Letter to the Editor
Afebrile Kawasaki disease is not a benign form of the disease: Reply
Department of Microbiology and Immunology, Saitama Children’s Medical Center and Departments of Pediatrics and
Nephrology, Dokkyo Medical University Saitama Medical Center, Minamikoshigaya, Koshigaya, Saitama, Japan
I appreciate the letter to the Editor from Yamazaki-Nakashi-
mada et al.
concerning our report.
First, we thank Yamazaki-
Nakashimada et al. for highlighting our mistake. Our report was
not the ﬁrst report on afebrile Kawasaki disease (KD). We
would like to express our sincere apologies to Pediatrics Inter-
national and its readers for this oversight.
As Yamazaki-Nakashimada et al. indicated, there were ﬁve
previous articles on afebrile KD (Table 1).
Four of the ﬁve
patients had coronary artery (CA) dilatations. Of these four
patients, and our patients who had CA dilatation, four of the
six were ≤7 months of age. Five of the six patients were male.
These ﬁve previous reports were in English, and were
retrievable using PubMed. Although there were a few other
articles on afebrile KD, they were not available on Pubmed or
were not in English, and only one patient had CA dilatation.
Because the diagnosis of KD is based on clinical symptoms,
the diagnosis of KD in patients without CA dilatation is more
difﬁcult than that in those with CA dilatation. KD cannot be
diagnosed deﬁnitively if the patient does not have both fever
and CA dilatation.
In contrast, if the patient has CA dilatation but does not have
fever, we must include the following six diseases in the differ-
ential diagnosis, as described in our article: KD, chronic active
Epstein–Barr virus infection, Yersinia pseudotuberculosis infec-
tion, systemic juvenile idiopathic arthritis, Takayasu disease,
and juvenile polyarteritis nodosa.
Regrettably, the ﬁve previ-
ously published articles did not describe this diagnostic process.
Moreover, only Hinze et al. reported the method of body
temperature (BT) measurement.
Furthermore, if there was a
description, it was reported only as “axillary”. Because there
is a report that described axillary BT measurement in KD is
not so accurate
, we measured BT carefully and frequently, to
conﬁrm that our patients were afebrile.
Nevertheless, I believe that most of the previously reported
must have had KD. I also believe that there must
be more undiagnosed patients who have KD without fever.
This may include some cases of sudden death in infancy. As
Yamazaki-Nakashimada et al. noted in their letter, we must
educate pediatricians about the existence of incomplete afeb-
rile KD and accumulate data to facilitate accurate diagnosis.
Table 1 Previous reports of afebrile Kawasaki disease
M + 2 weeks 4 + rhinorrhea,
M + 15 days 2 +
NA NA 4.1/11
M + No
2 + rhinorrhea,
NA “Discreet” NA/15370/
M + NA 0 NA Good 1.6/21300/
F – No
5 NA “Acutely
ASO, anti-streptolysin O antibody; BCG, bacillus Calmette–Guerin; BT, body temperature; CA, coronary artery; CRP, C-reactive pro-
tein; Hb, hemoglobin; Plt, platelets; NA, not available; WBC, white blood cells.
Correspondence: Atsunori Yoshino, MD PhD, Department of Nephrology, Dokkyo Medical University Saitama Medical Center (Former
Dokkyo University School of Medicine, Koshigaya Hospital), 2-1-50 Minamikoshigaya, Koshigaya, Saitama 343-8555, Japan.
Received 23 July 2017; revised 15 November 2017; accepted 15 December 2017.
© 2018 Japan Pediatric Society
Pediatrics International (2018) 60, 204–205 doi: 10.1111/ped.13484