Abstract

Risk factors for coronary artery lesion (CAL) development in patients with Kawasaki disease (KD) include male sex, age < 12 months, intravenous immunoglobulin (IVIG) resistance, and delayed diagnosis.. We aimed to explore the relationship between CAL development and Z-score. We enrolled 281 patients with KD who were treated with our protocol. Echocardiography was performed in three phases: pre-treatment (P1), post-treatment (P2), and 4 weeks after onset (P3). The highest Z-score of the right, left main, left anterior descending, and left circumflex coronary arteries was expressed as Zmax at each phase. P3-Zmax ≥ 2.5 represented CAL development. Clinical parameters, such as laboratory data and Z-scores, were retrospectively compared between patients with and without CAL development. Sixty-seven patients (23.8%) showed a P1-Zmax ≥ 2.0, and CAL development occurred in 21 patients (7.5%). Independent risk factors associated with CAL development were P1-Zmax, a ΔZmax (P2-Zmax - P1-Zmax) ≥ 1, male sex, < 12 months of age, and resistant to the first intravenous immunoglobulin (IVIG) administration (adjusted odds ratio [95% confidence interval]: 198 [1.01-3.92], 4.04 [1.11-14.7], 6.62 [1.33-33.04], 4.71 [1.51-14.68], 5.26 [1.62-17.13], respectively). Using receiver operating characteristic curve analysis, a P1-Zmax ≥ 1.43 detected CAL development with an area under the curve of 0.64 (sensitivity = 81.0%; specificity = 48.1%).Conclusion: Our results suggest that P1-Zmax and a ΔZmax (P2-Zmax - P1-Zmax) ≥ 1 may predict CAL development. What is Known: • KD is an acute vasculitis predominantly affecting the coronary artery of young children. • Although P1 Z-max ≥ 2.0 has been a predictor of CAL development, it has not yet been shown in Japan. What is New: • P1-Zmax and a ΔZmax ≥ 1 are presumably associated with CAL development. • In the ROC curve analysis, P1-Zmax ≥ 1.43 detected CAL development, a sensitivity (81%) and a specificity (48%). We need to consider intensified initial therapy for patients with these risk factors.

Highlights

  • Kawasaki disease (KD) is an acute systemic vasculitis occurring in infants and children, especially those younger than 5 years of age [1]

  • Patients who fulfilled at least one of the following criteria were excluded: 1) no intravenous immunoglobulin (IVIG) treatment or not following our treatment protocol; 2) recurrent KD; 3) follow-up echocardiographic measurements not available; 4) patients who might have an effect on Z-scores, such as a high body mass index (≥25), a history of cardiovascular disease, systemic arterial hypertension, and other syndromes, such as Noonan/LEOPARD syndrome

  • In terms of treatment response, 177 patients responded to the first IVIG administration, 45 patients responded to additional IVIG administration, and 59 patients (21.0%) did not response to either initial or additional IVIG administration

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Summary

Introduction

Kawasaki disease (KD) is an acute systemic vasculitis occurring in infants and children, especially those younger than 5 years of age [1]. Risk factors for coronary artery lesion (CAL) development in patients with Kawasaki disease (KD) include male sex, age

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