How is IV immunoglobulin (IVIG)-resistant Kawasaki disease treated?

Updated: Jul 29, 2018
  • Author: Tina K Sosa, MD; Chief Editor: Russell W Steele, MD  more...
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Treatment of patients in which IVIG fails after the first and/or second dose remains controversial and is variable across institutions and providers. Guidelines from the AHA recommend a second dose of IVIG, methylprednisolone, a longer tapering course of prednisolone or prednisone plus IVIG, or infliximab be considered for patients resistant to IVIG. [2]  Infliximab (Remicade) is a chimeric mouse-human monoclonal antibody directed against soluble and membrane bound tumor necrosis factor-alpha. [9] Several studies have found infliximab at a dose of 5 mg/kg to be useful in treating KD that is refractory to IVIG. [72, 73] Burns et al reported that infliximab was as effective as a second dose of IVIG in patients who did not respond to a first dose of IVIG. [74]  In another study, 43 patients with KD who were initially resistant to IVIG were randomized to receive either a first does infliximab (n=11) or second dose of IVIG (n=32). IVIG retreatment gave 65.6% of patients a response while infliximab gave 90.9% of patients a response. Infliximab provided less days of hospitalization and a shorter duration of fever. Adverse events and coronary artery outcomes resembled each other in the two groups. [75]

The AHA recommends that cyclosporine and other cytotoxic agents, immunomodulatory monoclonal antibody therapy, and plasma exchange be reserved for exceptional patients with particularly refractory KD.

In the future, by identifying a genetic signature for this group, more aggressive and targeted therapies may be used to reduce the risk of coronary complications. [33, 22]

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