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Answers regarding the precise aetiology and optimal treatments for Kawasaki disease (KD) remain frustratingly elusive. The medium vessel vasculitis with a predilection for coronary arteries continues to be the leading cause of acquired heart disease in developed countries and is being increasingly recognised in less resourced areas. While the cause remains unclear, epidemiological data suggest an infectious trigger for genetically susceptible individuals. Contemporary treatments have drastically improved outcomes, although a select group of patients continue to experience morbidity beyond the inflammatory phase of the condition.
Robust evidence supports the use of intravenous immunoglobulin (IVIG) and aspirin for reducing the risk of KD-related coronary artery aneurysms (CAA). The role of other adjunctive therapies, however, remains unclear despite over 40 years of observational and interventional studies exploring a range of treatment options. The use of primary corticosteroid treatment has been particularly controversial, although there has been a growing enthusiasm for this practice in select patients. Corticosteroids are a logical candidate therapy given their accessibility, low cost, broad immunosuppressive properties and demonstrated efficacy in other inflammatory conditions. Their side effect profile is very well understood and common adverse events fortunately tend to be mild and transient in children. Despite being the cornerstone for the management of other vasculitides, the evidence for corticosteroids in reducing the risk of persistent CAAs in KD is far from conclusive.
There has been some hesitancy to support the use of corticosteroids, largely owing to observational studies that have indicated poorer outcomes with …
Contributors WDR and AVR equally contributed to the conception, development, writing and editing of this manuscript.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent for publication Not required.
Provenance and peer review Commissioned; externally peer reviewed.
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