DISCUSSION

Several studies have suggested that immune activation and secretion of cytokines contribute to the pathogenesis of KD. Elevated levels of IL-1 have been reported in acute patients and have been correlated to vascular endothelial cell damage. 8–10 IVIG treatment was associated with a decrease in IL-1, but levels remain elevated in refractory patients. 9 These observations suggested a potential role for IL-1R blockers, which is supported by the lasting response to anakinra observed in our patient. Lee et al recently showed in a mice model for KD that IL-1 is indeed critically involved in coronary arteritis and that CAA development can be prevented by IL-1R blockade. 11 This is the first report of the beneficial use of an IL-1RA in KD. Although the clinical recovery may be coincidental, we have observed a clinical response of fever to IL-1RA twice in this patient. Relapse and rapid deterioration of both his clinical and coronary findings after stopping IL-1RA after 7 days suggest its beneficial role in preventing vascular injury from progressing or normalisation upon injury. This case suggests the potential benefit of IL-1 blockade in refractory KD. Further research regarding the efficacy of IL-1-blockade in KD appears to be warranted.