Pediatrics & Therapeutics

Pediatrics & Therapeutics
Open Access

ISSN: 2161-0665

+44 1478 350008


Utility of TNF-α as a Biomarker and the Possibility of anti-TNF-α Therapy for Kawasaki Disease

Keiichi Hirono and Fukiko Ichida

Kawasaki disease (KD) is the most common systemic vasculitis syndrome, primarily affecting the coronary arteries. Timely treatment with high-dose intravenous immunoglobulin (IVIG) reduces the duration of fever and incidence of Coronary Artery Lesions (CAL). However, even after IVIG treatment ∼5%-7% of patients develop aneurysms.

TNF-α is a cytokine with multiple biological effects produced primarily by monocytes and macrophages. In the last decade, TNF-α has been the focus of research aimed at uncovering its role during the acute phase of KD. Previous studies reported that TNF-α is responsible for the increase in its soluble receptors and is involved in the pathogenesis of the clinical features and CAL in KD.

Anti-TNF-α therapies, such as infliximab or etanercept, seem to be effective in controlling inflammation in patients with KD who fail to respond to IVIG. Several trials of the usage of infliximab as the first, second, or third line therapy for KD since 2004 showed that infliximab was safe and well tolerated, and patients treated with infliximab had fewer days of fever. Etanercept was recently shown to be safe and well tolerated as adjunctive initial therapy with IVIG in a small study of children with KD.