Pathophysiology of Kawasaki disease

Anne H. Rowley*, Stanford T. Shulman, Jan M. Orenstein

*Corresponding author for this work

Research output: Chapter in Book/Report/Conference proceedingChapter

2 Scopus citations


Kawasaki Disease (KD) vasculopathy, which most significantly affects the coronary arteries, is characterized by three linked pathological processes: necrotizing arteritis, subacute/chronic (SA/C) vasculitis, and luminal myofibroblastic proliferation (LMP). Necrotizing arteritis (NA), initiated at the endothelial luminal surface, leads to giant aneurysms that can rupture or thrombose. SA/C vasculitis begins in the adventitia and is closely associated with LMP. LMP consists of actively proliferating smooth muscle cell-derived myofibroblasts and their matrix products, and can result in progressive arterial luminal stenosis. All three processes begin in the first 2 weeks after fever onset. NA subsides in the first 2 weeks, while subacute/chronic vasculitis and LMP can persist for months or years. The clinical and epidemiological features of KD are best explained by infection with an as-yet-unidentified ubiquitous agent, likely a virus entering via the respiratory route. Recent advances in genomics and RNA sequencing are beginning to reveal specific immune response dysfunction in KD that could lead to new diagnostics and therapeutics for this important childhood illness.

Original languageEnglish (US)
Title of host publicationKawasaki Disease
Subtitle of host publicationCurrent Understanding of the Mechanism and Evidence-Based Treatment
PublisherSpringer Japan
Number of pages6
ISBN (Electronic)9784431560395
ISBN (Print)9784431560371
StatePublished - Jan 1 2016


  • Gene expression
  • Luminal myofibroblastic proliferation
  • Necrotizing arteritis
  • Pathology
  • Subacute/chronic vasculitis

ASJC Scopus subject areas

  • General Medicine
  • General Immunology and Microbiology


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