Background/Purpose: Kawasaki disease (KD) is an acute, systemic vasculitis which can lead to coronary artery (CA) aneurysms in young children. In 2018, we developed a clinical pathway to standardize KD care and improve outcomes. In this retrospective quality improvement (QI) study, we aimed to assess patients admitted with KD and determine the impact of the pathway on medication use and outcomes to inform QI initiatives.

Methods: We examined index admissions for KD with IVIG administration from January 2017 to March 2025. All echocardiogram (echo) data ≤60 days after the admission date were included. The right coronary and left anterior descending arteries were assessed. CA changes were classified based on 2024 American Heart Association guidelines. A normal initial echo was defined as CA Z-scores between -2 and 2 on the first echo within 3 days of admission. The KD pathway was published in January 2018 and updated in November 2022. The original pathway standardized steroid use for patients less than 6 months of age and those with CA aneurysms (Z-score ≥2.5). The updated pathway recommended infliximab and steroids for all IVIG-resistant patients. Electronic health record (EHR) order sets were created to implement the pathway’s diagnostic and treatment recommendations. All EHR and echo data are updated daily and displayed in a data visualization tool. Standard run chart rules were used to analyze process and outcome measures over time. p values were calculated using the Chi-square and t-tests for categorical and continuous measures, respectively, and the F-test to compare variances.

Results: Demographic, echo, and treatment data are shown in Table 1 (n=413). The mean age at admission was 3.7 ± 2.8 years, and 58% of patients were male. The emergency department and inpatient KD pathway order sets were used in 39% and 77% of cases, respectively. Abnormal initial echos were observed in 85 patients (21%), who were younger (3.0 vs 3.9 years, p< 0.05), had more echos (4.3 vs 3.0, p< 0.001), and were more likely to develop aneurysms (68% vs 6%, p< 0.001) and receive steroids (74% vs 35%, p< 0.001) and infliximab (19% vs 9%, p< 0.01). After the 2022 pathway update, second IVIG doses decreased from 33% to 4%, while infliximab administration increased from 6% to 25% (Figure 1). Steroid use increased after the onset of the COVID-19 pandemic in 2020 and remained higher after the pathway update. Among patients with normal initial echos, there was no change in the rate of CA aneurysm development or length of stay (LOS) (Figure 2). However, there was a decrease in LOS variance (0.16 vs 0.64, p< 0.05). In 2024, we identified 4 patients with normal initial echos who developed medium or large CA aneurysms.

Conclusion: After KD pathway implementation, we observed an increase in steroid and infliximab use and a decrease in second doses of IVIG. Though LOS remained stable, its variation decreased after updated pathway recommendations on management of IVIG-resistant KD, which may represent improved standardization of care. Though CA aneurysm development has not changed overall, we are currently using our real-time data visualization tools to investigate recent cases of medium and large aneurysms to further optimize KD care.

Table 1. Cohort characteristics among index admissions for Kawasaki disease (January 1, 2017 to March 31, 2025)

Figure 1. Medication Administration in All KD Admissions (2017-2025)​

Figure 2. Outcomes in KD Patients with Normal Initial Echocardiograms (2017-2025)​

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/impact-of-a-clinical-pathway-on-medication-use-and-outcomes-in-kawasaki-disease/