Original Studies
Diagnostic and Treatment Trends in Children With Kawasaki
Disease in the United States, 2006–2015
Samuel R. Dominguez, MD, PhD,*† Meghan Birkholz, MSPH,† Marsha S. Anderson, MD,*†
Heather Heizer, PA-C,*† Pei-Ni Jone, MD,*‡ Mary P. Glode, MD,*† and James K. Todd, MD*†
Objective: To evaluate variations in treatment practice and compliance with
national guidelines for the diagnostic evaluation of children with Kawasaki
disease (KD).
Study Design: We used the Pediatric Hospital Information System database
to analyze demographic, laboratory and treatment data from patients admit-
ted with KD between January 1, 2006, and December 31, 2015.
Results: During the study period, 12,089 children with KD were diagnosed.
Nearly all patients had a complete blood cell count, erythrocyte sedimen-
tation rate, and C-reactive protein ordered. Fewer patients had alanine
aminotransferase (48.6%) or a urinalysis (75.3%). A small percentage of
children had abdominal imaging (11.5%), neck imaging (5.9%), and lum-
bar punctures (4.5%), and 36.0% of patients received antibiotic therapy.
Obtaining echocardiograms pretreatment and the use of steroids and inflixi-
mab significantly increased over the study period (P < 0.001). For patients
who failed initial intravenous immunoglobulin (IVIG) monotherapy, 82.0%
received a second dose of IVIG, 7.7% received steroids, 6.5% received inf-
liximab, and 3.9% received combination therapy. Patients receiving inflixi-
mab or steroids as second therapy had a higher response rate than those who
received only a second IVIG dose (87.9% versus 83.0% versus 73.3%, P
< 0.001).
Conclusions: KD remains a challenging diagnosis. Opportunities exist for
earlier use of echocardiograms in the evaluation of children with potential
KD. Significant variations in practice exist surrounding second-line therapy.
Our data suggest superiority of second-line therapy use of infliximab or
steroids over IVIG in terms of reducing need for additional therapies. Pro-
spective, controlled studies are needed to confirm this finding.
Key Words: demography, blood cell count, urinalysis, infliximab, fever
(Pediatr Infect Dis J 2019;38:1010–1014)
K
awasaki disease (KD) is an acute, self-limited vasculitis of
childhood. Due to its potential to cause coronary artery aneu-
rysms, KD has become the leading cause of acquired heart dis-
ease in children in the developed world.1 KD remains a challeng-
ing clinical diagnosis because there is no definitive diagnostic test.
Children with KD present in a variety of ways, and KD mimics
other common febrile bacterial and viral infections of childhood.
The most recent American Heart Association (AHA) state-
ment regarding diagnosis, management and long-term follow-up of
patients with KD was issued in 2017.2 Since the previous publica-
tion in 2004,3 several papers have been published exploring new
adjunctive, initial therapies for high-risk children as well as new
Accepted for publication June 25, 2019.
From the *Department of Pediatrics, University of Colorado School of Medicine;
†Children’s Hospital Colorado, Section of Infectious Diseases and Epidemi-
ology; and ‡Children’s Hospital Colorado, Heart Institute.
The authors have no funding or conflicts of interest to disclose.
Address for correspondence: Samuel R. Dominguez, MD, PhD, Children’s Hos-
pital Colorado, 13123 E, 16th Ave, B055 Aurora, CO 80238. Email samuel.
dominguez@childrenscolorado.org
Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved.
ISSN: 0891-3668/19/3810-1010
DOI: 10.1097/INF.0000000000002422
1010 | www.pidj.com The Pediatric Infectious Disease Journal  •  Volume 38, Number 10, October 2019
Copyright © 2019 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited.
second-line therapies for children who are intravenous immuno-
globulin (IVIG) resistant.4–11 Although treatment with IVIG and
aspirin is standard primary therapy, there is no consensus on opti-
mal therapy for children who are IVIG-resistant and require addi-
tional therapy. Furthermore, controversy exists regarding whether
patients who are at high-risk for developing coronary artery lesions
(CALs) might benefit from more aggressive initial therapy and
what this therapy should be. Similarly, few data exists regarding
compliance with national guidelines for the diagnostic evaluation
of children with KD.
Forty-nine freestanding, pediatric hospitals in the United
States contribute patient information to the Pediatric Health Infor-
mation System (PHIS). Anonymous patient identifiers allow iden-
tification of demographics, medications used, and laboratory and
radiographic tests ordered for individual patients during hospitali-
zation for a given diagnosis. A previous study utilizing the PHIS
database demonstrated that from 2001 to 2006 there was a steady
