4.6 Article

Kawasaki disease: Aetiopathogenesis and therapeutic utility of intravenous immunoglobulin

Journal

AUTOIMMUNITY REVIEWS
Volume 9, Issue 6, Pages 441-448

Publisher

ELSEVIER SCIENCE BV
DOI: 10.1016/j.autrev.2009.12.004

Keywords

Kawasaki disease; Vasculitis; Endothelial cells; Intravenous immunoglobulin

Categories

Funding

  1. INSERM
  2. CNRS
  3. University Pierre et Marie Curie Paris 6
  4. Universite Paris Descartes-Paris V. Laboratoire Francais du Fractionnement et des Biotechnologies
  5. Assistance Publique des Hopitaux de Paris
  6. FERCM

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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. (C) 2009 Elsevier B.V. All rights reserved.

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