2024年6月11日发(作者：)

Guidelines

Revision of diagnostic guidelines for Kawasaki disease (6th revised

edition)

Purpose and background of the revision

The diagnostic guidelines for Kawasaki disease (KD) were last revised in 2002

1

(5th revision). Major points of revision

included: (i) the definition of fever was defined as fever per-sisting 5 days or more (inclusive of cases in which the fever had

subsided before the fifth day in response to therapy) and (ii) to explicitly state that incomplete KD cases can have coro-nary

artery lesions.

After the 5th revised edition of the diagnostic guidelines for KD was published, the proportion of patients receiving

early treatment increased and the incidence of coronary artery lesions decreased nationwide. On the other hand, the number of

incomplete KD cases increased yearly from 10% to the cur-rent level, which is greater than 20% of all KD patients.

In recent years, a standard method for expressing the coro-nary artery internal diameter of Japanese children was estab-

lished

2

and allowed us to define coronary artery internal dimensions in terms of standard deviations from the mean, or Z

scores). Incorporation of Z scores to facilitate the diagnosis of incomplete KD was a motivating factor for this revision. In the 5th

edition, the section titled “Other significant symptoms or findings” was not changed; therefore, the description from the 4th

edition lasted more than 30 years and was due for an update. In 2017, we consulted with the Japan Kawasaki Disease Society Steering

Committee Members regarding the necessity for a revi-sion of the 5th revised edition, and 75% of the committee mem-bers agreed to

revise. The Japan Kawasaki Disease Research Center and study group for vasculitis funded by the Ministry of Health, Labor and

Welfare also agreed to the revision.

In this revision, the writing group members conducted dis-cussions from 2018 to 2019. The original draft was presented to the 38th

Annual Meeting of the Japanese Society of Kawa-saki Disease in Wakayama. The draft was revised again, based on the steering committee

members’ suggestions, and the final draft of the 6th revision was completed. In the future it will be interesting to evaluate the impact of these

revised guideli-nes on the diagnosis of KD in Japan.

The previous 5th edition1 was published as an article in Japan Today in 2005, and was titled “Diagnostic Guidelines.” The recent format of

the “guidelines” has changed and requires full supporting evidence; “diagnostic guidance” or “criteria with clinical findings” may be more

appropriate as the title for this revision because there is not enough evidence for the diag-nosis of this disease. However, as the previous title

has been familiar with most pediatricians and primary care physicians, we would prefer to use the same title with only the change of the

edition number from the fifth to the sixth revised edition. Additionally, because such clinicians use these guidelines as the diagnostic criteria,

it is desirable to be as concise as possi-ble and to be presented as a few brief sheets of 1 or 2 pages.

As we also believe more detailed explanations are neces-sary to describe each item, including many examination findings, an additional

“guidebook” will be written by the committee members for publication.

The major changes of the revision are described below.

Principal clinical features

Several changes were made to the six principal clinical fea-tures, which have been well understood and disseminated for

almost all clinicians in Japan (Tables 1–3 and Figure 1).

1. The requirement for a specific duration of fever was deleted. In Japan, more than 90% of KD patients received high

dose intravenous immunoglobulin (IVIG) in a single dose. Although most pediatricians or primary care physi-cians know that

the classic definition of KD required a duration of fever for more than 5 days, the 24th Nation-wide Surveillance reports

that approximately 9%, 25%, and 35% of KD patients received the first IVIG treatment on the 3rd, 4th, and 5th days of

illness, respectively, and the prevalence of coronary artery lesions (CALs) has been lower than before. As we expect a continuous

decrease in CALs, we modified the fever definition to reflect current practice.

Table 1 Principal clinical features

.

ral bulbar conjunctival injection.

s

of lips and oral cavity: reddening of lips, strawberry tongue,

diffuse injection of oral and pharyngeal mucosa.

(including redness at the site of Bacille Calmette-

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