Role of the Egami score to predict immunoglobulin resistance in Kawasaki disease among a Western Mediterranean population

Sánchez-Manubens, Judith; Antón, Jordi; Bou, Rosa; Iglesias, E.; Calzada-Hernández, Joan; Borlan, Sergi; Gimenez-Roca, Clara; Rivera, Josefa

doi:10.1007/s00296-016-3499-y

Cited by 30 publications

(21 citation statements)

References 19 publications

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“…Scoring systems have been established and validated in the Japanese population but they have some limitations. First, resistance to IVIG has not a universal definition in terms of persistence or length of reappraisal of fever, second, fever alone may not be the unique indicator of insufficient response as persistence of elevated CRP is associated also with cardiac complications, and finally those scores have not been validated outside the Japanese population [15][16][17][18][19] . We took the opportunity of the Kawanet, the widest prospective epidemiologic tool ever set up in France, a non-Asian country including multiple ethnic groups and also mixed ethnicities, to evaluate the frequency of secondary treatment after standard treatment i.e.…”

Section: Discussionmentioning

confidence: 99%

“…However, and to our knowledge, for the first time, we observed a better performance of two of these scores in African/Afro-Caribbean patients compared to other ethnic groups, with the exclusion of Asians. Prediction of IVIg resistance is crucial to intensify initial treatment combined with IVIg to prevent cardiac complications 12,14,36,37 , but none of the currently www.nature.com/scientificreports www.nature.com/scientificreports/ published scores have good sensitivity in Caucasian populations [15][16][17][18][19]36,38 . In our multiethnic population, we identified predictive factors of IVIg resistance based on real-life practice, and built a scoring system and obtained for the first time, good sensitivity (77%) and acceptable specificity (60%) in our non-Asian population.…”

Section: Discussionmentioning

confidence: 99%

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Defining the risk of first intravenous immunoglobulin unresponsiveness in non-Asian patients with Kawasaki disease

Piram

Bello

Tellier

et al. 2020

Sci Rep

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About 10-20% of patients with Kawasaki disease (KD) are unresponsive to intravenous immunoglobulin (IVIg) and are at increased risk of coronary artery abnormalities (CAAs). Early identification is critical to initiate aggressive therapies, but available scoring systems lack sensitivity in non-Japanese populations. We investigated the accuracy of 3 Japanese scoring systems and studied factors associated with IVIg unresponsiveness in a large multiethnic French population of children with KD to build a new scoring system. Children admitted for KD between 2011-2014 in 65 centers were enrolled. Factors associated with second line-treatment; i.e. unresponsiveness to initial IVIg treatment, were analyzed by multivariate regression analysis. The performance of our score and the Kobayashi, Egami and Sano scores were compared in our population and in ethnic subgroups. Overall, 465 children were reported by 84 physicians; 425 were classified with KD (55% European Caucasian, 12% North African/ Middle Eastern, 10% African/Afro-Caribbean, 3% Asian and 11% mixed). Eighty patients (23%) needed second-line treatment. Japanese scores had poor performance in our whole population (sensitivity 14-61%). On multivariate regression analysis, predictors of secondary treatment after initial IVIG were hepatomegaly, ALT level ≥30 IU/L, lymphocyte count <2400/mm 3 and time to treatment <5 days. The best sensitivity (77%) and specificity (60%) of this model was with 1 point per variable and cutoff ≥2 points. The sensitivity remained good in our 3 main ethnic subgroups (74-88%). We identified predictors of IVIg resistance and built a new score with good sensitivity and acceptable specificity in a non-Asian population. Kawasaki disease (KD) is the leading cause of acquired heart disease in childhood in developed countries 1. The level of coronary artery involvement mainly determines the prognosis of this systemic vasculitis affecting predominantly young children, although pericarditis, myocarditis and valvular dysfunction are not uncommon 1,2. Occasionally, KD can be complicated during the acute phase by shock syndrome 3 , macrophage activation syndrome 4 , or myocardial infarction 1. Although the mortality rate is relatively low during the acute phase, sudden death due to myocardial ischemia could occur many years later in children or adults with coronary artery sequelae 1. The efficacy of early treatment with intravenous immunoglobulin (IVIg) is well established 5,6 and has reduced the prevalence of coronary artery abnormalities (CAAs) from 26-30% to 2.5-5% at 1 month after disease onset 6,7. However, 30% to 40% of KD patients develop coronary dilatations within the first days of the disease 8. In addition,

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Defining the risk of first intravenous immunoglobulin unresponsiveness in non-Asian patients with Kawasaki disease

Piram

Bello

Tellier

et al. 2020

Sci Rep

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“…In cohort studies, Jakob et al ( 43 ) and Sánchez-Manubens et al ( 44 ) found that IVIG resistance and the risk of developing CAAs were not assessed with Asian risk scores in German and Spanish cohorts (95% of the German cohort and 81.1% of the Spanish cohort).…”

Section: Discussionmentioning

confidence: 99%

How useful are Kawasaki disease risk scoring systems to the Turkish population?

