This retrospective study aimed to investigate the effects of anti-inflammatory drugs (ADs) on intravenous immunoglobulin (IVIG) therapy in the acute phase of Kawasaki disease. In total, 182 pediatric patients who received IVIG therapy for Kawasaki disease between 1999 and 2013 at the Department of Pediatrics, Aomori Prefectural Central Hospital were enrolled. Patients were divided into 2 groups: an S group, including 111 patients who received single IVIG therapy with delayed administration of ADs, and a T group, including 71 patients who received concomitant AIDs with IVIG. During the study, the only ADs administered were aspirin (A: 30 mg/kg/day) or flurbiprofen (F: 3-5 mg/kg/day). Steroids were not administered to any patient. The regimen of the S group was partially used after 2004 and was used to all patients after 2009. The following clinical findings were significantly different between the S and T groups: disease onset before 2003 (0 vs. 59%, P < 0.001) and after 2009 (70 vs. 0%, P < 0.001), use of 2-g/kg/day IVIG therapy (100 vs. 93%, P = 0.034), ADs type (A/F: 62/49 vs. 17/54, P < 0.001), and the prevalence of coronary artery lesions (CAL) up to (1/111 vs. 11/71, P < 0.001) and after 30 days of illness (0/111 vs. 4/71, P = 0.022). Logistic regression analysis revealed that IVIG therapy only (S group; P = 0.009) and 2-g/kg/day IVIG therapy (P = 0.015) were significant factors for CAL suppression. The findings revealed a possible negative impact of ADs on initial IVIG therapy in the acute phase of Kawasaki disease. Initial single IVIG therapy with delayed administration of ADs may be useful to suppress CAL caused by Kawasaki disease.

An acute phase treatment for prevention of coronary artery stenosis caused by Kawasaki disease (KD) has not been established. The objective of this study was to clarify the medium-term outcomes of patients who received acute phase treatment in our department. This retrospective study included data from 214 patients with KD who received acute phase treatment from January 2009 to May 2020. A total of 196 (92.1%) received an initial single dose of intravenous immunoglobulin (IVIG) therapy. One patient with status epilepticus at presentation received initial IVIG plus steroid therapy. A total of 17 patients did not receive IVIG. The rate of coronary artery lesions (CALs) 1 month and 1 year after KD onset were 1.9% and 0.9%, respectively. Two patients had CAL 1 year after KD onset. However, no patients had coronary artery stenosis. One patient with a right giant CAL had a medium CAL before initial therapy. During a median follow-up period of 3 years and 4 months, no patients had cardiac events that required therapy.

To study the histopathology of the myocardium in Kawasaki Disease (KD) with various coronary angiographic fmdings, right endomyocardial biopsy specimens were evaluated on 20 boys and 16 girls with this disorder. KD with coronary artery lesions (CAL) showed various histopathological abnormalities, such as myocellular hypertrophy in 44%, degeneration in 61%, interstitial fibrosis in 44% and endomyocardial changes in 22%. KD without CAL revealed myocellular hypertrophy in 44%, degeneration in SO%, disarray in 28% and interstitial fibrosis in 6%. As in patients with long-standing KD, more than three years after onset, myocardial changes such as myocellular hypertrophy, disarray and interstitial fibrosis persisted, it is suggested that cardiomyopathy may develop in some patients with KD. (Acta Paediatr Jpn 1989; 3 1 : 706 -7 1 1) References Kawasaki T. Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes. Jpn J Allergy 1967; 16: 178-222 (in Jpn). Kato H, lchinose E, Yoshioka F et al. Fate of coronary aneurysm in Kawasaki disease: serial coronary angiography and long-term follow-up study. Am J Cardiol 1982; 49: 1758-1766. Nakano H. Ueda K, Saito A et al. Repeated quantitative angiograms in coronary arterial aneurysm in Kawasaki disease. Am J Cardiol 1985; 56: 846-851.

Background:The acute phase treatment to prevent coronary artery stenosis have not been established in Kawasaki disease (KD). Objective: To ascertain the outcomes of coronary artery lesions (CAL) in patients who received an initial intravenous immunoglobulin (IVIG) therapy (2 g/kg) with delayed use of aspirin (DUA) for KD. Materials and Methods:The outcomes in 201 patients who underwent initial IVIG therapy at 2 g/kg/dose with DUA for KD were investigated retrospectively. The patients who received flurbiprofen instead of aspirin were also included. These subjects were divided into those who were associated with CAL (CAL group, n = 4) and those without CAL (non-CAL group, n = 197). Statistical analyses were performed using Stat Flex Version 6 for Windows (Artech Co., Ltd., Osaka, Japan). Chi-square, Fisher's exact, and Mann-Whitney U tests were used as appropriate, with sample size considerations.Results: The prevalence of resistant patients and those who required rescue therapies in the CAL group were significantly higher than those in non-CAL group (75.0% vs. 21.3%, P = 0.036 and 75.0% vs. 11.1%, P = 0.006, respectively). The values of neutrophil to lymphocyte ratio (NLR) after initial IVIG therapy in CAL group were significantly higher than those in non-CAL group (median: 5.91, range: 1.01-12.65 vs. 0.87, 0.01-11.29, P = 0.013). The one patient in CAL group who required plasma exchange had the highest value of the NLR. No patient had the large CAL>6.0 mm with high-risk for stenosis, and only one patient had persistent CAL of moderate-sized solitary right coronary artery at 60 th day of illness. Conclusion:An initial IVIG therapy (2 g/kg) with DUA may be useful for prevention of coronary artery stenosis caused in KD.

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