Page 96 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

72 David S. Younger

in Caucasians [70]. Before revision of the criteria for KD, the classification was based either
on Japanese [71] or the American Heart Association (AHA) classification [72].

The former criteria, employed in Japanese epidemiological studies of KD, required the
presence of five of the following six criteria: characteristic fever, bilateral conjunctivitis,
changes in lips and oral cavity, polymorphous exanthema, changes of peripheral extremities
and cervical lymphadenopathy, while those of the latter employed in American and Caucasian
studies, generally required fever plus four of the remaining five criteria.

Two recent modifications to the criteria for KD made by the European League Against
Rheumatism (EULAR)/Pediatric Rheumatology European Society (PReS) consensus criteria
conference [62], which may alter the carriage of epidemiologic studies in the future included
the addition of perineal desquamation describing changes in the extremities; moreover, fewer
than four of the remaining five criteria were deemed necessary in the presence of fever and
coronary arterial involvement demonstrated by echocardiography.

To emphasize pediatric vasculitis disease even prior to retrospective and prospective
epidemiologic studies, a half century of 1,335,045 postmortem examinations from the Annual
of Pathological Autopsy Cases in Japan from 1958 to 2008 identified 380 cases of vasculitis
in children, more than one-half of which were KD and other disease entities including
unclassified vasculitis, PAN, purpuric vasculitis, TAK and others. Moreover, the postmortem
findings for 24 of 125 childhood vasculitides performed before 1976 and diagnosed as non-
KD were later consistent with KD.

Epidemiology
The global incidence and prevalence of KD is shown in Table 5.
Saundankar and colleagues [73] identified hospitalized patients in Western Australia with

the diagnosis of KD noting a steady increase in the mean annual incidence from 7.96 between
1990 and 1999 to 9.34 per 100,000 children age < 5 between 2000 and 2009, with the peak
incidence of 15.7 per 100,000 in 2005.

Lin and colleagues [74] identified hospitalized discharges with the diagnosis of KD in
Ontario noting a mean annual incidence of 26.2 per 100,000 for < 5 year olds, and 6.7 per
100,000 for 5 to 9 year old children, and 0.9 per 100,000 for those 10 to 14 years old, that
steadily increased from 14.39 to 26.24 per 100,000 from 1995 to 2006. Ma and colleagues
[75] studied all children sent to one of 50 hospitals in Shanghai noting a mean annual
incidence of 46.32 per 100,000 children < 5 years of age that steadily increased from 36.78 to
53.28 between 2003 and 2007. Li and coworkers [76] identified case of KD < 5 years of age
among 212 hospitals in the Sichuan Province noting a steady increase in the incidence in the
children from 8.57 to 9.81 per 100,000, with an average incidence throughout the latest five
years of 7.06 per 100,000. Du and coworkers [77] conducted a hospital-based survey of KD
in 45 Beijing hospitals identifying 1107 KD patients with a mean annual incidence of 49.4
per 100,000 <5 year old children and a steady increase from 2000 to 2004 that varied from
40.9 to 55.1 per 100,000. Fischer and colleagues [78] performed a population-based hospital
study of KD children in Denmark from 1981 to 2004 identifying 360 children younger than
15 years and noting a mean annual incidence of 4.5 to 5 per 100,000 person-years with a
gradual increase over the study period. Holman and colleagues [79] conducted a retrospective
analysis of children age <18 years notably those <5 years hospitalized in Hawaiian hospitals
from 1996 to 2006 noting a mean annual incidence of 50.4 per 100,000 children < 5 years
ranging from 45.5 to 56.5. Japanese children who had the highest mean annual incidence of

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