Kawasaki disease is an acute vasculitis of childhood affecting small and medium arteries, with coronary artery aneurysms the principal long-term threat. Timely treatment to shorten fever and prevent coronary lesions is the central goal.
Standard of care
Intravenous immunoglobulin (2 g/kg as a single infusion), ideally within 10 days of fever onset, remains first-line across all guidelines and reduces both fever duration and coronary artery lesions. Aspirin is used alongside — higher or moderate doses in the acute phase, then low-dose for its antiplatelet effect during convalescence.
Adjunctive and refractory therapy
For high-risk or IVIG-resistant patients, several additions improve outcomes. Infliximab (anti-TNF) as adjunctive initial therapy reduces fever duration and coronary lesions versus IVIG alone and promotes vascular healing in immunoglobulin-resistant disease. Corticosteroids added to IVIG reduce coronary lesions and fever more than IVIG alone in high-risk patients. Cyclosporine helps in multi-refractory disease with ongoing fever despite IVIG and steroids. Anakinra (IL-1 receptor antagonist) and other anti-cytokine biologics are options for refractory disease, though their coronary benefit over IVIG is not yet established.
Recent trends
Since 2015, and especially in the post-COVID-19 era, guidelines and practice have increasingly incorporated steroids and biologics as adjuncts, particularly in severe cases; the pandemic also brought a rise in incomplete Kawasaki presentations and altered management patterns.
| Therapy | Indication | Key effect |
|---|---|---|
| IVIG + aspirin | All acute KD | Reduces fever and coronary lesions; most effective initial therapy |
| IVIG + infliximab | High-risk / refractory | Better fever control, fewer coronary lesions |
| IVIG + corticosteroids | High-risk / refractory | Further reduces fever and coronary lesions |
| IVIG + cyclosporine | Multi-refractory | Effective for hard-to-treat disease |
| Anakinra / anti-cytokine | Refractory | Fever control; coronary benefit unclear |
Further reading: 2021 ACR/Vasculitis Foundation Kawasaki disease guideline; network meta-analysis of KD pharmacotherapy (EBioMedicine 2022); Cochrane review of corticosteroids in KD (2022).