How Should We Classify Kawasaki Disease?

Kawasaki syndrome is an acute, self-limited vasculitis that occurs in children of all ages and presents a challenge for the clinician: the disorder can be difficult to recognise; there is no diagnostic laboratory test; there is an extremely effective therapy; and there is a 25% chance of serious cardiovascular damage if the treatment is not given early in the course of the disease. This review includes discussion of the history of the syndrome, the diagnostic challenges, epidemiology, aetiology, pathology, immunopathogenesis, therapy, genetic influences, and the long-term cardiovascular sequelae.

Kawasaki disease (KD) is a systemic vasculitis that mainly affects children younger than 5 years. Although Dr. Tomisaku Kawasaki first reported KD over 40 years ago, the cause of the disease remains unknown. Currently, KD has been diagnosed in more than 60 countries, including those in Asia, the Middle East, Latin America, and Africa, as well as in North America and Europe. The purpose of this review is to describe the epidemiologic features of KD—particularly its incidence, seasonality, and the occurrence of coronary artery abnormalities—primarily in Japan and the United States, but also in Europe and other Asian countries.

To explore the increased incidence of intravenous immunoglobulin- (IVIG) resistance among San Diego County patients with Kawasaki disease (KD) in 2006 and to evaluate a scoring system to predict IVIG-resistant patients with KD. We performed a retrospective review of patients with KD treated within 10 days of fever onset. With multivariate analysis, independent predictors of IVIG-resistance were combined into a scoring system. In 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 percent bands, and higher concentrations of C-reactive protein, alanine aminotransferase, and gamma-glutamyl transferase. They also had lower platelet counts and age-adjusted hemoglobin concentrations and were more likely to have aneurysms (P = .0008). A scoring system developed to predict IVIG-resistant patients using illness day, percent bands, gamma-glutamyl transferase, and age-adjusted hemoglobin had a sensitivity of 73.3% and specificity of 61.9%. 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.