18. Kawasaki Disease

  1. Gary S. Hoffman MD, MS3,
  2. Cornelia M. Weyand MD, PhD4,
  3. Carol A. Langford MD, MHS3 and
  4. Jörg J. Goronzy MD, PhD4
  1. Rae S. M. Yeung MD, PhD, FRCPC1,2

Published Online: 3 MAY 2012

DOI: 10.1002/9781118355244.ch18

Inflammatory Diseases of Blood Vessels, Second Edition

Inflammatory Diseases of Blood Vessels, Second Edition

How to Cite

Yeung, R. S. M. (2012) Kawasaki Disease, in Inflammatory Diseases of Blood Vessels, Second Edition (eds G. S. Hoffman, C. M. Weyand, C. A. Langford and J. J. Goronzy), Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781118355244.ch18

Editor Information

  1. 3

    Department of Rheumatic and Immunologic Diseases, Center for Vasculitis Care and Research, Cleveland Clinic, Lerner College of Medicine, Cleveland, OH, USA

  2. 4

    Department of Medicine, Stanford University School of Medicine, Stanford, CA, USA

Author Information

  1. 1

    Departments of Pediatrics, Immunology and Medical Science, University of Toronto, Canada

  2. 2

    The Hospital for Sick Children, Toronto, ON, Canada

Publication History

  1. Published Online: 3 MAY 2012
  2. Published Print: 8 JUN 2012

ISBN Information

Print ISBN: 9781444338225

Online ISBN: 9781118355244



  • Kawasaki's disease;
  • vasculitis;
  • inflammation;
  • coronary artery;
  • aneurysms;
  • TNF;
  • echocardiography;
  • IVIG;
  • ASA;
  • children


Kawasaki's disease (KD) is a common multisystem vasculitis of childhood. The coronary arteries are targets of long-term inflammation and damage. The challenge at the bedside is recognition of children who present with an incomplete clinical presentation. A high index of suspicion in every child with prolonged fever and signs or symptoms consistent with KD, coupled with close clinical follow-up and appropriate laboratory investigations and imaging is needed. Vascular inflammation leading to coronary artery damage and aneurysm formation is the underlying pathology in KD. The link between the systemic immune response seen in the acute phase of KD and subsequent damage to the coronary arteries is not clearly understood. Evidence points to TNF-α and its downstream effector molecules as key players in mediating coronary artery damage. Early identification and treatment of affected children, with high dose intravenous immunoglobulin and aspirin, to suppress the inflammatory response is one of the objectives of therapy.