Severe Kawasaki disease
Conditions
Interventions
Intervention type:DRUG
Name of intervention:Cyclosporin A(CsA)
Dose form / Japanese Medical Device Nomenclature:ORAL SOLUTION
Route of administration / Site of application:ORAL
Dose per administration
Sponsors
Coordinating Committee
Chiba University Hospital
Tokyo Women's Medical University Yachiyo Medical Center
Wakayama Medical University Hospital
Japanese Red Cross Kumamoto Hospital
Kumamoto Regional Medical Center
National Center for Child Health and Development
Hiroshima City Hospital
Ehime University Hospital
Okinawa Prefectural Nanbu Medical Center and Children's Medical Center
Funabashi Municipal Medical Center
Kimitsu Chuo Hospital
Showa University Northern Yokohama Hospital
Hokkaido University Hospital
Ehime Prefe
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: 1)Pediatric patients with a diagnosis of KD according to the Kawasaki Disease Diagnostic Guideline (the 5th revised edition). Patients who meet 5 of the 6 main symptoms below are diagnosed as having KD. 2)Severe KD patients with a risk score of 5 points or higher. The risk score is defined as the sum of all items in the risk-scoring system (5 blood exam data, 2 demographic data of the subjects) developed by Kobayashi et al. Of note, the risk scoring will be performed using a method employed in the previous literature in which the worst values of the blood exam obtained within the acceptable range of time points will be adopted. 3)Age of 4 months or more at the time of signing the informed consent form. 4)Inclusion in the study within 7 days of disease onset. (considering day 1 to be the day when the fever develops.) 5)Informed consent form signed by the patient or a legal guardian.
Exclusion criteria
Exclusion criteria: 1)A history of KD recurrence. 2)CAAs prior to enrolment. 3)No presence of fever prior to enrolment. 4)Suspicion that the symptoms may correspond to a disease other than KD. (haemolytic streptococcal infection, EB virus infection, Yersinia infection, measles or Stevens-Johnson syndrome.) 5)Initiation of IVIG treatment later than 9 days after disease onset. 6)Administration of IVIG within 180 days prior to obtaining informed consent. 7)Treatment with steroids (except external preparations), steroid pulse, biological agents, neutrophil elastase inhibitors, immunosuppressants, or plasmapheresis within 30 days of screening. 8)History of hypersensitivity to CsA preparations, immunoglobulin preparations, or aspirin. 9)Having had treatment with tacrolimus, pitavastatin, rosuvastatin, bosentan, aliskiren, asunaprevir or vaniprevir. 10)Aspartate aminotransferase or, alanine aminotransferase values of 500 IU/L or higher. 11)An estimated glomerular filtration rate of 50 mL/min/1.73 m2 or lower. 12)Presence of an active bacterial infection: septicaemia, meningitis purulenta, peritonitis or bacterial pneumonia. 13)Treatment with other investigational drugs within 12 weeks of study commencement. 14)Others: patients who are judged to be inappropriate for participants of the clinical study for safety reasons by the investigators or the sub-investigators.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Frequency of CAAs during the study period (central review of echocardiography data) | — |
Secondary
| Measure | Time frame |
|---|---|
| [Efficacy] 1)Frequency of CAA at week 4 2)Frequency of treatment resistance (initial treatment unresponsiveness or relapse during the 12 weeks after treatment initiation.) 3)Z scores for the right coronary artery, and the left main coronary trunk and anterior descending artery. 4)Fever duration 5)Changes in body temperature, frequency of defervescence. 6)Change in serum concentration of C reactive protein (CRP). 7)Genotype frequency of ITPKC and CASP3 SNPs. 8)Additional treatment and follow-up treatment. [Safety] 1)Frequency of AEs | — |
Countries
Japan
Contacts
Public ContactYasuhisa Fujii
Chiba University Hospital
Outcome results
None listed