Hostname: page-component-586b7cd67f-t7czq Total loading time: 0 Render date: 2024-11-28T07:37:20.475Z Has data issue: false hasContentIssue false

Stratified therapy for Kawasaki disease using a new scoring system to predict the response to a lower dose of intravenous immunoglobulin therapy

Published online by Cambridge University Press:  10 June 2021

Misa Matsuura
Affiliation:
Department of Pediatrics, Saitama Medical Center, Jichi Medical University, Saitama, Japan
Daisuke Sugawara
Affiliation:
Department of Pediatrics, Saitama Medical Center, Jichi Medical University, Saitama, Japan
Eishi Makita*
Affiliation:
Department of Pediatrics, Saitama Medical Center, Jichi Medical University, Saitama, Japan
Yuka Hirakubo
Affiliation:
Department of Pediatrics, Saitama Medical Center, Jichi Medical University, Saitama, Japan
Kazuhito Nonaka
Affiliation:
Department of Pediatrics, Saitama Medical Center, Jichi Medical University, Saitama, Japan
Shinichiro Yamashita
Affiliation:
Department of Pediatrics, Saitama Medical Center, Jichi Medical University, Saitama, Japan
Ko Ichihashi
Affiliation:
Department of Pediatrics, Saitama Medical Center, Jichi Medical University, Saitama, Japan
*
Author for correspondence: Dr E. Makita, MD, Department of Pediatrics, Saitama Medical Center, Jichi Medical University, 1-847 Amanuma-cho, Omiya-ku, Saitama 330-8503, Japan. Tel: +81 48 647 2111; Fax: +81 648 0960. E-mail: [email protected]

Abstract

Background:

Several studies have reported treatment options for patients with Kawasaki disease refractory to standard immunoglobulin therapy; however, no studies have reported low-dose immunoglobulin therapy for patients with a low risk of Kawasaki disease.

Methods:

A total of 277 patients with Kawasaki disease were included in this study. We used Kobayashi score and our Less high-risk score to divide the patients into three groups. Patients in the high-risk group (Kobayashi score ≥ 5 points) received 2 g/kg intravenous immunoglobulin and prednisolone. Patients in the moderate-risk group (Kobayashi score < 5 points and Less high-risk score ≥ 2 points) received 2 g/kg intravenous immunoglobulin treatment. Patients in the low-risk group (Kobayashi score < 5 points and Less high-risk score < 2 points) received 1 g/kg intravenous immunoglobulin treatment. The response rate and the incidence of coronary artery lesions at 4 weeks after treatment were evaluated in each group.

Results:

The treatment response rates in the high-risk (n = 110), moderate-risk (n = 80), and low-risk (n = 87) groups were 74.5, 72.5, and 77.0%, respectively. Coronary artery lesions occurred in 7.3, 3.8, and 2.3% of patients in the high-, moderate-, and low-risk groups, respectively. There were no significant differences between the groups regarding treatment response or coronary artery lesion rate.

Conclusion:

The therapeutic response rate and the therapeutic effect of low-dose intravenous immunoglobulin in the low-risk group identified with our new scoring were satisfactory. Stratified therapies for patients with Kawasaki disease based on the scoring system may be useful.

Type
Original Article
Copyright
© The Author(s), 2021. Published by Cambridge University Press

