日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

Online ISSN: 2187-2988 Print ISSN: 0911-1794
特定非営利活動法人日本小児循環器学会 Japanese Society of Pediatric Cardiology and Cardiac Surgery
〒162-0801東京都新宿区山吹町358-5アカデミーセンター Japanese Society of Pediatric Cardiology and Cardiac Surgery Academy Center, 358-5 Yamabuki-cho, Shinju-ku, Tokyo 162-0801, Japan
Pediatric Cardiology and Cardiac Surgery 33(1): 43-49 (2017)
doi:10.9794/jspccs.33.43

原著Original

大量ガンマグロブリン療法不応の川崎病に対するインフリキシマブ療法:A single-institute studyInfliximab for Intravenous Immunoglobulin-Resistant Patients with Kawasaki Disease: A Single-Institute Study

1国立成育医療研究センター総合診療部Department of General Pediatrics & Interdisciplinary Medicine, National Center for Child Health and Development ◇ Tokyo, Japan

2国立成育医療研究センター循環器科Division of Cardiology, National Center for Child Health and Development ◇ Tokyo, Japan

3国立成育医療研究センター研究所高度先進医療研究室Division of Advanced Medicine for Virus Infections, National Center for Child Health and Development ◇ Tokyo, Japan

4国立成育医療研究センター臨床研究開発センター開発企画部臨床研究企画室Division of Clinical Research Planning, Department of Development Strategy Center for Clinical Research and Development, National Center for Child Health and Development ◇ Tokyo, Japan

5国立成育医療研究センター教育研修部Development of Postgraduate Education and Training, National Center for Child Health and Development ◇ Tokyo, Japan

6横浜市立大学大学院医学研究科発生成育小児医療学Department of Pediatrics, Graduate School of Medicine, Yokohama City University ◇ Kanagawa, Japan

受付日:2016年11月11日Received: November 11, 2016
受理日:2016年12月24日Accepted: December 24, 2016
発行日:2017年1月1日Published: January 1, 2017
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背景:大量ガンマグロブリン(IVIG)療法不応川崎病に対するインフリキシマブ(IFX)療法が増加している.

目的:IFX療法の有効性,安全性を明らかにする.

方法:2008年12月~2016年9月にIVIG不応川崎病に対して,IFX療法を行った55例を後方視的に検討した.

結果:男34名(62%),月齢は,中央値31か月(4~131),IFX投与病日は,中央値は第10病日(7~24)であった.IFX療法は40例(73%)が3rd line以内に行った.投与48時間以内に38例(69%)が解熱した.10例(18%)でIFX療法後に追加治療を行った.IFX投与前後で白血球数,好中球(%),CRPなどの炎症マーカーが有意に低下した.IFX解熱例(n=38)と非解熱例(n=17)の2群間では,性,月齢,投与病日の有意差を認めなかった.冠動脈病変(CAL)は,IFX投与時にすでに7例で認め,投与後新たに認めた症例は4例で,そのうち1例で巨大瘤を認めた.有害事象は13例(23%)で,過去にIFXの投与経験がある1例でinfusion reactionを認めた.

考察・結語:IFX療法はIVIG不応川崎病に対して有効な治療法である.CAL合併の頻度は過去のIFXの有効性を論じた報告と比べて同程度だった.重篤な有害事象は認めなかったが長期的な影響については今後も注視する必要がある.

Background: Infliximab (IFX) therapy is increasingly used in the treatment of intravenous immunoglobulin (IVIG)-resistant patients with Kawasaki disease (KD).

Purpose: To analyze the effects and safety of IFX therapy in IVIG-resistant patients with KD.

Methods: Fifty-five patients who were administered IFX after initial IVIG between December 2008 and September 2016 were included in this study.

Results: Of the 55 patients treated with IFX for KD, 34 (62%) were male, with a median age of 31 (4–131) months. The median number of days of illness at the initiation of IFX therapy was 10 (7–24) days. In 40 (73%) patients, this treatment was provided as part of second- or third-line therapy. Following IFX administration, 38 (69%) patients became afebrile within 48 h (responders). Ten patients (18%) were given additional treatments after IFX administration. No significant differences in sex, age, or days of IFX administration were observed between the responders (n=38) and non-responders (n=17). After IFX administration, the levels of WBC and percentage of neutrophils and CRP decreased. In seven cases, coronary arterial lesions (CAL) were present at the time of IFX administration. In addition, four cases developed CAL after IFX administration. Adverse events occurred in 13 (23%) cases, but no patients developed severe complications.

Discussion/Conclusion: IFX therapy is effective in alleviating fever and reducing the levels of inflammatory markers. CAL occurred at a similar frequency as that observed in previous reports. Although no serious adverse events were observed, close observation is needed in case long-term complications arise.

Key words: Kawasaki disease; infliximab; intravenous immunoglobulin; coronary artery lesions; infusion reaction

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