Clinical question: How is refractory Kawasaki disease (rKD) treated in the United States?
Background: Kawasaki disease (KD) is an immunologically mediated disease of primarily small to medium-sized arteries. It is the most common cause of acquired heart disease in children in the United States.
The current standard of care for KD treatment is a single 2 g/kg dose of intravenous immunoglobulin (IVIG), infused over 10 to 12 hours, accompanied by aspirin (80 to 100 mg/kg/day by mouth in four divided doses). Fevers persistent more than 36 hours after initial treatment represent refractory Kawasaki disease (rKD). There are no current national guidelines or standards for rKD treatment, although a 2004 joint statement from the American Academy of Pediatrics and the American Heart Association suggested a second dose of IVIG for rKD.
Study design: Multicenter, retrospective, cross-sectional study.
Setting: Forty freestanding children’s hospitals.
Synopsis: Researchers examined data obtained from the Pediatric Health Information System (PHIS), a clinical and financial database of care provided at 43 nonprofit, freestanding children’s hospitals in the United States. Data from 40 of these hospitals were deemed complete enough for analysis and were collected from Jan. 1, 2005, to June 30, 2009. Subjects were included if they received at least one dose of IVIG and had a principal diagnosis of KD. To be considered rKD, the subject must have received additional treatment after the initial diagnosis of rKD.
The most commonly used treatment after initial IVIG treatment was retreatment with IVIG (65%), followed by intravenous methylprednisolone (27%), then infliximab (8%). Significant regional variation was observed, with hospitals in the Northeast using methylprednisolone most frequently for rKD (55%). Infliximab was used at a much higher frequency in the West (29%) compared with other regions.
Bottom line: Retreatment with IVIG is the most common treatment for rKD, but significant regional variation exists, possibly due to the influence of regional experts.
Citation: Ghelani SJ, Pastor W, Parikh K. Demographic and treatment variability of refractory Kawasaki Disease: a multicenter analysis from 2005 to 2009. Hospital Pediatrics. 2012;2:71-76.
Reviewed by Pediatric Editor Weijen Chang, MD, SFHM, FAAP, associate clinical professor of medicine and pediatrics at the University of California at San Diego School of Medicine, and a hospitalist at both UCSD Medical Center and Rady Children’s Hospital.