Recent advances in the understanding and management of kawasaki disease

Research output: Contribution to journalReview articlepeer-review

29 Scopus citations

Abstract

disease (KD) is an acute systemic inflammatory illness of childhood that can result in coronary artery aneurysms, myocardial infarction, and sudden death. Clinical and epidemiologic data point to an unknown infectious agent as the cause. We discovered that an oligoclonal IgA immune response is present in arterial tissue in acute KD. Synthetic versions of prevalent IgA antibodies in the KD arterial wall identify cytoplasmic inclusion bodies in acute KD ciliated bronchial epithelium and other inflamed KD tissues. Light and electron microscopic studies show that the inclusion bodies are consistent with aggregates of viral protein and RNA, and are likely formed by the KD etiologic agent. KD susceptibility is likely to be polygenic. Treatment of gammaglobulin nonresponders usually consists of additional intravenous immunoglobulin, methylprednisolone, and/or infliximab. Additional data regarding KD pathogenesis are urgently needed to provide other targets for therapy for those patients at highest risk of developing coronary artery abnormalities.

Original languageEnglish (US)
Pages (from-to)96-102
Number of pages7
JournalCurrent Infectious Disease Reports
Volume12
Issue number2
DOIs
StatePublished - Mar 2010

Keywords

  • Coronary artery aneurysm
  • IgA
  • Inclusion bodies
  • Intravenous immunoglobulin

ASJC Scopus subject areas

  • Infectious Diseases

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