The narratives of Kawasaki disease

Kawasaki disease is a rash/fever illness of early childhood in which coronary artery aneurysms (CAA), sometimes fatal, may develop in up to 25 percent of untreated children. Because its etiology and pathophysiology are unknown and no diagnostic laboratory test exists, diagnosis is made via a list of clinical signs; however, a significant number of children fail to meet the clinical criteria and go on to develop CAA. We suspected a connection between these missed cases and the continuing difficulty in identifying the etiological agent(s) and mechanisms for CAA. In search of that connection, we launched a historical investigation into the institutionalization of the clinical criteria, and explored how this process influenced the framing of research questions. Our findings suggest that the canonization of the Kawasaki disease case definition was as much due to the enshrinement of the historical narrative as to compelling scientific findings. The Kawasaki disease narrative encompasses interrelated issues of definition, discovery, and naming; these, in turn, have profoundly influenced diagnosis, treatment, and research. "Atypical" cases, despite being at risk for CAA, often fail to receive prompt diagnosis and treatment; consequently, research has been limited to the population that meets the diagnostic criteria for Kawasaki disease, rather than including those who are at risk of CAA. Although clinical concerns prompted this investigation, it nevertheless has important implications for the history of medicine: it provides an illustration of how a historical interrogation of a syndrome's construction can free medical researchers to pursue novel approaches. Equally important, it demonstrates how historians can make unique contributions as collaborators in clinical care and medical research.