Prevention of Large Coronary Artery Lesions Caused by Kawasaki Disease

Appropriate therapy during the acute phase of Kawasaki disease to prevent large coronary artery lesions (CAL) has not been established. The aim of this retrospective study was to investigate the usefulness of initial intravenous immunoglobulin (IVIG) therapy with delayed administration of anti-inflammatory drugs (ADs). A total of 132 pediatric patients who received IVIG therapy with delayed administration of ADs for Kawasaki disease between 2004 and 2014 were enrolled at the Department of Pediatrics, Aomori Prefectural Central Hospital. An initial IVIG regimen of 2 g/kg/day, starting on day 5, was used as firstline therapy when possible. Second-line therapy was additional IVIG therapy, and third-line therapy was an urinastatin infusion or plasma exchange. All 132 patients received 2 g/kg/day initial IVIG therapy. 74 patients received aspirin and 58 patients received flurbiprofen after completion of initial IVIG infusion. Initial IVIG therapy resistance occurred in 31 of 132 patients (23%), and 10 patients (8%) received additional IVIG. One patient received urinastatin and one patient received plasma exchange as third-line therapy. Before the 30 th day, the prevalence of CAL was 2% (2/132); after 30 days, it was 1% (1/132). The maximal internal CAL diameters were 4.8 mm (Z score = 6.3) among all patients. Initial single IVIG therapy with delayed administration of ADs may be useful for the prevention of large CAL caused by Kawasaki disease.