Kawasaki Disease

J Am Coll Cardiol. 2016 Apr 12;67(14):1738-49. doi: 10.1016/j.jacc.2015.12.073.

Abstract

Kawasaki disease is an acute, self-limited vasculitis of unknown etiology that occurs predominantly in infants and children. If not treated early with high-dose intravenous immunoglobulin, 1 in 5 children develop coronary artery aneurysms; this risk is reduced 5-fold if intravenous immunoglobulin is administered within 10 days of fever onset. Coronary artery aneurysms evolve dynamically over time, usually reaching a peak dimension by 6 weeks after illness onset. Almost all the morbidity and mortality occur in patients with giant aneurysms. Risk of myocardial infarction from coronary artery thrombosis is greatest in the first 2 years after illness onset. However, stenosis and occlusion progress over years. Indeed, Kawasaki disease is no longer a rare cause of acute coronary syndrome presenting in young adults. Both coronary artery bypass surgery and percutaneous intervention have been used to treat Kawasaki disease patients who develop myocardial ischemia as a consequence of coronary artery aneurysms and stenosis.

Keywords: aneurysm; coronary aneurysm; coronary thrombosis; immunoglobulin; intravenous; mucocutaneous lymph node syndrome; myocardial infarction.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Adaptive Immunity
  • Adrenergic beta-Antagonists / therapeutic use
  • Anti-Inflammatory Agents, Non-Steroidal / therapeutic use
  • Aspirin / therapeutic use
  • Coronary Aneurysm / prevention & control
  • Coronary Thrombosis / prevention & control
  • Diagnostic Imaging
  • Glucocorticoids / therapeutic use
  • Humans
  • Immunity, Innate
  • Immunoglobulins, Intravenous / therapeutic use
  • Motor Activity
  • Mucocutaneous Lymph Node Syndrome / complications*
  • Mucocutaneous Lymph Node Syndrome / diagnosis
  • Mucocutaneous Lymph Node Syndrome / therapy*
  • Myocardial Ischemia / prevention & control
  • Myocardial Revascularization
  • Risk Factors

Substances

  • Adrenergic beta-Antagonists
  • Anti-Inflammatory Agents, Non-Steroidal
  • Glucocorticoids
  • Immunoglobulins, Intravenous
  • Aspirin