Corticosteroid treatment of refractory Kawasaki disease.
OBJECTIVE
To review the indications for corticosteroids in patients with Kawasaki disease (KD) treated by pediatric rheumatologists in Canada and to determine their efficacy on fever in patients with refractory KD.


METHODS
All practicing pediatric rheumatologists in Canada identified KD patients treated with corticosteroids and completed a standard data form that included demographics, clinical and laboratory features, imaging studies, and therapeutic interventions, by chart review.


RESULTS
Thirty-two patients with KD (14 female; 18 male: mean age 4.6 years) were treated with corticosteroids. Corticosteroids were used in 26 patients (81%) for persistent fever despite treatment with intravenous immunoglobulin (IVIG) (refractory KD), 5 patients (19%) for congestive heart failure, and 1 patient for persistent acute phase symptoms other than fever. The 26 patients with refractory KD are the primary subject of this report. Twenty-two patients (85%) had rapid, sustained resolution of fever after corticosteroids. There were no serious reported adverse effects. Eight patients (31%) treated with corticosteroids developed coronary artery (CA) aneurysms and 9 (35%) developed CA dilatations without aneurysms. Of those who developed CA aneurysm, 4 had aneurysms detected prior to IV methylprednisolone (MP) on echocardiograms performed on days 6-27 (mean day 13) of illness. The remaining 4 patients had CA aneurysm detected after IVMP therapy, on echocardiograms performed on days 13-49 (mean day 23) of illness, 1-25 days (mean 9 days) after IVMP. In patients with one year or more of followup, 46% had resolution of CA abnormalities.


CONCLUSIONS
Corticosteroids are effective in the treatment of fever in most patients with IVIG-refractory KD. A multicenter prospective study is needed to determine the effect of corticosteroids on CA outcome in patients with refractory KD.
Canadian Pediatric Rheumatology Association, Dalhousie University, IWK Health Centre, Halifax, Nova Scotia, Canada. bianca.lang@iwk.nshealth.ca
Bianca A
BA
Lang
Rae S M
RS
Yeung
Kiem G
KG
Oen
Peter N
PN
Malleson
Adam M
AM
Huber
Mark
M
Riley
Regan
R
Ebbeson
Suzanne E
SE
Ramsey
Ronald M
RM
Laxer
Earl D
ED
Silverman
Brian W
BW
McCrindle
Savithiri
S
Ratnapalan
Brian M
BM
Feldman
D002648:Child; D002675:Child, Preschool; D005260:Female; D005334:Fever / Q000188:drug therapy; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D007223:Infant; D007275:Injections, Intravenous; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use*; D009080:Mucocutaneous Lymph Node Syndrome / Q000175:diagnosis / Q000188:drug therapy* / Q000503:physiopathology; D010372:Pediatrics / Q000379:methods*; D012008:Recurrence; D012189:Retrospective Studies; D017211:Treatment Failure; D016896:Treatment Outcome
D005938:Glucocorticoids; D008775:Methylprednisolone