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Kawasaki, T, Kosaki, F, Okawa, S, Shigematsu, I, Yanagawa, H. A new infantile acute febrile mucocutaneous lymph node syndrome (MLNS) prevailing in Japan. Pediatrics 1974; 54: 271276.CrossRefGoogle ScholarPubMed
McCrindle, BW, Rowley, AH, Newburger, JW, et al. Diagnosis, treatment, and long-term management of Kawasaki disease: a scientific statement for health professionals from the American Heart Association. Circulation 2017; 135: 927999.CrossRefGoogle ScholarPubMed
Kobayashi, T, Inoue, Y, Takeuchi, K, et al. Prediction of intravenous immunoglobulin unresponsiveness in patients with Kawasaki disease. Circulation 2006; 113: 26062612.CrossRefGoogle ScholarPubMed
Egami, K, Muta, H, Ishii, M, et al. Prediction of resistance to intravenous immunoglobulin treatment patients with Kawasaki disease. J Pediatr 2006; 149: 237240.CrossRefGoogle ScholarPubMed
Sano, T, Kurotovi, S, Matsuzaki, K, et al. Prediction of non-responsiveness to standard high-dose gamma-globulin therapy in patients with acute Kawasaki disease before starting initial treatment. Eur J Pediatr 2007; 166: 131137.CrossRefGoogle ScholarPubMed
Kobayashi, T, Saji, T, Otani, T, et al. Efficacy of immunoglobulin plus prednisolone for prevention of coronary artery abnormalities in severe Kawasaki disease (RAISE study): a randomized, open-label, blinded-endpoints trial. Lancet 2012; 379: 16131620.CrossRefGoogle ScholarPubMed
Hamada, H, Suzuki, H, Onouchi, Y, et al. Efficacy of primary treatment with immunoglobulin plus ciclosporin for prevention of coronary artery abnormalities in patients with Kawasaki disease predicted to be at increased risk of non-response to intravenous immunoglobulin (KAICA): a randomized controlled, open-label, blinded-endpoints, phase 3 trial. Lancet 2019; 393: 11281137.CrossRefGoogle ScholarPubMed
Okada, K, Hara, J, Maki, I, et al. Osaka Kawasaki disease study group: pulse methylprednisolone with gammaglobulin as an initial treatment for acute Kawasaki disease. Eur J Pediatr 2009; 168: 181185.CrossRefGoogle Scholar
Ogata, S, Ogihara, Y, Honda, T, et al. Corticosteroid pulse combination therapy for refractory Kawasaki disease: a randomized trial. Pediatrics 2012; 129: e17e23.CrossRefGoogle ScholarPubMed
Ichihashi, K, Shiraishi, H, Momoi, M. Prediction of responsiveness or on-responsiveness to treatment of acute Kawasaki disease using 1 g/kg of immunoglobulin- an effective and cost-saving schedule of therapy. Cardiol Young 2009; 19: 224227.CrossRefGoogle ScholarPubMed
Kobayashi, T, Ayusawa, M, Suzuki, H, et al. Revision of diagnostic guidelines for Kawasaki disease (6th revised edition). Pediatr Int 2020; 62: 11351138.CrossRefGoogle Scholar
Kobayashi, T, Fuse, S, Sakamoto, N, et al. A new z score curve of the coronary arterial internal diameter using the Lambda-Mu-Sigma method in a pediatric population. J Am Soc Echocardiogr 2015; 29: 794801.CrossRefGoogle Scholar
Kanda, Y. Investigation of the freely available easy-to-use software ‘EZR’ for medical statistics. Bone Marrow Transplant 2013; 48: 452458.CrossRefGoogle ScholarPubMed
Japanese Society of Pediatric Cardiology and Cardiac Surgery. The guidelines on acute stage Kawasaki disease treatment, Japanese, Tokyo. Pediatric Cardiol Card Surg 2020; 36: S1.1S1.29.Google Scholar
Klasesen, TP, Rowe, PC, Gafnl, A. Economic evaluation of intravenous immune globulin therapy for Kawasaki syndrome. J Pediatr 1993; 122: 538542.CrossRefGoogle Scholar
Sano, N, Sugimura, T, Akagi, T, et al. Selective high dose gamma-globulin treatment in Kawasaki disease: assessment of clinical aspects and cost effectiveness. Pediatr Int 1999; 41: 17.Google Scholar
Sakata, K, Hamaoka, K, Ozawa, S, et al. A randomized prospective study on the use of 2 g-IVIG or 1 g-IVIG as therapy for Kawasaki disease. Eur J Pediatr 2007; 166: 565571.CrossRefGoogle ScholarPubMed
Barron, KS, Murphy, DJ, Silverman, ED, et al. Treatment of Kawasaki syndrome: a comparison of two dosage regimens of intravenously administered immune globulin. J Pediatr 1990; 117: 638644.CrossRefGoogle ScholarPubMed
Japan Kawasaki Disease Research Center. Descriptive epidemiology of Kawasaki disease in Japan, 2017–2018: from the results of the 25th Nationwide Survey. 2019. https://www.jichi.ac.jp/dph/inprogress/kawasaki/.Google Scholar
Bonilla, FA. Intravenous immunoglobulin: adverse reactions and management. J Allergy Clin Immunol 2008; 122: 12381239.CrossRefGoogle ScholarPubMed