Key aspects of the medical management of Kawasaki disease (KD) are not yet supported by a high evidence level, thus making room for individual recommendations. We performed a structured comparison of existing international KD guidelines to analyze potential differences in the implementation of evidence-based KD recommendations regarding diagnosis and therapy. To identify country-specific guidelines, we took a multilateral approach including a comprehensive PubMed literature, online research, and directly contacting national pediatric associations. We then ran a structured guidelines\' analysis and evaluated the diagnostic and therapeutic differences in the context of evidence-based medicine. In this structured guideline analysis, we identified nine national and one European guidelines. According to them all, the diagnosis of KD still relies on its clinical presentation with no reliable biomarker recommended. First-line treatment consistently involves only intravenous immunoglobulin (IVIG) therapy. Recommendations in terms of acetylsalicylic acid, corticosteroids, and additional therapeutic options vary considerably.
CONCLUSIONS: According to all guidelines, KD is diagnosed clinically with some variance in defining incomplete KD and being a non-responder to treatment. First-line treatment consistently includes IVIG. Recommendations for additional therapeutic strategies are more heterogeneous.
BACKGROUND: • The diagnosis of KD relies on the clinical presentation, entailing challenges in timely diagnosis. • Other treatment options then IVIG are not supported by a high evidence level, making room for individual recommendations.
BACKGROUND: • Definition of incomplete KD and being non-responsive to an initial treatment vary to some extent between the national guidelines. • Only IVIG is consistently proposed throughout all guidelines, further therapeutic recommendations vary between the national recommendations.

Kawasaki disease (KD), an acute, generalized vasculitis, is associated with an increased risk of coronary heart disease and is the most common cause of acquired heart disease in childhood. The incidence of KD is increasing worldwide. There are numerous international treatment guidelines. Our study aims to perform the first one so far comparison of them. While the gold standard therapy remains still the same (intravenous immunoglobulins and aspirin), there is currently a lack of evidence for choosing optimal treatment for high-risk patients and refractory KD. In this review, we also discuss the treatment of complications of KD and Kawasaki-like phenotypes, present an anti-inflammatory treatment in the light of new scientific data, and present novel potential therapeutic targets for KD.

Objective: Previous studies showed the efficacy of glucocorticoids on prevention of coronary artery lesions (CAL) among Kawasaki disease (KD) patients, and clinical guideline for KD in Japan was changed regarding glucocorticoid use in 2012. However, little is known regarding how the guideline change had impacts on healthcare utilizations and clinical outcomes. Methods: We conducted a retrospective observational study using national inpatient database in Japan among KD patients aged under 18 years during 2010-2015. Recent trends in practice patterns were analyzed, and we divided the hospitals into four groups based on glucocorticoid use: (1) consistently using hospital, (2) started using hospital, (3) stopped using hospital, and (4) never using hospital. Then, we compared healthcare utilizations and risks of coronary artery lesions before and after the guideline change. Results: We identified 24,517 inpatients with KD. From 2010 to 2014, use of glucocorticoid increased from 8.9 to 17.4% of KD inpatients. All types of hospitals showed reduction in coronary artery lesions, but the reduction was the most prominent in hospitals that started using glucocorticoid therapy after clinical guideline change in 2012 (adjusted OR, 0.22; 95%CI, 0.07-0.68). Also, Glucocorticoid consistently using hospitals, started using hospitals, and never using hospitals showed reductions in hospitalization costs, whereas hospitals that stopped using glucocorticoids after clinical guideline change had elevated healthcare costs as opposed to natural trends observed in other groups. Guideline complying hospitals had the greatest reductions in healthcare costs. Conclusions: The early stage glucocorticoid use could be a cost-saving strategy for treatment for KD patients without increasing risks of CAL.

OBJECTIVE: Current guidelines for Kawasaki disease (KD) recommend intravenous immunoglobulin infusion and echocardiography. However, no previous studies have evaluated the relationship between the treating hospital\'s concordance with guidelines and the prevention of coronary artery aneurysm in patients with KD.
METHODS: KD patients between 2010 and 2013 were identified in a Japanese national inpatient database. Guideline concordance was defined as the proportions of patients who received echocardiography as well as treatment with both immunoglobulin and aspirin, which were divided into quartiles. Multivariable logistic regression analysis was conducted to examine the association between the guideline concordance and the occurrence of coronary artery aneurysm with adjustment for patient backgrounds.
RESULTS: In sum, 20 156 patients with KD were identified. The very high (>83.3%) concordance group had a lower rate of coronary artery aneurysm than the very low (<59.0%) concordance group (4.9% vs 9.9%; odds ratio, 0.45; 95% CI, 0.27 to 0.74; P = .002).
CONCLUSIONS: All patients with KD should be treated according to the guidelines.