Objectives-To explore the increased incidence of intravenous immunoglobulin (IVIG) resistance among San Diego County Kawasaki disease (KD) patients in 2006 and to evaluate a scoring system to predict IVIG-resistant patients with KD.Study design-We performed a retrospective review of patients with KD treated within 10 days of fever onset. Using multivariate analysis, independent predictors of IVIG-resistance were combined into a scoring system. 2006, 38.3 % of patients with KD in San Diego County were IVIG-resistant, a significant increase over previous years. IVIG-resistance was not associated with a particular brand or lot of IVIG. Resistant patients were diagnosed earlier, had higher % bands, and higher concentrations of C-reactive protein, alanine aminotransferase, and γ-glutamyl transferase (GGT). They also had lower platelet counts and age-adjusted hemoglobin (zHgb) concentrations and were more likely to have aneurysms (p=0.0008). A scoring system developed to predict IVIG-resistant patients using illness day, % bands, GGT, and zHgb, had a sensitivity of 73.3% and specificity of 61.9%.
Results-InConclusions-An unexplained increase in IVIG-resistance was noted among patients with KD in San Diego County in 2006. Scoring systems based on demographic and laboratory data were insufficiently accurate to be clinically useful in our ethnically diverse population. Publisher's Disclaimer: This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
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Conflict of Interests:The authors have no conflicts of interest to report. Kawasaki disease (KD), the leading cause of pediatric acquired heart disease in the United States and Japan, is an acute, systemic vasculitis. Treatment with a single dose of intravenous immunoglobulin (IVIG) and high-dose aspirin results in resolution of fever in most patients and significantly reduces the rate of coronary artery aneurysms (1). Despite this success, 10-20% of children will have persistent or recrudescent fever after their first infusion of IVIG (2-5). These patients are at increased risk of developing coronary artery abnormalities (5,6). Additional therapies used in these patients include retreatment with IVIG, immunomodulatory agents such as infliximab, high dose methylprednisolone, cyclophosphamide, and plasmapheresis. (7-11). Identification of patients who are likely to be IVIG-resistant would allow the use of additional therapies early in the course of their illness when prevention of coronary artery damage might still be possible.
NIH Public AccessA number of recent studies from Asia have identified demographic and laboratory characteristics, including age...