Diagnostic approaches to Kawasaki disease worldwide: the results from the JIR-CliPS network

To evaluate worldwide differences of diagnostic approaches to Kawasaki disease (KD), focusing on aspects, which vary throughout major international guidelines, including criteria for diagnostic and risk stratification.

An online English-language survey was distributed to physicians worldwide between June 2022 and November 2024 through the JIR-CliPS (juvenile inflammatory rheumatism; clinical practice strategies) network, aiming to collect real-life diagnostic and management practices in juvenile rheumatic conditions, including KD. Responses addressing the definitions of complete and incomplete KD and criteria identifying patients at high-risk for coronary artery lesions (CALs), were analysed. Descriptive statistics, comparisons of categorical variables, sensitivity and weighted analysis were performed.

One hundred and ninety-two physicians from 51 countries completed the survey. All respondents accepted ≥5 days of fever plus four criteria as diagnostic for complete KD. Thirty-two percent (n = 62/192, 95% CI [25.74, 39.40]) also accepted shorter fever duration, particularly physicians from France (n = 16/24, 63%, 95% CI [44.68, 84.37]). Ninety-four percent (n = 178/189, 95% CI [89.83, 97.06]) diagnosed incomplete KD based on fever and three clinical criteria. Forty-four percent (n = 84/189, 95% CI [37.23, 51.83]) accepted the presence of CALs as diagnostic in febrile patients, even without other clinical criteria; this approach was more common among Europeans. Infant age and elevated coronary Z-scores were identified as key indicators for coronary involvement. Years of experience did not influence the results.

Despite shared core diagnostic principles, country-specific differences persist in real-life clinical practice regarding fever duration, minimum clinical criteria and the role of coronary artery findings in diagnosing complete and incomplete KD. Future real-life studies are needed to support timely and consistent diagnosis.