Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2022 Dec;41(12):3759-3768.
doi: 10.1007/s10067-022-06319-4. Epub 2022 Aug 8.

Risk scores for Kawasaki disease, a management tool developed by the KAWA-RACE cohort

Carlos D Grasa  1   2   3 , Elisa Fernández-Cooke  4   5   6 , Sara Domínguez-Rodríguez  7   8 , Javier Aracil-Santos  1 , Ana Barrios Tascon  9 , Judith Sánchez-Manubens  10   11 , Beatriz Mercader  12 , Jordi Antón  10 , Esmeralda Nuñez  13 , Enrique Villalobos  14 , Matilde Bustillo  15 , Marisol Camacho  16 , Manuel Oltra Benavent  17 , Gemma Giralt  18 , Ana Maria Bello Naranjo  19 , Beatriz Rocandio  20 , Cristina Calvo  1   2   3   21 ; Spanish group for the study of Kawasaki Disease (KAWA-RACE)
Collaborators, Affiliations

Risk scores for Kawasaki disease, a management tool developed by the KAWA-RACE cohort

Carlos D Grasa et al. Clin Rheumatol. 2022 Dec.

Abstract

Introduction/objectives: Asian scores developed to predict unresponsiveness to intravenous immunoglobulin (IVIG) or development of coronary artery aneurysms (CAA) in patients with Kawasaki disease (KD) are not appropriate in Western populations. The purpose of this study is to develop 2 scores, to predict unresponsiveness to IVIG and development of CAA, appropriate for Spanish population.

Method: Data of 625 Spanish children with KD collected retrospectively (2011-2016) were used to identify variables to develop the 2 scores of interest: unresponsiveness to IVIG and development of CAA. A statistical model selected best variables to create the scores, and scores were validated with data from 98 patients collected prospectively.

Results: From 625 patients of the retrospective cohort, final analysis was performed in 439 subjects: 37 developed CAA, and 212 were unresponsive to IVIG. For the score to predict CAA, a cutoff ≥ 8 was considered for high risk, considering a score system with a different weight for each of the eight variables. External validation showed a sensitivity of 22% and a specificity of 75%. The score to predict unresponsiveness to IVIG established a cutoff ≥ 8 for high risk, considering a score system with a different weight for each of the nine variables. External validation showed a sensitivity of 78% and a specificity of 50%.

Conclusions: Two risk scores for KD were developed from Spanish population, to predict development of CAA and unresponsiveness to IVIG; validation in other cohorts could help to implement these tools in the management of KD in other Western populations.

Keywords: Coronary artery aneurysm; Kawasaki disease; Score; Unresponsiveness IVIG.

PubMed Disclaimer

References

    1. McCrindle BW, Rowley AH, Newburger JW et al (2017) Diagnosis, treatment, and long-term management of Kawasaki disease: a scientific statement for health professionals from the American Heart Association. Circulation 135:e927–e999. https://doi.org/10.1161/CIR.0000000000000484 - DOI - PubMed
    1. Newburger JW, Takahashi M, Burns JC et al (1986) The treatment of Kawasaki syndrome with intravenous gamma globulin. N Engl J Med 315:341–347. https://doi.org/10.1056/nejm198608073150601 - DOI - PubMed
    1. Mori M, Miyamae T, Imagawa T et al (2004) Meta-analysis of the results of intravenous gamma globulin treatment of coronary artery lesions in Kawasaki disease. Mod Rheumatol 14:361–366. https://doi.org/10.1007/s10165-004-0324-3 - DOI - PubMed
    1. Davies S, Sutton N, Blackstock S et al (2015) Predicting IVIG resistance in UK Kawasaki disease. Arch Dis Child 100:366–368. https://doi.org/10.1136/archdischild-2014-307397 - DOI - PubMed
    1. Egami K, Muta H, Ishii M et al (2006) Prediction of resistance to intravenous immunoglobulin treatment in patients with Kawasaki disease. J Pediatr 149:237–240. https://doi.org/10.1016/j.jpeds.2006.03.050 - DOI - PubMed

MeSH terms

Substances

LinkOut - more resources