Kawasaki disease: the mystery continues

Kawasaki disease (KD) is an acute vasculitis of infancy and early childhood that continues to baffle researchers and clinicians alike. Although the acute illness resolves spontaneously, permanent damage to the coronary arteries occurs in 20-25% of untreated children. The cause of KD remains unknown and there is no specific laboratory test to identify affected children. Nonetheless, high dose intravenous g-globulin plus aspirin administered within the first 10 days of fever significantly reduces the risk of coronary artery damage by unknown mechanisms. KD thus presents a unique dilemma: the disease may be difficult to recognize, there is no diagnostic laboratory test, there is an extremely effective therapy, and there is a 25% chance of serious cardiovascular damage or death if the therapy is not administered. This review will highlight some of the many unanswered questions about KD.