[Cyclosporin A treatment for refractory Kawasaki disease].

The association between functional polymorphism of inositol 1,4,5-trisphosphate 3- kinase-C(ITPKC) and susceptibility to Kawasaki disease(KD) and formation of coronary arterial lesions was reported in 2008. Since ITPKC acts as a negative regulator of T-cell activation, activated T cells may play a pivotal role in the pathogenesis of KD. Cyclosporin A(CsA), which potently suppresses the activity of T cells through negative regulation of the nuclear factor of activated T cells(NFAT) pathway, may be a promising candidate for the treatment of refractory KD. In this review, we summarize the results of our clinical trials of CsA for refractory KD, the changes in the levels of cytokines before and after CsA treatment, and the future direction of CsA treatment for refractory KD.