The Epidemiology and Management of Kawasaki Disease in Australia

Abstract

Kawasaki disease (KD) is a syndrome of systemic inflammation with the potential to cause life-threatening aneurysms of the coronary arteries. I sought to contribute to our understanding of this important condition, particularly with regard to Australian children.

By determining the hospitalisation rate and IVIG-treatment rate I estimated the incidence of KD to be about 14 per 100,000 children under the age of 5 between 2007 and 2015. I also showed that the hospitalisation rate nationally had increased on average 3.5% annually between 1993 and 2018, with significant changes in the age distribution over that period.

In collaboration with the Paediatric Active Enhanced Disease Surveillance (PAEDS) network, I undertook a large multicentre prospective surveillance study of KD in Australia. My analysis of that cohort confirmed several of the findings from the survey, such as the preference of Australian clinicians for low-dose aspirin from the time of diagnosis, and the considerable variability around how IVIG resistance is diagnosed and managed. Importantly, I observed that a significant subset of children diagnosed with, and treated for, KD do not meet the diagnostic criteria outlined in the 2017 statement by the American Heart Association.

This work has contributed significantly to the understanding of KD’s epidemiology, management, and outcomes in Australia. I have shown that the incidence of the condition is increasing, and the clinical picture is changing. I identified important areas of practice variation and highlighted the need for international collaboration around agreed definitions (such as for IVIG resistance). Finally, I have played a central role in establishing an important resource for future resource: prospective surveillance of KD in Australia continues, with well over 700 cases recruited so far. It is hoped that this work will be of benefit to the researchers, clinicians, patients, and families affected by KD now, and into the future.