Kawasaki Disease Management and Coronary Artery Abnormalities

Kawasaki disease is an acute, self-limited vasculitis that predominantly affects children under five years of age and carries a risk of coronary artery abnormalities ranging from transient dilatation to giant aneurysms. Prompt recognition and treatment with high-dose intravenous immunoglobulin (IVIG) and aspirin within the first ten days of fever onset substantially reduce the incidence of coronary complications. Despite this standard therapy, approximately 10–20% of patients exhibit IVIG resistance and require adjunctive anti-inflammatory agents such as corticosteroids, tumour necrosis factor inhibitors or interleukin-1 blockers. Serial echocardiography, utilising coronary artery dimensions and body-surface-area-adjusted z-scores, is essential for risk stratification and long-term surveillance. Emerging evidence supports tailored approaches guided by early biomarkers and genetic risk profiles to optimise treatment intensity. In affected children, persistent aneurysms may lead to thrombosis, stenosis or myocardial ischaemia, underscoring the importance of a multidisciplinary framework encompassing paediatric cardiology, rheumatology and primary care to ensure seamless transition to adult services.

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Kawasaki Disease Management and Coronary Artery Abnormalities publication trend

The graph below shows the total number of articles in kawasaki disease management and coronary artery abnormalities across all publications each year (not limited to Nature Index journals).

Technical terms

Intravenous immunoglobulin (IVIG): A preparation of pooled immunoglobulins administered to modulate immune response and reduce vascular inflammation.

Coronary artery aneurysm (CAA): A focal dilation of the coronary artery wall exceeding 1.5 times the adjacent normal segment, graded by z-score.

Z-score: A statistical measure normalising arterial diameter to body surface area for accurate assessment of dilatation.

Platelet-monocyte aggregates (MPAs): Complexes formed by activated platelets and monocytes that drive inflammatory cascades in vascular endothelium.

PDGFRβ: Platelet-derived growth factor receptor beta, a tyrosine kinase involved in endothelial cell signalling and vascular remodelling.

Mitophagy: The selective autophagic removal of damaged mitochondria, critical for maintaining endothelial cell homeostasis under stress.

References

1. Platelet–Monocyte Aggregate Instigates Inflammation and Vasculopathy in Kawasaki Disease. Advanced Science (2024).
2. Inhibition of PDGFRβ alleviates endothelial cell apoptotic injury caused by DRP-1 overexpression and mitochondria fusion failure after mitophagy. Cell Death & Disease (2023).
3. Coronary Artery Aneurysms in Kawasaki Disease: Risk Factors for Progressive Disease and Adverse Cardiac Events in the US Population. Journal of the American Heart Association (2016).
4. Epidemiology of Kawasaki Disease in Asia, Europe, and the United States. Journal of Epidemiology (2012).
5. Global gene expression profiling identifies new therapeutic targets in acute Kawasaki disease. Genome Medicine (2014).
6. Dissecting Kawasaki disease: a state-of-the-art review. European Journal of Pediatrics (2017).