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BIANCA A. LANG, RAE S.M. YEUNG, KIEM G. OEN, PETER N. MALLESON, ADAM M. HUBER, MARK RILEY, REGAN EBBESON, SUZANNE E. RAMSEY, RONALD M. LAXER, EARL D. SILVERMAN, BRIAN W. MCCRINDLE, SAVITHIRI RATNAPALAN, and BRIAN M. FELDMAN ABSTRACT. 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. Conclusion. 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. (J Rheumatol 2006;33:803–9)
Key Indexing Terms: KAWASAKI DISEASE
From the Canadian Pediatric Rheumatology Association; Dalhousie University, IWK Health Centre, Halifax, Nova Scotia; University of Toronto, The Hospital for Sick Children, and Bloorview MacMillan Children's Centre, Toronto, Ontario; University of Manitoba, Health Sciences Centre, Winnipeg, Manitoba; University of British Columbia, Children's and Women's Centre of British Columbia, Vancouver, British Columbia, Canada. B.A. Lang MD, FRCPC, Dalhousie University, IWK Health Centre; R.S.M. Yeung, MD, PhD, FRCPC, University of Toronto, The Hospital for Sick Children; K.G. Oen, MD, University of Manitoba, Health Sciences Centre; P.N. Malleson, MBBS, MRCP(UK), FRCPC, University of British Columbia, Children's and Women's Centre of British Columbia; A.M. Huber, MD, FRCPC, Dalhousie University, IWK Health Centre; M. Riley, MD, FRCPC; R. Ebbeson, MD, Children's and Women's Centre of British Columbia; S.E. Ramsey, MD, FRCPC, Dalhousie University, IWK Health Centre; R.M. Laxer, MD, FRCPC; E.D. Silverman, MD, FRCPC; B.W. McCrindle, MD, MPH, FRCPC; S. Ratnapalan, MBBS, M Ed, MRCP, FRCPC, FAAP, The Hospital for Sick Children; B.M. Feldman, MD, MSc, FRCPC, University of Toronto, Bloorview MacMillan Children's Centre. Address reprint requests to Dr. B.A. Lang, Division of Rheumatology, IWK Health Centre, 5850/5980 University Ave., P.O. Box 9700, Halifax, Nova Scotia, Canada, B3K 6R8. E-mail: bianca.lang@iwk.nshealth.ca Accepted for publication November 11, 2005.
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