Treatment options for refractory Kawasaki disease: Alternative treatments for infliximab nonresponders

In a recent study, infliximab (IFX) therapy resulted in dramatic improvement in 85–90% of children with Kawasaki disease who did not respond to repeated intravenous immunoglobulin (IVIG) infusion or steroid therapy as second-line treatment. Although studies have confirmed the clinical efficacy and safety of IFX therapy in children with refractory Kawasaki disease, there is no consensus regarding treatment of IFX nonresponse, which is defined as the presence of persistent fever (<37 °C) despite optimal treatment. In a treatment algorithm from two Japanese institutions, IFX was given to children who did not respond to second IVIG or methylprednisolone pulse (IMP) therapy. Overall, 10–26% were IFX nonresponders, and treatments for this group included IVIG, methylprednisolone pulse, and cyclosporin A. Persistent fever resolved in IFX nonresponders after these additional treatments, although some patients developed coronary artery abnormalities. There has been no evidence-based study of optimal treatment for IFX nonresponders. If fever or elevation of C-reactive protein does not resolve after IFX therapy, therapies other than IFX, such as re-IVIG, re-IMP, and other immunosuppressive agents, should be started.