TNF and IL-1 Targeted Treatment in Kawasaki Disease

Abstract

Kawasaki disease (KD) is a multisystemic vasculitis affecting young children and the most common cause of acquired heart disease in children in the developed world. Current treatment recommendations for acute KD include IVIG and aspirin, but there are no evidence-based guidelines for children who do not respond to IVIG treatment. Widely applicable risk stratification algorithms to identify patients at high-risk of treatment failure and poor coronary artery outcome are not available. Over the past few years, increasing knowledge of the pathophysiology of disease have resulted in the identification of key inflammatory mediators and the use of biologic pathway targeting agents such as TNF and IL-1 inhibitors for children with IVIG-resistant disease. However, despite considerable efforts, adequately powered, randomized, controlled and prospective trials are lacking. In this review, we summarize the recent advances in our understanding of disease pathobiology and provide an overview of the currently available studies on anti-TNF and IL-1 therapy in KD.