2020

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Objective: Kawasaki disease (KD) is the most common cause of coronary artery aneurysm (CAA) in children. The available risk scores to predict intravenous immunoglobulin (IVIG) resistance and CAA were developed in Asian populations in whom their effectiveness has been proven, but data on non-Asian children are limited. This study aimed to evaluate the ability of 5 risk scoring systems to predict IVIG resistance and CAA in Turkey patients with KD. Methods: Patients with KD were retrospectively evaluated with clinical, laboratory, and echocardiographic findings. Data analyses were performed in 5 scoring systems (Harada, Kobayashi, Egami, Formosa, and Sano). Results: A total of 259 patients (Male: Female, 1.7) were treated for KD in our hospital. The mean age of diagnosis in patients with KD, CAA, and IVIG resistance were 3.31, 2.19, and 2.06, respectively. CAA development and IVIG resistance were seen in 11.6% and 12.3% of cases, respectively. IVIG resistance was detected in 35.6% of patients with CAA. In our study, 5 risk scoring systems were applied to our patients. ROC analysis results were found highest in Kobayashi scoring system for IVIG resistance (AUC, 0.864) and in Harada scoring system for CAA development (AUC, 0.727). Conclusion: Harada score was significant in predicting CAA risk, and Kobayashi score was significant in predicting the risk of developing IVIG resistance. It is necessary to determine more specific and sensitive risk scores that increase the risk of IVIG resistance and the development of CAA in Turkey.

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“…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.…”

Section: Treatmentmentioning

confidence: 99%

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

et al. 2017

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Kawasaki disease (KD) is a pediatric vasculitis with coronary artery aneurysms (CAA) as its main complication. The diagnosis is based on the presence of persistent fever and clinical features including exanthema, lymphadenopathy, conjunctival injection, and changes to the mucosae and extremities. Although the etiology remains unknown, the current consensus is that it is likely caused by an (infectious) trigger initiating an abnormal immune response in genetically predisposed children. Treatment consists of high dose intravenous immunoglobulin (IVIG) and is directed at preventing the development of CAA. Unfortunately, 10–20% of all patients fail to respond to IVIG and these children need additional anti-inflammatory treatment. Coronary artery lesions are diagnosed by echocardiography in the acute and subacute phases. Both absolute arterial diameters and z-scores, adjusted for height and weight, are used as criteria for CAA. Close monitoring of CAA is important as ischemic symptoms or myocardial infarction due to thrombosis or stenosis can occur. These complications are most likely to arise in the largest, so-called giant CAA. Apart from the presence of CAA, it is unclear whether KD causes an increased cardiovascular risk due to the vasculitis itself. Conclusion: Many aspects of KD remain unknown, although there is growing knowledge on the etiology, treatment, and development and classification of CAA. Since children with previous KD are entering adulthood, long-term follow-up is increasingly important. What is known: • Kawasaki disease (KD) is a pediatric vasculitis with coronary artery damage as its main complication. • Although KD approaches its 50th birthday since its first description, many aspects of the disease remain poorly understood. What is new: • In recent years, multiple genetic candidate pathways involved in KD have been identified, with recently promising information about the ITPKC pathway. • As increasing numbers of KD patients are reaching adulthood, increasing information is available about the long-term consequences of coronary artery damage and broader cardiovascular risk.

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Role of the Egami score to predict immunoglobulin resistance in Kawasaki disease among a Western Mediterranean population

Cited by 30 publications

References 19 publications

Defining the risk of first intravenous immunoglobulin unresponsiveness in non-Asian patients with Kawasaki disease

Defining the risk of first intravenous immunoglobulin unresponsiveness in non-Asian patients with Kawasaki disease

How useful are Kawasaki disease risk scoring systems to the Turkish population?

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

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