Abstract

An acute phase treatment for prevention of coronary artery stenosis caused by Kawasaki disease (KD) has not been established. The objective of this study was to clarify the medium-term outcomes of patients who received acute phase treatment in our department. This retrospective study included data from 214 patients with KD who received acute phase treatment from January 2009 to May 2020. A total of 196 (92.1%) received an initial single dose of intravenous immunoglobulin (IVIG) therapy. One patient with status epilepticus at presentation received initial IVIG plus steroid therapy. A total of 17 patients did not receive IVIG. The rate of coronary artery lesions (CALs) 1 month and 1 year after KD onset were 1.9% and 0.9%, respectively. Two patients had CAL 1 year after KD onset. However, no patients had coronary artery stenosis. One patient with a right giant CAL had a medium CAL before initial therapy. During a median follow-up period of 3 years and 4 months, no patients had cardiac events that required therapy.

Highlights

  • Kawasaki disease (KD) is an acute systemic vasculitis of unknown cause that mostly affects infants and children [1]

  • The main findings of this study were that the medium-term outcomes of patients with KD who received acute phase treatment in our department were favorable and that the rate of Coronary artery lesions (CALs) and coronary artery stenosis 1 year after onset were 0.9% and 0.0%, respectively

  • An initial single dose of intravenous immunoglobulin (IVIG) therapy (2 g/kg/dose) with delayed use of aspirin (DUA) for patients without complications before initial treatment was useful for the prevention of coronary artery stenosis

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Summary

Introduction

Kawasaki disease (KD) is an acute systemic vasculitis of unknown cause that mostly affects infants and children [1]. An acute phase treatment to prevent coronary artery stenosis has not been established. Studies regarding acute phase treatment for KD have been published [2]–[5]. The standard therapy for the acute KD phase includes intravenous immunoglobulin (IVIG) therapy at 2 g/kg/dose with concomitant medium-dose or high-dose aspirin [7]. Studies suggest that aspirin may inhibit CAL prevention and that delayed use of aspirin (DUA) may be beneficial for the prevention of coronary artery stenosis in KD [10]–[13]. This study aimed to clarify the medium-term outcomes of patients who received acute phase treatment in our department and ascertain the usefulness of an initial single dose of IVIG therapy (2 g/kg/dose) with DUA to prevent coronary artery stenosis in patients without CALs before initial treatment

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