TY - JOUR
T1 - Cyclosporin a treatment for kawasaki disease refractory to initial and additional intravenous immunoglobulin
AU - Suzuki, Hiroyuki
AU - Terai, Masaru
AU - Hamada, Hiromichi
AU - Honda, Takafumi
AU - Suenaga, Tomohiro
AU - Takeuchi, Takashi
AU - Yoshikawa, Norishige
AU - Shibuta, Shoichi
AU - Miyawaki, Masakazu
AU - Oishi, Ko
AU - Yamaga, Hironobu
AU - Aoyagi, Noriyuki
AU - Iwahashi, Seiji
AU - Miyashita, Ritsuko
AU - Onouchi, Yoshihiro
AU - Sasago, Kumiko
AU - Suzuki, Yoichi
AU - Hata, Akira
N1 - Funding Information:
Supported by the Ministry of Health, Labour and Welfare, Japan (2008–2010) through a Grant-in-Aid.
PY - 2011/10
Y1 - 2011/10
N2 - 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.
AB - 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.
KW - Kawasaki disease
KW - T-cell activation
KW - cyclosporin A
KW - intravenous immunoglobulin-resistant (IVIG-resistant)
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U2 - 10.1097/INF.0b013e318220c3cf
DO - 10.1097/INF.0b013e318220c3cf
M3 - Article
C2 - 21587094
AN - SCOPUS:80052923016
VL - 30
SP - 871
EP - 876
JO - Pediatric Infectious Disease Journal
JF - Pediatric Infectious Disease Journal
SN - 0891-3668
IS - 10
ER -