Pathology of the Coronary Arteries and Myocardium in Kawasaki Disease

Kawasaki disease (KD) has replaced rheumatic fever as the most common cause of pediatric acquired heart disease across the globe. The acute illness, characterized by fever and associated mucocutaneous features, is associated with a coronary artery arteritis and myocarditis. The destruction of the arterial wall leads to aneurysm formation in 25% of untreated children. Myocardial inflammation accompanies the vasculitis, and the long-term consequences of this acute inflammation are still being defined. Our incomplete understanding of the pathology stems in part from the unknown etiology of this vasculitis. We review here the current understanding of the pathology of KD and the animal models used to elucidate KD pathogenesis and define new therapeutic targets. Improved imaging techniques and cell-free RNA studies are critically contributing to our understanding of KD pathology, but much remains to be learned before we gain more complete knowledge of this complex and important condition.