Abstract

ObjectiveKawasaki disease (KD) is a systemic vasculitis in childhood that can lead to coronary artery lesions (CALs). Although early diagnosis and treatment is important for preventing KD patients from development of CALs, diagnosis depends on the clinical features of KD. We studied the usefulness of leucine-rich alpha-2-glycoprotein 1 (LRG1) and angiotensinogen (AGT), previously reported as KD-related proteins, for KD diagnosis and estimation of intravenous immunoglobulin (IVIG) efficacy.MethodsWe undertook a prospective cohort study with patients having two or more KD symptoms in multiple centers in Japan, between July 2017 and February 2019.ResultsTwo hundred forty-two patients were included. In multivariable analysis, one unit increase in LRG1 was associated with higher odds of KD diagnosis (Odds ratio [OR] 1.02 [95% confidence interval (CI) 1.001–1.03]). Double-positivity for AGT (≥ 26 μg/mL) and LRG1 (≥ 123.5 μg/mL) was an independent biomarker for KD diagnosis in both the total cohort and the subgroup of patients with two to four KD symptoms (OR 5.01 [95% CI 1.86–13.50] and 3.71 [95% CI 1.23–11.16], respectively). There was no association between LRG1/AGT and IVIG efficacy.ConclusionDouble-positivity for LRG1 and AGT is an biomarker for KD diagnosis, especially useful in diagnosing incomplete KD from non-KD. Future studies with larger cohorts should seek to determine whether LRG1 and AGT are valuable as definitive data referred at the diagnosis of KD and for estimating the risk of CALs.

Highlights

  • Kawasaki disease (KD) is a systemic vasculitis in childhood that can lead to coronary artery lesions (CALs) [1,2,3]

  • We studied the usefulness of leucine-rich alpha-2-glycoprotein 1 (LRG1) and angiotensinogen (AGT), previously reported as KD-related proteins, for KD diagnosis and estimation of intravenous immunoglobulin (IVIG) efficacy

  • One unit increase in LRG1 was associated with higher odds of KD diagnosis (Odds ratio [OR] 1.02 [95% confidence interval (CI) 1.001–1.03])

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Summary

Introduction

Kawasaki disease (KD) is a systemic vasculitis in childhood that can lead to coronary artery lesions (CALs) [1,2,3]. Patients without five features but with supportive lab studies and no other diagnosis were considered for incomplete KD [5]. Incomplete KD leads to development of CALs as frequently as complete KD with five or more KD symptoms [6, 7]. Supplemental laboratory data such as leukocytosis with neutrophilia and immature forms, hypoalbuminemia, and hyponatremia are sometimes referred as “significant findings” in Japanese and the American Heart Association (AHA) KD guidelines [5, 8]. We performed a prospective cohort study to evaluate the usefulness of LRG1 and AGT for KD diagnosis and estimation for IVIG efficacy

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