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Review
. 2010 Apr;9(6):441-8.
doi: 10.1016/j.autrev.2009.12.004. Epub 2010 Jan 13.

Kawasaki disease: aetiopathogenesis and therapeutic utility of intravenous immunoglobulin

Caroline Galeotti  1 , Jagedeesh Bayry, Isabelle Kone-Paut, Srinivas V Kaveri
Affiliations
Review

Kawasaki disease: aetiopathogenesis and therapeutic utility of intravenous immunoglobulin

Caroline Galeotti et al. Autoimmun Rev. 2010 Apr.

Abstract

Kawasaki disease (KD) is an acute febrile childhood vasculitis, associated with the development of coronary artery abnormalities in 25-30% of untreated patients. The aetiopathogenesis is not well known but it is accepted that an undefined infectious trigger in genetically predisposed individuals results in the disease. KD is characterized by an endothelial cell injury, which could be due to abnormal cytokine production and to generation of cytotoxic antibodies against the endothelial cells. Intravenous immunoglobulin IVIG is an effective treatment in preventing the occurrence of coronary artery abnormalities in KD. Several mechanisms may explain the anti-inflammatory effects of IVIG in this disease. They include modification of the cytokine balance, and alteration on both the differentiation and the function of monocytes/macrophages, neutrophils and lymphocytes.

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Figures

Fig. 1
Fig. 1
A schematic representation of the proposed mechanisms of action of IVIG in Kawasaki disease.
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