Progress In IVIG Resistance Kawasaki Disease

Kawasaki disease is a systemic vasculitis of the medium-sized arteries,predominantly affecting the coronary arteries.The principal objective of kawasaki disease treatment is minimizing the risk of developing coronary artery aneurysms.In practice,this means quickly suppressing the acute-phase inflammatory reaction caused by Kawasaki disease.Standard first line treatment for Kawasaki disease consists of intravenous immunoglobulin(IVIG)and aspirin.Current guidelines recommend 2g/kg of IVIG and 80 to 100mg/kg of aspirin administered within the first 7 days of illness.For most patients,treatment with single dose of IVIG and high dose aspirin resolve the fever and reduce the rate of coronary artery aneurysms significantly.However,after their first infusion of IVIG,10% to 20% of children with Kawasaki disease will have persistent or recrudescent fever.These IVIG resistance children are at increased risk for development of coronary artery abnormalities.This study focused on the progress in IVIG resistance Kawasaki disease.Additional therapies for these patients include retreatment with IVIG,intravenous methylprednisolone,immunomodulatory agents such as infliximab,cyclophosphamide,methotrexate and plasmapheresis.Identification of patients who are likely to be IVIG resistance would allow the use of additional therapies early in the course of their illness when prevention of coronary artery damage might still be possible.