Role of the Egami Score in Predicting Intravenous Immunoglobulin Resistance in Kawasaki Disease Among Different Ethnicities

Abstract

Early treatment with intravenous immunoglobulin (IVIG) is necessary to help reduce the risk of coronary artery abnormalities, such as coronary artery aneurysms and to help alleviate symptoms, in Kawasaki disease. Some patients, however, do not respond to an initial dose of IVIG and require additional doses. Prediction of these IVIG nonresponders may be of assistance in altering initial therapy to make it more effective. The Egami score has been validated in the Japanese population to predict IVIG nonresponders but has shown to be ineffective in US populations. This study evaluates the Egami score in a Midwest US population, subdividing patients by race and the diagnosis of typical or atypical type of Kawasaki disease. Patients were included in the study if they met criteria for Kawasaki disease and received IVIG in the inpatient setting. A total of 182 patients were studied, and in all studied groups, the Egami score had poor sensitivity at predicting IVIG nonresponders. Sensitivity of the score differed between races and differed between typical and atypical Kawasaki disease. The Egami score, as well as other systems, have been validated to predict IVIG nonresponders. These, however, lack sensitivity in the US population. Other scores developed in the United States have also lacked sensitivity, likely due to the absence of race or Kawasaki disease classification as variables. The development of a sensitive scoring system to predict IVIG nonresponders in US populations will require the incorporation of race and Kawasaki disease classification, factors that seem to alter IVIG response.

A novel model for predicting non‐responsiveness to intravenous immunoglobulins in Kawasaki disease: The Singapore experience

“…A likely difference in the performance of the risk‐scoring methods among various populations can be postulated to be secondary to the genetic differences in the study populations. These same factors could likewise play a role in the overall lower IVIG non‐responsiveness rate observed in our population (9.7%) compared to the generally higher range reported in the literature, 9.7–32.2% (Table ) …”

“…These scoring methods include the Egami, Kobayashi, Sano and Fukunishi, all developed in the Japanese populations. The predictive performances of these risk‐scoring methods have, however, shown variable success in populations of different ethnic backgrounds, with generally poor sensitivities (SN). Even in more closely related ethnic populations such as Chinese and Koreans, lower SN were observed.…”

Prognostic nutrition index as a predictor of coronary artery aneurysm in Kawasaki Disease

“…Assuming that both research methods were appropriately and strictly designed, we may presume that an unknown ongoing process involved nutrition status, in addition to vascular inflammation. However, early validation research on Japan scoring models yield inconsistent result between different races (2,(22)(23)(24). It showed multiple ethnicity-exclusive models are required.…”

Dissecting Kawasaki disease: a state-of-the-art review

“…In Japan, risk-scores have been developed to identify patients with a higher risk of IVIG resistance [35, 66, 106]. Unfortunately, these risk-scores do not perform adequately in Western, ethnically mixed, and in Chinese populations [24, 75, 104, 112, 115]. A possible method to decrease IVIG resistance is to intensify the initial treatment.…”