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Encyclopedia of Medical Immunology pp 1277–1284Cite as

Vasculitis: Kawasaki Disease

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Synonyms

Mucocutaneous lymph node syndrome

Definition

Kawasaki Disease (KD) is a childhood vasculitis affecting small- and medium-sized blood vessels and manifesting classically as fever, bilateral conjunctivitis, erythema of the lips and oral mucosa, cervical lymphadenopathy, rash, and extremity changes.

Introduction

Kawasaki Disease (KD) is the second most common childhood vasculitis and the leading cause of acquired heart disease among children in the developed world (Burns and Glodé 2004). It is an acute, self-limited febrile illness of unknown cause, characterized by fever and signs of mucocutaneous inflammation. Fifteen to 25 % of untreated children develop coronary artery abnormalities, placing them at risk of developing ischemic heart disease or sudden death later in life (Kato et al. 1996). Therapy in the acute phase is aimed at reducing inflammation using intravenous immunoglobulin (IVIG), thereby minimizing the risk of coronary artery aneurysms and other late sequelae.

Diagno...

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Author information

Authors and Affiliations

  1. Boston Children’s Hospital, Boston, MA, 02115, USA

    Elizabeth Ang

  2. Boston Children's Hospital, Harvard Medical School, Boston, MA, 02115, USA

    Robert Sundel

Authors
  1. Elizabeth Ang
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  2. Robert Sundel
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Corresponding author

Correspondence to Elizabeth Ang .

Editor information

Editors and Affiliations

  1. Department of Biochemistry and Molecular Biology, Monash University, Clayton, Victoria, Australia

    Ian R. Mackay

  2. Center for Autoimmune Disease Research, Department of Molecular Microbiology and Immunology, Department of Pathology, Johns Hopkins University, Baltimore, MD, USA

    Noel R. Rose

  3. The Feinstein Institute for Medical Research, Manhasset, NY, USA

    Betty Diamond

  4. The Feinstein Institute for Medical Research, Manhasset, NY, USA

    Anne Davidson

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© 2014 Springer Science+Business Media New York

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Ang, E., Sundel, R. (2014). Vasculitis: Kawasaki Disease. In: Mackay, I.R., Rose, N.R., Diamond, B., Davidson, A. (eds) Encyclopedia of Medical Immunology. Springer, New York, NY. https://doi.org/10.1007/978-0-387-84828-0_394

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  • DOI: https://doi.org/10.1007/978-0-387-84828-0_394

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  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-0-387-84827-3

  • Online ISBN: 978-0-387-84828-0

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