A child with severe relapsing Kawasaki disease rescued by IL-1 receptor blockade and extracorporeal membrane oxygenation

Kawasaki disease (KD) is an acute inflammatory vasculitis that predominantly occurs in children under 5 years of age. 1 It is associated with the development of coronary artery aneurysms (CAA) in 15-25% of untreated cases. 2 Standard treatment consists of high-dose intravenous immunoglobulins (IVIG) along with aspirin. 3 About 15% of patients do not respond to a single dose of IVIG and need retreatment. 4 When ongoing signs of active disease are present, methylprednisolone pulses are often administered. 5 If there is a lack of response, alternative anti-inflammatory medication such as infliximab or plasmapheresis have been suggested in individual case series. 6 7 We report, for the first time, the beneficial use of an interleukin-1 receptor antagonist (IL-1RA) in relapsing 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.