title: Cyclosporin A treatment for Kawasaki disease refractory to initial and additional intravenous immunoglobulin.
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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Pediatrics, Wakayama Medical University, Wakayama, Japan. hsuzuki@wakayama-med.ac.jp
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Suzuki
authors.forename: Masaru
authors.initials: M
authors.lastname: Terai
authors.forename: Hiromichi
authors.initials: H
authors.lastname: Hamada
authors.forename: Takafumi
authors.initials: T
authors.lastname: Honda
authors.forename: Tomohiro
authors.initials: T
authors.lastname: Suenaga
authors.forename: Takashi
authors.initials: T
authors.lastname: Takeuchi
authors.forename: Norishige
authors.initials: N
authors.lastname: Yoshikawa
authors.forename: Shoichi
authors.initials: S
authors.lastname: Shibuta
authors.forename: Masakazu
authors.initials: M
authors.lastname: Miyawaki
authors.forename: Ko
authors.initials: K
authors.lastname: Oishi
authors.forename: Hironobu
authors.initials: H
authors.lastname: Yamaga
authors.forename: Noriyuki
authors.initials: N
authors.lastname: Aoyagi
authors.forename: Seiji
authors.initials: S
authors.lastname: Iwahashi
authors.forename: Ritsuko
authors.initials: R
authors.lastname: Miyashita
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Onouchi
authors.forename: Kumiko
authors.initials: K
authors.lastname: Sasago
authors.forename: Yoichi
authors.initials: Y
authors.lastname: Suzuki
authors.forename: Akira
authors.initials: A
authors.lastname: Hata
mesh_terms: D002675:Child, Preschool; D016572:Cyclosporine / Q000008:administration & dosage* / Q000009:adverse effects; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage*; D007166:Immunosuppressive Agents / Q000008:administration & dosage* / Q000009:adverse effects; D007223:Infant; D008297:Male; D009080:Mucocutaneous Lymph Node Syndrome / Q000188:drug therapy*; D010865:Pilot Projects
chemical_list: D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D016572:Cyclosporine