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  • Population Study Article
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Association between the absence of individual principal clinical features and coronary artery abnormalities in complete Kawasaki disease

Pediatric Research (2026)Cite this article

Abstract

Background

A previous study reported that among patients with complete Kawasaki Disease (KD), those exhibiting all six principal clinical features were more likely to develop coronary artery (CA) sequelae than those exhibiting only five features. We aimed to determine which specific features are associated with CA sequelae.

Methods

This retrospective cohort study analyzed 14,732 patients diagnosed with complete KD across Japan from January 2019 to March 2020. Separate multivariable conditional logistic regression analyses were performed to evaluate relative risk for CA sequelae in patients with all six principal clinical features, compared individually to those lacking each specific feature.

Results

7234 (49.1%) exhibited all six principal clinical features, while 7498 (50.9%) presented with five features. CA sequelae occurred in 2.1% of those with six features versus 1.7% with five. Multivariable conditional logistic regression analysis determined that patients with conjunctival injection were significantly more likely to develop CA sequelae compared with those lacking it (adjusted odds ratio [95% confidence interval], 3.6 [1.3–10.1]).

Conclusions

Among patients with complete KD, the absence of conjunctival injection—a relatively rare presentation—was associated with a lower cumulative incidence of CA sequelae. This finding may help identify distinct low-risk phenotypes of KD and support risk stratification.

Impact

  • This study emphasizes the importance of feature-specific risk for coronary artery (CA) sequelae among patients with complete Kawasaki Disease (KD).

  • We found that among patients with complete KD, those with conjunctival injection were more likely to develop CA sequelae than were those lacking it.

  • The absence of conjunctival injection—a relatively rare presentation in KD—is associated with a markedly lower cumulative incidence of CA sequelae.

  • This finding may help identify a distinct low-risk phenotype of KD and aid risk stratification.

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Fig. 1: Framework of the analysis.
Fig. 2: Prevalence of each principal clinical feature by age group.
Fig. 3: Presence of coronary artery sequelae across age groups: Comparison between patients exhibiting all six principal clinical features of Kawasaki disease and those lacking only one feature (exhibiting five features).

Data availability

Data are available from the Japan Kawasaki Disease Research Center for researchers who meet the criteria for access to confidential data.

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Acknowledgements

The authors thank all the pediatricians who contributed to the Japanese nationwide Kawasaki disease survey. We also extend our gratitude to the late Dr. Tomisaku Kawasaki and the late Dr. Itsuzo Shigematsu, as well as Dr. Hiroshi Yanagawa, Dr. Yosikazu Nakamura, and all others who have played leading roles in the nationwide survey of Kawasaki disease over the past several decades. Finally, we thank Angela Morben, DVM, ELS, from Edanz (https://jp.edanz.com/ac), for editing a draft of this manuscript.

Funding

RA acknowledges funding from grants and from a non-profit organization, the Japan Kawasaki Disease Research Center. The funder played no active role in the design or conduct of the study; collection, management, analysis, or interpretation of the data; preparation, review, or approval of the manuscript; or decision to submit the manuscript for publication.

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Authors and Affiliations

  1. Division of Public Health, Center for Community Medicine, Jichi Medical University, Shimotsuke, Tochigi, Japan

    Naoto Kato, Tohru Kobayashi, Masanari Kuwabara, Hiroya Masuda & Ryusuke Ae

  2. Yamagata Prefectural Shinjo Hospital, Shinjo, Yamagata, Japan

    Naoto Kato

  3. Center for Clinical Research and Advanced Medicine, Shiga University of Medical Science, Seta Tsukinowa-cho, Otsu, Shiga, Japan

    Jun Matsubayashi

  4. Department of Pediatrics, Shiga University of Medical Science, Seta Tsukinowa-cho, Otsu, Shiga, Japan

    Shinsuke Hoshino

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  1. Naoto Kato
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Contributions

N.K., J.M., S.H., T.K., M.K., H.M., and R.A. contributed to the study conception and design. Data acquisition and database management were performed by M.K. and R.A.; N.K., J.M., and R.A. contributed to the analysis. The first draft of the manuscript was written by N.K.; All authors contributed to the interpretation, revised the manuscript critically and approved the final version for submission. RA is the overall guarantor.

Corresponding author

Correspondence to Ryusuke Ae.

Ethics declarations

Competing interests

This study was supported by grants and funding from a non-profit organization, the Japan Kawasaki Disease Research Center. No other disclosures were reported.

Ethics approval & consent to participate

The study was approved by the Ethics Committee of Shiga University of Medical Science (Receipt ID: R2022-013) and the Jichi Medical University Clinical Research Ethics Committee (Receipt IDs: 23-130 and 24-083). These Committees approved the waiver of the requirement for informed consent.

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Kato, N., Matsubayashi, J., Hoshino, S. et al. Association between the absence of individual principal clinical features and coronary artery abnormalities in complete Kawasaki disease. Pediatr Res (2026). https://doi.org/10.1038/s41390-026-04770-6

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