Background Factors Associated with the Complications of 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 an initial single intravenous immunoglobulin (IVIG) therapy. In this study, I included a total of 200 pediatric patients who had received 2g/kg/day IVIG therapy for Kawasaki disease between 1999 and 2015 at the Department of Pediatrics, Aomori Prefectural Central Hospital. An initial IVIG therapy starting on day 5 was used as first-line therapy when possible. The second-line therapy was additional IVIG therapy, and the third-line therapy was an urinastatin infusion or plasma exchange. All patients received an initial single IVIG therapy with delayed or with concomitant administration of aspirin or flurbiprofen. Initial IVIG therapy resistance occurred in 48 of 200 patients (24%), and 17 patients (9%) received additional IVIG therapy. Four patients received urinastatin and one patient received plasma exchange as the third-line therapy. Before the 30th day, the prevalence of CAL was 5% (10/200); after 30 days, it was 2% (4/200). The maximal internal CAL diameter was 4.8 mm (Z score = 6.3) among all patients. Variable factors including IVIG resistance, responsiveness, and relapse of disease were associated with CAL complications. An initial single IVIG therapy may be useful for the prevention of large CAL caused by different factors of Kawasaki disease.