syndrome pp 393–406Cite as

Kawasaki Disease-Associated Cytokine Storm Syndrome

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Abstract

Kawasaki disease (KD) is an acute systemic vasculitis predominantly affecting young children characterized by fever, non-suppurative conjunctival injection, rash, oral mucositis, extremity changes, and cervical lymphadenopathy. Cytokine storm syndrome (CSS) is an acute complication in ~2% of KD patients; however, the incidence is likely underestimated as many clinical and laboratory features of both diseases overlap. CSS should be entertained when a child with KD is unresponsive to IVIG therapy with recalcitrant fever. Among laboratory criteria, hyperferritinemia (>500 ng/mL) is the most practical addition to standard KD laboratory tests, but the usefulness of serial laboratory measurements to detect trends in transaminases, white blood cell and platelet counts cannot be overemphasized. Early recognition and prompt institution of immunomodulatory treatment can substantially reduce the mortality and morbidity of CSS in KD. Given the known pathogenetic role of IL-1β in both syndromes, the early use of IL-1 blockers in refractory KD with CSS deserves consideration.

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Disclosures

R.U.: Nothing to disclose.

M.A.: Nothing to disclose.

A.D.: Nothing to disclose.

R.S.M.Y.: Research grant funding from the Canadian Institutes of Health Research, Genome Canada, ZonMw, Reumafonds, Arthritis Foundation, The Arthritis Society, CARRA, and the Heart and Stroke Foundation of Canada.

Financial support for this work: None.

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Authors and Affiliations

  1. Division of Cell Biology, The Hospital for Sick Children, Department of Paediatrics, University of Toronto, Toronto, ON, Canada

    Rolando Ulloa-Gutierrez, Martin Prince Alphonse & Rae S. M. Yeung

  2. Department of Immunology, University of Toronto, Toronto, ON, Canada

    Martin Prince Alphonse & Rae S. M. Yeung

  3. Division of Rheumatology, The Hospital for Sick Children, Department of Paediatrics, University of Toronto, Toronto, ON, Canada

    Anita Dhanranjani & Rae S. M. Yeung

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  1. Rolando Ulloa-Gutierrez
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Editors and Affiliations

  1. UAB School of Medicine, University of Alabama at Birmingham, Birmingham, AL, USA

    Randy Q. Cron

  2. Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA

    Edward M. Behrens

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Ulloa-Gutierrez, R., Alphonse, M.P., Dhanranjani, A., Yeung, R.S.M. (2019). Kawasaki Disease-Associated Cytokine Storm Syndrome. In: Cron, R., Behrens, E. (eds) Cytokine Storm Syndrome. Springer, Cham. https://doi.org/10.1007/978-3-030-22094-5_23

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