The Clinical Utility and Safety of a New Strategy for the Treatment of Refractory Kawasaki Disease.

OBJECTIVE: To assess the clinical utility and safety of a strategy for refractory Kawasaki disease, defined by Egami score ≥3. STUDY DESIGN: First-line treatment was with intravenous methylprednisolone (30 mg/kg, 2 hours, 1 dose) plus intravenous immunoglobulin (2 g/kg, 24 hours) treatment. Patients resistant to first-line treatment received additional intravenous immunoglobulin as a second-line treatment. Patients resistant to second-line treatment who had received Bacillus Calmette-Guérin vaccination 6 months earlier were treated with infliximab; otherwise, plasma exchange was performed. A total of 71 refractory patients with Kawasaki disease (median age: 2.4 years) of 365 patients with Kawasaki disease were treated according to our strategy from April 2007 to April 2016. Treatment resistance was defined as a persistent fever at 36 hours after treatment. We evaluated coronary artery lesions at the time of the diagnosis, at 1 month, and at 1 year after the diagnosis in accordance with the American Heart Association guidelines and the criteria of the Japanese Ministry of Health, Labour, and Welfare. RESULTS: First-line therapy was effective for 58 of 71 patients (81.6%), and second-line therapy was effective for 9 of 13 patients (69.2%). At third line, 3 patients were treated by infliximab, and 1 was treated with plasma exchange. Of the 18 patients with coronary artery abnormalities at diagnosis, 13 patients at 1 month and 6 patients at 1 year had coronary artery dilatation (median z score 3.0, 2.6, and 1.4, respectively). There were no patients with coronary artery aneurysm (CAA). CONCLUSIONS: Our strategy for refractory Kawasaki disease was safe and effective in preventing CAA.

Percutaneous coronary intervention versus coronary artery bypass grafting for stenotic lesions after Kawasaki disease.

OBJECTIVE: To compare the long-term efficacy of percutaneous coronary intervention (PCI) and coronary artery bypass grafting (CABG) for stenotic lesions after Kawasaki disease. STUDY DESIGN: Questionnaires were sent to pediatricians in Japan in hospitals with 100 or more beds. A total of 1637 questionnaires were sent, and 1347 (82%) were returned. The primary endpoints of the study were the composite of deaths from any cause and the Q-wave myocardial infarction. The secondary endpoints were the repeat-revascularization for the target vessel. RESULTS: A total of 67 patients underwent PCI and 81 patients underwent CABG. Although the rates of the primary endpoints did not differ between the groups (hazard ratio 1.35 [95% confidence interval 0.29 to 6.32], P=.7), the rate of the secondary endpoints in the PCI group was significantly higher than that in the CABG group (hazard ratio 2.23 [95% confidence interval 1.04 to 4.76], P=.04). The benefit with CABG for the secondary endpoints was notable in patients younger than 12 years old, and who had ischemic change. CONCLUSIONS: PCI after Kawasaki disease resulted in a lower efficacy in comparison to CABG because of increased repeat-revascularization procedures.

Health-related quality of life in adolescents and young adults with a history of Kawasaki disease.

OBJECTIVE: To investigate the health-related quality of life (HRQOL) in adolescents and young adults with Kawasaki disease (KD). STUDY DESIGN: We conducted a cross-sectional observational study. Patients were eligible for this study when they were > or =16 years of age and had received a diagnosis of KD > or =5 years before the start of this study. The patients were divided in 3 groups according to their coronary status: normal, aneurysms, and giant aneurysms/ischemia. A self-administered questionnaire (Medical Outcome Study Short Form 36) was mailed to the patients to evaluate the HRQOL. RESULTS: Of 624 total questionnaires mailed, 377 were delivered, and 250 were returned. The response rates of the normal, aneurysm, and giant aneurysms/ischemia groups were 33%, 62%, and 72% of all eligible patients, respectively. All subscale scoress of HRQOL, except vitality and role-emotional (limitations in the usual role activities because of emotional problems) in patients with KD were significantly higher than scores from the national norms. CONCLUSIONS: The HRQOL of adolescents and young adults with a history of KD is favorable. However, long-term follow-up is necessary, because the general health perceptions of older patients tended to be lower.

Prediction of resistance to intravenous immunoglobulin treatment in patients with Kawasaki disease.

OBJECTIVES: The objective of this study was to find the predictors and generate a prediction score of resistance to intravenous immunoglobulin (IVIG) in patients with Kawasaki disease (KD). STUDY DESIGN: Patients diagnosed as having KD were sampled when they received initial high-dose IVIG treatment (2 g/kg dose) within 9 days of illness (n = 320). These patients were divided into 2 groups: the resistance (n = 41) and the responder (n = 279). The following data were obtained and compared between resistance and responder: age, sex, illness days at initial treatment, and laboratory data. RESULTS: Multivariate logistic regression analysis identified age, illness days, platelet count, alanine aminotransferase (ALT), and C-reactive protein (CRP) as significant predictors for resistance to IVIG. We generated prediction score assigning 1 point for (1) infants less than 6 months old, (2) before 4 days of illness, (3) platelet count or= 8 mg/dL, as well as 2 points for (5) ALT >or= 80 IU/L. Using a cut-off point of 3 and more with this prediction score, we could identify the IVIG-resistant group with 78% sensitivity and 76% specificity. CONCLUSIONS: Resistance to IVIG treatment can be predicted using age, illness days, platelet count, ALT, and CRP. Randomized, multicenter clinical trials are necessary to create a new strategy to treat these high-risk patients.

Early intravenous gamma-globulin treatment for Kawasaki disease: the nationwide surveys in Japan.

OBJECTIVE: To determine the optimal period of intravenous gamma-globulin (IVGG) treatment, using the database from nationwide Kawasaki disease surveys in Japan. STUDY DESIGN: We selected patients who first visited a doctor within 3 days of illness and received IVGG treatment within 9 days of illness. We divided these patients into 2 groups: an early group (treated on days 1-4: 4731 cases) and a conventional group (days 5-9: 4020 cases). We compared the rate of additional IVGG and prevalence of cardiac sequelae between these groups. RESULTS: The rate of additional IVGG in the early group was significantly higher than those of the conventional group (OR, 1.12 [95% CI, 1.10-1.16]). There were no significant differences in cardiac sequelae between the two groups. CONCLUSIONS: There is no evidence that IVGG treatment on day 4 or earlier has greater efficacy in preventing cardiac sequelae than treatment on days 5 to 9. In addition, early treatment is likely to result in a greater requirement for additional IVGG. However, there is also no evidence that early treatment increases the prevalence of cardiac sequelae in a clinical practice setting, where additional IVGG can be given to those whose initial treatment fails.