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Published on: September 14, 2010
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.
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.
Abstract:
Kawasaki disease (KD) is an acute febrile childhood inflammatory disease, associated with coronary artery abnormalities. The disease is believed to result from an aberrant inflammatory response to an infectious trigger in a genetically predisposed individual. KD is associated with an endothelial cell injury as a consequence of T cell activation and cytotoxic effects of various proinflammatory cytokines. Intravenous immunoglobulin (IVIG) infusion and aspirin are the standard treatment of acute KD. However, 10-20% of patients show resistance to IVIG therapy and present higher risk of coronary vasculitis. The relative roles of second IVIG infusion, corticosteroids, calcineurin inhibitors, interleukin-1 antagonists and anti-tumor necrosis factor agents remain uncertain. In this review, we highlight the predisposing factors, pathogenesis and therapeutic intervention of KD, particularly new therapeutics for IVIG-resistant patients.
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