Language
English
Publication Date
12-16-2025
Journal
Children
DOI
10.3390/children12121695
PMID
41462837
PMCID
PMC12731380
PubMedCentral® Posted Date
12-16-2025
PubMedCentral® Full Text Version
Post-print
Abstract
Background: The best treatment for children with KD who fail to respond to the first dose of IVIG (refractory KD) is currently unknown. The purpose of this study was to determine treatment practices of pediatric rheumatologists in North America who manage IVIG-refractory KD.
Methods: A 34-item web-based survey was sent to 102 randomly selected members of the Childhood Arthritis and Rheumatology Research Alliance (CARRA). The anonymous survey addressed the use of primary intensification as well as the treatment of IVIG-refractory KD.
Results: The response rate was 82%; 56% (all pediatric rheumatologists) completed the survey. Primary intensification was used for macrophage activation syndrome (MAS), KD shock, and those at high risk for coronary artery aneurysms (CAAs) by 84%, 76% and 52% of responders, respectively, with corticosteroids (CSs) used most frequently. For IVIG-refractory KD without CAA, a second dose of IVIG was used most often (63% alone; 23% plus CS). With non-giant CAAs, only 15% used a second IVIG alone, 40% used IVIG plus CS, and 35% took infliximab, usually with CS/IVIG. With giant CAA, treatments used most frequently were CS, a second IVIG, and infliximab (91%, 69%, and 58%, respectively), usually as combinations of two or more medications.
Conclusions: Treatment of IVIG-refractory KD varies significantly among North American pediatric rheumatologists, particularly in the presence of CAAs. Our findings emphasize the need for research to identify the most effective therapy for this KD subgroup. The current use of primary intensification and the presence and size of the CAA will need to be considered as consensus treatment plans are developed.
Keywords
Kawasaki disease, IVIG-refractory, coronary artery aneurysm, treatment variation, consensus treatment plan, primary intensification
Published Open-Access
yes
Recommended Citation
Ibanez, Daniel; Lang, Bianca; Shalen, Julia; et al., "Clinical Practice Variation Among Pediatric Rheumatologists Treating Kawasaki Disease: Results of a North American Survey" (2025). Faculty, Staff and Students Publications. 5894.
https://digitalcommons.library.tmc.edu/baylor_docs/5894