increase in the number of patients with KD admitted and a small,
but steady, increase in the use of infliximab.12 We used the PHIS
database to analyze data from patients admitted with their first
diagnosis of KD between January 1, 2006, and December 31, 2015.
Our goals were to describe changes in hospital admissions, demo-
graphics, diagnostic evaluations, and treatment of patients with
acute KD to identify areas of variation in practice and potential
areas for further research.
METHODS
Data for this study were obtained from the PHIS, an admin-
istrative database affiliated with the Children’s Hospital Associa-
tion (Overland Park, KS). The PHIS hospitals are 49 of the largest
and most advanced children’s hospitals in America and constitute
the most demanding standards of pediatric service in America. The
PHIS database contains diagnosis and procedure codes, and billed
utilization data of inpatient encounters but does not provide any
data regarding specific clinical symptoms or testing results. The
Children’s Hospital Association partners with the PHIS hospitals to
ensure data quality and validity.
The study population comprised inpatients 0–18 years of
age, discharged between January 1, 2006, and December 31, 2015,
from the 33 PHIS hospitals with complete information available
for that time period. De-identified data covering demographics,
diagnoses, laboratory and radiographic testing and medications
were obtained. Patients included had either an International Clas-
sification of Diseases, 9th revision, Clinical Modification code of
446.1 or an International Classification of Diseases, 10th revision,
Clinical Modification code of M30.3 as well as at least one dose
of IVIG.
Ethnicity and race in patients with KD were compared with
the entire population of patients admitted to the hospitals. Race data
for the year 2006 were excluded from the analysis due to a change
in the coding structure that occurred in 2007. Second therapy was
defined as receipt of medication greater than or equal to 1 day after
receipt of IVIG and included therapies received during the initial
visit or in a readmission within 7 days of discharge. “Pretreatment
The Pediatric Infectious Disease Journal  •  Volume 38, Number 10, October 2019 Kawasaki Disease Trends in the US
echocardiogram (ECHO)” was defined as receipt of ECHO at least
one day before or the same day as IVIG. “Posttreatment ECHO”
was defined as ECHO obtained one day or more after receipt of
IVIG. Therapies analyzed included IVIG, infliximab and steroids.
Steroid therapy included receipt of dexamethasone, methylpredni-
solone, prednisolone or prednisone.
All study procedures were approved by the Colorado Multi-
ple Institutional Review Board and were performed in compliance
with the PHIS External Data Release Guidelines. χ2testing was used
for comparison of demographic characteristics and treatment out-
comes. Difference in trends over time were compared using linear
regression. A P value of less than or equal to 0.05 was considered
significant for all analyses. Data analysis and statistical calculations
were performed using Tableau version 10.3 (Seattle, WA) and SAS
version 9.4 (Cary, NC).
RESULTS
During the 10-year study period, 12,089 children (0.3% of
all admissions) with KD were diagnosed, admitted to the hospital, and
treated. There was a significant yearly increase in the number of patients
diagnosed, ranging from 1084 in 2006 to 1322 in 2015 (P = 0.002). The
gender distribution of patients remained constant during the 10 years,
with approximately 60% of admitted patients with KD being male.
There was a slight increase in the median age of diagnosis from 2.4
(1.2–4.3) to 2.8 (1.3–5.2) years (P = 0.007), reflecting an increase in
the percentage of patients with KD in the 5–9 years (16.1%–21.0%)
and greater than 10 years of age groups (2.1%–4.9%). There was a
significant overrepresentation in the KD population of Asian children
compared with the overall pediatric hospital admission population
(9.3% versus 2.8%, P < 0.001). KD admissions displayed seasonal
variation, with a peak in the winter and early spring months from
December to April, with the highest average number of admission per
year being in the months of January and March.
The percent of patients who received various laboratory and
radiographic diagnostic evaluations is shown in Table 1. With the
exception of group A streptococcal testing (which decreased from
41.5%–28.1%, P < 0.001) and urinalysis (which increased from
69.2%–79.1%, P < 0.001, data not shown), the diagnostic evalua-
tions performed in children diagnosed with KD remained constant
during the 10 years of the study. Nearly all patients had a complete
blood cell count, erythrocyte sedimentation rate (ESR) and C-reac-
tive protein (CRP). Only 48.6% of patients had an alanine ami-
