Predisposing factors, pathogenesis and therapeutic intervention of Kawasaki disease

Caroline Galeotti1, Srini V Kaveri2, Rolando Cimaz3

  • 1Institut National de la Santé et de la Recherche Médicale Unité 1138, Paris F-75006, France; Sorbonne Universités, UPMC Univ Paris 06, UMR S 1138, Paris F-75006, France; Centre de Recherche des Cordeliers, Equipe - Immunopathology and Therapeutic Immunointervention, Paris F-75006, France; Department of Pediatric Rheumatology, National Referral Centre of Auto-inflammatory Diseases, CHU de Bicêtre, le Kremlin Bicêtre, University of Paris Sud, F-94270, France.

Drug Discovery Today
|August 11, 2016
PubMed

Insights

Kawasaki disease (KD) is a childhood inflammatory condition affecting coronary arteries. This review explores KD causes, immune responses, and treatments, focusing on options for patients resistant to standard intravenous immunoglobulin (IVIG) therapy.

Area of Science:

  • Pediatric rheumatology
  • Immunology
  • Cardiology

Background:

  • Kawasaki disease (KD) is an acute febrile illness in children.
  • It is characterized by inflammation of coronary arteries, potentially leading to abnormalities.
  • The exact cause is unknown but involves an abnormal immune response to an infection in genetically susceptible children.

Purpose of the Study:

  • To review predisposing factors and pathogenesis of Kawasaki disease.
  • To highlight current and emerging therapeutic interventions for KD.
  • To focus on novel treatments for patients resistant to intravenous immunoglobulin (IVIG) therapy.

Main Methods:

  • Literature review of studies on Kawasaki disease.
  • Analysis of pathogenesis, including endothelial cell injury and immune cell activation.
  • Evaluation of standard and alternative treatment strategies.

Main Results:

  • Standard treatment includes IVIG and aspirin, effective in most cases.
  • 10-20% of patients are resistant to IVIG, increasing the risk of coronary vasculitis.
  • The efficacy of second IVIG infusions, corticosteroids, and other immunomodulators is still under investigation.

Conclusions:

  • Understanding KD pathogenesis is crucial for effective treatment.
  • New therapeutic agents are needed for IVIG-resistant KD.
  • Further research is required to clarify the roles of various immunomodulatory agents in KD management.

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