Outcomes of Older Children with Kawasaki Disease Who Received Intravenous Immunoglobulin Therapy with Delayed Use of Anti-inflammatory Drugs

Background: The prevalence of coronary artery lesions (CAL) is higher in older children compared with younger children. Anti-inflammatory drugs including aspirin appeared to have a negative impact on the suppressive effects of initial intravenous immunoglobulin (IVIG) therapy for CAL development during the acute phase of Kawasaki disease and an initial single IVIG therapy with the delayed use of anti-inflammatory drugs (DUA) might be effective for CAL suppression. The outcomes regarding CAL and non-cardiac complications after single IVIG therapy with DUA remain unclear.

Aims: To ascertain the outcomes of the older children with Kawasaki disease who received single IVIG therapy with DUA.

Methods: The retrospective data of 25 children who were ≧ 60 months old and received this therapy between 2004 and 2017 in our department were collected and outcomes were investigated. Anti-inflammatory drugs were initiated within 24 hours after the end of initial IVIG infusion.

Results: No patients were associated with CAL. However, three patients were associated with non-cardiac complications including transient blindness, intractable arthritis, and prolonged restriction of cervical movement caused by atlantoaxial rotatory fixation. All three children were IVIG therapy-resistant and had statistically higher C-reactive protein values (median: 9.64 interquartile range: 7.82-18.29 vs. 1.905: 0.76-4.77 mg/dL, P = 0.007), neutrophil counts (9545: 9312-9660 vs. 2766.5: 1936-3812 /mm3, P = 0.006), and neutrophil to lymphocyte ratios (10.38: 4.85-11.29 vs. 1.44: 0.92-2.69, P = 0.006) after IVIG therapy compared to those without complications.

Conclusion: Initial IVIG therapy-resistant three patients with high values of inflammatory biomarkers after initial therapy with DUA were associated with non-cardiac complications.