title: Calcineurin inhibitor treatment of intravenous immunoglobulin-resistant Kawasaki disease.
abstract: OBJECTIVE
  To describe the clinical course and outcome of 10 patients with Kawasaki disease (KD) treated with a calcineurin inhibitor after failing to respond to multiple therapies.
  
  
  METHODS
  Demographic and clinical data were prospectively collected using standardized case report forms. T-cell phenotypes were determined by flow cytometry, and KD risk alleles in ITPKC (rs28493229), CASP3 (rs72689236), and FCGR2A (rs1801274) were genotyped.
  
  
  RESULTS
  Intravenous followed by oral therapy with cyclosporine (CSA) or oral tacrolimus was well tolerated and resulted in defervescence and resolution of inflammation in all 10 patients. There were no serious adverse events, and a standardized treatment protocol was developed based on our experiences with this patient population. Analysis of T-cell phenotype by flow cytometry in 2 subjects showed a decrease in circulating activated CD8(+) and CD4(+) T effector memory cells after treatment with CSA. However, suppression of regulatory T-cells was not seen, suggesting targeting of specific, proinflammatory T-cell compartments by CSA.
  
  
  CONCLUSIONS
  Treatment of refractory KD with a calcineurin inhibitor appears to be a safe and effective approach that achieves rapid control of inflammation associated with clinical improvement.
authors.affiliation: Department of Pediatrics, University of California at San Diego and Rady Children's Hospital, La Jolla, CA, USA.
authors.forename: Adriana H
authors.initials: AH
authors.lastname: Tremoulet
authors.forename: Paige
authors.initials: P
authors.lastname: Pancoast
authors.forename: Alessandra
authors.initials: A
authors.lastname: Franco
authors.forename: Matthew
authors.initials: M
authors.lastname: Bujold
authors.forename: Chisato
authors.initials: C
authors.lastname: Shimizu
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Onouchi
authors.forename: Alyson
authors.initials: A
authors.lastname: Tamamoto
authors.forename: Guliz
authors.initials: G
authors.lastname: Erdem
authors.forename: Debra
authors.initials: D
authors.lastname: Dodd
authors.forename: Jane C
authors.initials: JC
authors.lastname: Burns
mesh_terms: D002648:Child; D002675:Child, Preschool; D002985:Clinical Protocols; D016572:Cyclosporine / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007155:Immunologic Factors / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007223:Infant; D008297:Male; D009080:Mucocutaneous Lymph Node Syndrome / Q000188:drug therapy* / Q000276:immunology; D013601:T-Lymphocytes / Q000276:immunology; D016559:Tacrolimus / Q000627:therapeutic use; D017211:Treatment Failure
chemical_list: D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D016572:Cyclosporine; D016559:Tacrolimus