notransferase (ALT) and a full liver function panel, and 32.2% had
a gamma-glutamyl transferase ordered during their admission. A
small percentage of children had abdominal imaging (11.5%), neck
imaging (5.9%) and lumbar punctures (4.5%) as part of their diag-
nostic evaluation. The percentage of children who had a pretreat-
ment ECHO obtained increased significantly from 57.3%–66.2%
(P < 0.001).
All patients received at least IVIG monotherapy as initial
therapy. A small subset of patients received intensified initial ther-
apy with either the addition of steroids (3.1%) or infliximab (0.5%)
to IVIG (Tables 2). The use of steroids (6.4%–12.1%, P = 0.003)
and infliximab (1.2%–6.5%, P = 0.006) significantly increased
over the 10-year period, with a sharp increase in use beginning in
2012 (Figure 1). The percent of patients who received steroids and
infliximab varied by hospital, with a range of 1.1%–37.1% and
0.2%–21.8%, respectively. The median interquartile range (IQR)
percent of patients who received steroids by hospital was 6.8%
(4.8%–9.1%), and there were 5 hospitals where greater than 10%
of patients with KD received steroids. The median (IQR) percent of
patients who received infliximab by hospital was 1.3% (0.6–2.2%),
and there were 2 hospitals where greater than 10% of patients with
KD received infliximab.
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TABLE 1.  Laboratory and Radiographic Evaluations
and Outcomes of Children Admitted With a Diagnosis
of KD
Test N=12,089 (%)
Blood cultures 7011 (58%)
Group A streptococcal testing 4485 (37.1%)
CRP 11,136 (92.1%)
ESR 10,753 (88.9%)
CBC 11,206 (92.7%)
ALT and LFT panel 5879 (48.6%)
GGT 3898 (32.2%)
Urinalysis 9115 (75.4%)
Abdominal imaging 1394 (11.5%)
Neck imaging 709 (5.9%)
Lumbar puncture 549 (4.5%)
Outcome
Received antibiotics 4350 (36%)
Admitted to PICU 806 (6.7%)
Median (IQR) length of stay 3 (2–5) d
ALT indicates alanine aminotransferase; CBC, complete blood count; CRP, C-reac-
tive protein; ESR, erythrocyte sedimentation rate; GGT, gamma-glutamyl transferase;
IQR, interquartile range; LFT, liver function test; PICU, pediatric intensive care unit.
23.2% of patients received a second therapy, presumably due
to a failure of symptoms to resolve. There was variation in choice of
a second-line therapy after initial IVIG monotherapy, with 82.0%
receiving the second dose of IVIG, 7.7% receiving steroids, 6.5%
receiving infliximab and 3.9% receiving some combination of
these therapies (Table 3). Overall, 75.3% of patients who received
a second-line therapy did not go on to receive additional therapies.
Patients receiving only infliximab or steroids as second therapy
were significantly less likely to need additional therapies than those
patients receiving IVIG alone as a second therapy (12.1% in the
infliximab group, 17% in the steroid group, versus 26.7% in the
IVIG alone group P < 0.001, Tables 3). Other less commonly used
third and fourth line therapies included cyclosporine (30 patients),
methotrexate (13 patients), anakinra (10 patients), etanercept (6
patients) and tacrolimus (2 patients).
90.5% of patients received aspirin as part of their treatment.
2.8% received additional anticoagulant therapies including clopi-
dogrel (1.0%), enoxaparin (1.1%), warfarin (0.5%) and alteplase
(0.9%). One patient received argatroban.
The median length of stay for admitted patients with KD
was 3 (IQR, 2–5) days. 36.0% of patients received antibiotics.
There was variation in antibiotic use between hospitals, ranging
from 18.5%–50.9% of patients receiving antibiotics with a median
of 38.7% (IQR, 33.1%–43.5%). Overall, 6.6% of patients required
admission to the intensive care unit (ICU) with 1.8% of patients
receiving dopamine. The rates of antibiotic use and admission to
the ICU remained constant over the 10-year period.
DISCUSSION
Utilizing primarily hospital discharge data, epidemiology
studies of KD from 1990–2010 in the United States have shown
a relatively stable incidence with estimates ranging from 17–25
per 100,000 children less than 5 years of age.13–17 A more recent
study, however, found a slight decrease in US KD hospitalization
rates from 2003–2012.18 This is in contrast to other parts of the
world, particularly Japan, where there has been a steady increase
in the incidence of KD over the past 4 decades.17,19 Using a simi-
lar methodology to ours, however, Son et al12 reported an increase
in the number of KD admission to US pediatric hospitals from
2001–2006. We found a similar trend, with a significant increase in
hospital admissions for KD from 2006–2015. Because of the nature
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Dominguez et al The Pediatric Infectious Disease Journal  •  Volume 38, Number 10, October 2019

