Acute-phase Treatment and Prevention of Large Coronary Artery Lesions in Kawasaki Disease

Kawasaki disease (KD) is a form of acute febrile systemic vasculitis that primarily affects children younger than 5 years. Coronary artery lesions (CALs) are a severe complication of KD. However, an acute-phase treatment for prevention of large CALs and coronary artery stenosis caused by KD has not been established. In this book, I have reviewed findings of clinical trials conducted in our department regarding acute-phase treatment and prevention of large CALs in KD.

The mainstay of the current standard therapy for acute-phase KD is intravenous immunoglobulin (IVIG) therapy at 2 g/kg with the concomitant use of medium-/high-dose aspirin. However, the efficacy of combining medium- or high-dose aspirin with IVIG therapy at 2 g/kg has not been fully investigated. Some studies suggested that aspirin may inhibit CAL prevention in IVIG therapy and that the delayed use of aspirin (DUA) for IVIG therapy may be beneficial for the suppression of CALs and prevention of coronary artery stenosis in patients with KD. Several factors are associated with CAL complications. Single IVIG therapy does not modify the clinical course of KD, and this characteristic allows clinicians to manage treatment progress and to provide rescue therapies for IVIG resistance and KD relapse at appropriate times. The majority of KD patients had favorable medium-term outcomes with acute-phase management using risk stratification before and after initial treatment, initial single IVIG therapy (2 g/kg/dose) with DUA, and appropriate rescue therapies using IVIG and plasma exchange. Moreover, an initial single dose of IVIG therapy at 2 g/kg with DUA had benefits regarding prevention of coronary artery stenosis, low rates of KD relapse, cost-effectiveness, and protection against severe infections.