Interleukin-1 (IL-1) plays a key role in the pathogenesis of Kawasaki disease (KD), especially in the development of coronary artery aneurysms.

Based on current evidence, derived from case reports and one open-label, phase II study, the IL-1 inhibitor anakinra is a fast-acting, effective, and reasonably safe drug.

Anakinra may be considered as an option to treat KD resistant to a first intravenous immunoglobulin (IVIG) dose, especially in the presence of severe coronary involvement.