TABLE 2.  First-Line Therapies for Treatment of Patients With KD and Need for
Additional Therapies

First % # %
Therapy N = 12,089 of Total Responded Responded P (χ2)

IVIG 11,652 96.4% 8961 76.9% P = 0.40

IVIG + Steroids 373 3.1% 277 74.3%
IVIG + Infliximab 60 0.5% 48 80.0%
Other 4 0.0% 4 100.0%
TOTAL 12,089 100.0% 9286 76.8%

TABLE 3.  Second-Line Therapies for Treatment of Patients With KD and Need for
Additional Therapies

Second Therapy % # %
After Initial IVIG N = 2691 of Total Responded Responded P (χ2)

IVIG 2206 82.0% 1618 73.3% P < 0.001

Steroids 206 7.7% 171 83.0%
Infliximab 174 6.5% 153 87.9%
Other/combination therapy 105 3.9% 84 80.0%
TOTAL 2691 100.0% 2026 75.3%

of our data set, however, we are not able to calculate nationwide
incidence rates. As previously reported, we found that KD admis-
sions were more common during the winter and early spring, with
peak incidence in the months of January and March. Similarly, we
found a higher incidence in males and an overrepresentation in
Asians.2,15,16,20 We also found a slight increase in the median age of
diagnosis over the decade of our study. This increase was largely
due to an increase in the number of patients diagnosed who were
>5 years of age. This is an interesting finding as it may represent a
true increased incidence in this age group suggesting delayed sus-
ceptibility to disease, or may reflect increased provider recognition
that KD can occur in older children.
In the absence of a diagnostic test, KD remains a clinical
diagnosis with supporting laboratory findings. Diagnosis is made
by the presence of fever plus 4 of the 5 cardinal clinical features.
However, some children with suspected KD may have less than 4
of the 5 principal clinical features, and these children may be evalu-
ated for incomplete KD. An algorithm to aid clinicians in evalua-
tion of children with suspected incomplete KD has been recently
published as part of the AHA diagnosis and management of KD
expert consensus guideline.2 For these children, a diagnosis of KD
can be made with supporting laboratory findings and ECHO find-
ings. In particular, the AHA guideline recommends, in addition
to obtaining inflammatory markers (ESR and CRP), obtaining a
blood cell count (to evaluate for anemia, leukocytosis and throm-
bocytosis), albumin and ALT (to evaluate for hypoalbumenia and
an elevated ALT) and a urinalysis (to evaluate for sterile pyuria) as
adjunctive criteria for KD.2 While almost all patients in our data-
base obtained an ESR and CRP, only about 3-quarters had a uri-
nalysis and only about half had liver function tests obtained as part
of their diagnostic evaluation. While some of these patients may
have had these tests obtained prior to admission and so were not
reflected in our analysis or clinicians may have been confident in
the diagnosis of KD without these supporting tests, this likely rep-
resents an area needing improvement. Similarly, although ECHO
lacks sensitivity for diagnosing KD, the finding of CALs, defined
as a coronary artery z-score of ≥ 2.5 for the proximal left anterior
descending or right coronary artery branches, has high specificity
and can be used to help rule in a diagnosis of KD.21,22 ECHO find-
ings are also needed to diagnose the presence of severe disease to
guide antithrombotic therapy. Furthermore, it is now appreciated
that in the majority of patients who develop CALs, these lesions

FIGURE 1.  Use of steroids and infliximab in

children with KD over time.

