Abstract: Objective To explore the clinical characteristics of Kawasaki disease (KD) with cholestatic jaundice as the initial symptom, to enhance awareness and understanding of KD. Methods A retrospective analysis was conducted on 4,381 KD patients admitted between January 1, 2018 and December 31, 2024. Demographic, clinical, and laboratory data were collected from patients with febrile cholestatic jaundice as their first symptom. Patients were categorized into IVIG-resistant and IVIG-non-resistant groups based on their response to IVIG to identify the potential risk factors for IVIG resistance. Results Among 4,381 KD cases, 29 (0.66%) presented with cholestatic jaundice as the initial symptom. The median age of onset was 4.33 (3.17, 6.17) years, and jaundice appeared on the 3rd (3,3.5) day of the illness. At admission, the median total bilirubin (TBil) and direct bilirubin (DBil) levels were 82.75 (72.70, 102.10) μmol/L and 63.5 (45.65, 71.55) μmol/L, respectively. All patients underwent cardiac and hepatobiliary ultrasound before IVIG administration, revealing coronary artery abnormalities (CAA) in 10 cases and hepatobiliary abnormalities in 12 cases. The median time from onset to KD diagnosis was 6 (5,6) days, and IVIG treatment was initiated immediately after diagnosis, with 12 cases showing IVIG resistance. KD with cholestatic jaundice as the initial symptom exhibited a significantly higher IVIG resistance rate (χ²=62.01, P=0.00). Comparing the IVIG-resistant and non-resistant groups, those in the IVIG-resistant group received IVIG treatment earlier (Z=-1.568, P=0.049). Conclusion Clinicians should be vigilant for KD cases presenting with cholestatic jaundice, and this subset is more likely to develop IVIG resistance.

Key words: Kawasaki disease, Cholestatic jaundice, IVIG resistance