Kawasaki disease (KD) is named after the Japanese pediatrician Tomisaku

Kawasaki who in 1967 described 50 cases of infants with persistent fever,

accompanied by rash, lymphadenopathy, edema, conjunctival injection,
redness and cracking of the lips, "strawberry tongue," and convalescent
desquamation. Today KD is understood as 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.
The incidence is highest in Japan with an annual rate of 130-140/100,000
children under 5 years of age. In comparison, incidence for the continental
U.S. varies between 9 and 20/100,000 children under 5 years of age and for
Japanese Americans living in Hawaii between 120 and130/100,000 in
children under 5 years of age. Because its etiologic agent(s) and patho-
physiological mechanisms remain unknown, and because there is no
diagnostic laboratory test for KD, diagnosis relies on the observation and
recognition of clinical signs that comprise the KD case definition. With the
establishment of intravenous immunoglobulin (IVIG) as an effective therapy,
prompt diagnosis has become essential for timely therapy to ensure a good
cardiac outcome.
Although researchers have attempted to uncover the etiology of KD since the
1960s, we appear to be no closer to an answer. Among those who assume
there is an infectious agent, disagreement continues over whether the agent
is bacterial or viral and whether or not it acts as a super-antigen. Immune
response remains a crucial arena of investigation; yet no robust hypothesis
has convincingly linked the sign complex and immune cascade with the
development of CAA.
In an effort to widen research questions related to the etiology of KD, the
Kawasaki Disease Research Program at the University of California, San
Diego (UCSD) School of Medicine initiated a medical historical investigation
of KD in the spring of 1998. We hoped that such an investigation might
uncover useful clues in the search for an etiology of Kawasaki disease.
The team (see below) collected a variety of data including historical records
of clinical cases and autopsy reports. Because KD was only recently
recognized, first-hand accounts are available from those involved in its
"discovery." We conducted a series of KD-focused interviews with
pediatricians, pathologists, and medical researchers from Japan, North
America, and Europe. Topics covered in these open-ended interviews
included a recollection of the person's earliest clinical observations, their
histories of research into the characteristics and the etiology of the
syndrome, and their professional conversations about it. To gain a context
for the interview data, we also observed practicing physicians, including
members of our team, as they considered and eliminated KD and possible
alternative diagnoses. In addition, the observational data were triangulated
with our historical understanding derived from the review of existing written
sources.
In 2003, the team, along with the Kawasaki Disease Foundation, a national
parents group led by Greg Chin in Massachusetts, applied for and was
awarded a three-year National Library of Medicine Grant (National Institutes
of Health) to develop a website and produce a film on the history of
Kawasaki Disease. This website will include transcriptions and films of these
interviews; PowerPoint presentations of our preliminary findings, as well as
our publications to date.