Cervical lymphadenopathy (CLA) is the least common main feature in patients with Kawasaki disease (KD), comprising only 42%-65% of all diagnoses. Nonetheless, several studies have shown that KD patients who first present with remarkable CLA and fever (NF-KD) are older in age and exhibit stronger inflammation than that of typical KD (tKD) patients. Therefore, whether NF-KD patients are also a high-risk group for intravenous immunoglobulin resistance (rIVIG) and coronary arterial aneurysm (CAA), and whether tKD with CLA (tKD-CLA) is associated with higher inflammatory indices than tKD without CLA warrants investigation. Previous retrospective (R) and prospective (P) studies have shown varied results (Table). In this study, we reviewed 10 years of medical records from a tertiary referral hospital and identified 42 NF-KD patients. These NF-KD patients were then compared with 113 tKD-CLA and 105 tKD patients from the same database over the same period. Significant trends of increasing values were noted in age, neutrophils, CRP, ESR, GOT, and doses of IVIG among these 3 groups of patients (p = 0.03 to < 0.0001). NF-KD patients presented with larger cervical LN (3.7 ± 7.0 cm) complicated by higher CAA (12/42 vs. 25/218, p = 0.008) and by moderate to extremely high CAA (6/42 vs. 4/218, p = 0.002), and suffered more CHF or shock at the acute stage (6/42 vs. 6/218, p < 0.001) than all of the tKD patients. The size of LN did not correlate with CAA occurrence, but the presence of bilateral CLA showed a higher risk for CAA than unilateral CLA did (6/10 vs. 6/32, p = 0.02). Author(Hospital No.) Method NF-KD tKD-CLA tKD LN(cm) CAA rIVIG Nomura Y (1) R 16 106 65 > 1.5 + + Kanegaye JT (1) P 53 . 287 3.0 ± 1.0 - - Kubota M (1) R 29 62 107 > 1.5 - - Sung RYT (14) R . 170 526 > 1.5 - - Chang JS (1) R 42 113 105 3.7 ± 7.0 + + This study showed that NF-KD patients, particularly those with larger lymph nodes, are a high risk group for CAA complications. The tKD-CLA patients also exhibited a tendency for higher inflammation. Previous pathological studies of swollen CLA have reported tissue edema in the sinus and paracortical areas of LN, as well as vessel thrombi in severe cases. Further study of swollen CLA is warranted to determine the pathological correlation between CLA severity and CAA complications in KD patients.