Cyclosporin a treatment for kawasaki disease refractory to initial and additional intravenous immunoglobulin

Hiroyuki Suzuki, Masaru Terai, Hiromichi Hamada, Takafumi Honda, Tomohiro Suenaga, Takashi Takeuchi, Norishige Yoshikawa, Shoichi Shibuta, Masakazu Miyawaki, Ko Oishi, Hironobu Yamaga, Noriyuki Aoyagi, Seiji Iwahashi, Ritsuko Miyashita, Yoshihiro Onouchi, Kumiko Sasago, Yoichi Suzuki, Akira Hata

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88 Citations (Scopus)

Abstract

BACKGROUND: There are still no definite treatments for refractory Kawasaki disease (KD). In this pilot study, we evaluated the use of cyclosporin A (CyA) treatment in patients with refractory KD. METHODS: We prospectively collected clinical data of CyA treatment (4-8 mg/kg/d, oral administration) for refractory KD patients using the same protocol among several hospitals. Refractory KD is defined as the persistence or recurrence of fever (37.5°C or more of an axillary temperature) at the end of the second intravenous immunoglobulin (2 g/kg) following the initial one. RESULTS: Subjects were enrolled out of 329 KD patients who were admitted to our 8 hospitals between January 2008 and June 2010. Among a total of 28 patients of refractory KD treated with CyA, 18 (64.3%) responded promptly to be afebrile within 3 days and had decreased C-reactive protein levels, the other 4 became afebrile within 4 to 5 days. However, 6 patients (21.4%) failed to become afebrile within 5 days after the start of CyA and/or high fever returned after becoming afebrile within 5 days. Although hyperkalemia developed in 9 patients at 3 to 7 days after the start of CyA treatment, there were no serious adverse effects such as arrhythmias. Four patients (1.2%), 2 before and the other 2 after the start of CyA treatment, developed coronary arterial lesions. CONCLUSION: CyA treatment is considered safe and well tolerated, and a promising option for patients with refractory KD. Further investigations will be needed to clarify optimal dose, safety, and timing of CyA treatment.

Original languageEnglish
Pages (from-to)871-876
Number of pages6
JournalPediatric Infectious Disease Journal
Volume30
Issue number10
DOIs
Publication statusPublished - 2011 Oct

Keywords

  • Kawasaki disease
  • T-cell activation
  • cyclosporin A
  • intravenous immunoglobulin-resistant (IVIG-resistant)

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Microbiology (medical)
  • Infectious Diseases

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