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Cyclosporin A for IVIG Nonresponders

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Kawasaki Disease
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Abstract

The 2012 treatment guideline for Kawasaki disease (KD) proposes several second- and third-line therapies for patients unresponsive to intravenous immunoglobulin (IVIG). However, there are still no definite treatment recommendations for refractory KD. In 2005 we used cyclosporin A (CsA) to treat a case of refractory KD, and in 2008 it was reported that functional polymorphism of inositol 1,4,5-trisphosphate 3-kinase-C (ITPKC) is associated with susceptibility to KD and the risk of developing coronary arterial lesions. Because ITPKC acts as a negative regulator of T-cell activation through the NFAT pathway, activated T cells may be important in KD. CsA suppresses the activity of T cells through the same pathway and might therefore be a promising candidate for treatment of refractory KD. Here, we summarize the results of our clinical trials of CsA for refractory KD and propose a new CsA treatment option for KD.

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Correspondence to Hiroyuki Suzuki M.D., Ph.D. .

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Suzuki, H. (2017). Cyclosporin A for IVIG Nonresponders. In: Saji, B., Newburger, J., Burns, J., Takahashi, M. (eds) Kawasaki Disease. Springer, Tokyo. https://doi.org/10.1007/978-4-431-56039-5_20

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  • DOI: https://doi.org/10.1007/978-4-431-56039-5_20

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  • Publisher Name: Springer, Tokyo

  • Print ISBN: 978-4-431-56037-1

  • Online ISBN: 978-4-431-56039-5

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