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The Pediatric Infectious Disease Journal  •  Volume 38, Number 10, October 2019 Kawasaki Disease Trends in the US
are present at the time of diagnosis.23 Patients with CALs at diag-
nosis may represent a group of patients to consider for intensified
initial therapy24 to try to mitigate on-going damage to the coronary
arteries. For all these reasons, obtaining an ECHO early in the diag-
nostic evaluation of children with KD is valuable. Likely reflecting
these new findings and recommendations, there was an increase in
the use of ECHOs pretreatment.
KD remains a challenging diagnosis because several other
febrile illnesses share some of the principal clinical features seen
in KD. These include staphylococcal and streptococcal toxin-medi-
ated disease (conjunctivitis, strawberry tongue, rash, extremity
edema), bacterial cervical adenitis (enlarged cervical node), mea-
sles (rash, conjunctivitis, lymphadenopathy, pharyngeal erythema)
and drug hypersensitivity reactions (rash; sometimes conjunctivitis,
oral changes and edema). The choice of laboratory tests and medi-
cations for some patients ultimately diagnosed with KD seems to
reflect the diagnostic dilemma. A third of patients in this data set
had streptococcal testing performed (rapid strep antigen test, throat
culture or streptococcal antibodies titers), and half of the patients
had blood cultures sent as part of their diagnostic evaluation. Also,
in support of this, one-third of patients received antibiotics dur-
ing their admission for KD, and there was significant variation in
antibiotic use between hospitals. Interestingly, 11.5% of patients
had abdominal imaging reinforcing previous reports that KD can
have a primary gastrointestinal presentation which often confuses
and delays the diagnosis.25–29 Similarly, 5.9% of patients had neck
imaging emphasizing the clinical overlap between KD and cervical
lymphadenitis.30,31 Furthermore, a small but significant percentage
of patients required admission to the PICU likely reflecting those
children presenting with KD shock syndrome.32,33 Together these
data argue for continued education about the multitude of clinical
presentations seen in children with KD, underscore the diagnostic
challenges clinicians encounter, suggest a potential role for antibi-
otic stewardship, and highlight the urgent need for a diagnostic test.
Worldwide, IVIG remains the standard first-line therapy
for KD.2 In the decade we studied, the need for a second therapy
remained constant at about 23% of patients with KD. Interest-
ingly, the use of primary intensified initial therapy with the addition
of steroids or infliximab to IVIG has increased, likely reflecting a
desire to target high-risk patients with more aggressive therapy as
has been done successfully in more homogeneous Japanese popula-
tions.34 Indeed, the overall use of steroids and infliximab had a sharp
increase in 2012 coincident with the publication of the efficacy of
immunoglobulin plus prednisolone for prevention of coronary artery
abnormalities in severe Kawasaki Disease (RAISE) study in Japan
and several published articles reporting on the use of infliximab in
patients with KD patients in the United States.4,5,8,10,34
The optimal second-line therapy for children with KD who
are IVIG-resistant remains controversial. Our study demonstrates
this uncertainty by highlighting the existence of significant prac-
tice variation in second-line therapies. Of note, patients treated
with infliximab or steroids had a significantly lower rate of need
for additional therapies compared with those treated with a sec-
ond dose of IVIG. This is an important finding as it has previously
been noted that patients with KD who need additional therapies and
have prolonged inflammation are at higher risk for development of
CALs and aneurysms.35 Carefully conducted, prospective studies
are needed to verify this observation.
Our study has several limitations. Though the PHIS database
represents a very large pediatric population in the US, potential bias
toward sicker children or those living in proximity to a tertiary care
facility may exist. Because the data rely on discharge diagnoses in
de-identified patients, we are unable to verify if patients were cor-
rectly classified or documented by treating physicians. As the data
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Copyright © 2019 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited.
only reflect emergency room and inpatient visits, diagnostic evalu-
ations that occurred prior to admission or transfer to the included
hospitals could not be captured and, therefore, the percent of chil-
dren with particular testing might be underrepresented. Similarly,
transfers/readmissions to another facility could not be captured.
In addition, although our data support a difference in response to
therapy we do not have any data about CAL outcomes. Finally, as
only individual dates assigned to medication can be retrieved the
exact timing of medications and tests on individual days could not
be ascertained.
CONCLUSIONS
In summary, KD remains a challenging diagnosis with a
significant percentage of patients being evaluated or treated for
other illnesses before the diagnosis of KD. Opportunities exist for
increased standardized laboratory diagnostic testing and earlier use
of ECHOs in the evaluation of children with potential KD. Although
IVIG remains the standard first-line therapy, significant variations
in practice exist surrounding second-line therapy for IVIG-resistant
patients. Our data suggest superiority of use of infliximab or ster-
oids over IVIG as second-line therapy in terms of reducing the need
for additional therapies. Prospective, controlled studies are needed
to confirm this finding.
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