Therapeutics
CAGRI YILDIRIM-TORUNER, MD
Assistant Professor of Pediatrics, Attending Physician in Pediatric Rheumatology
Baylor College of Medicine/ Texas Children's Hospital
Houston, Texas, United States
BACKGROUND: Optimal treatment for children with KD who fail to respond to the first dose of IVIG (refractory KD) is currently unknown. The purpose of this survey was to determine treatment preferences in North American CARRA centers to inform future development of consensus treatment plans (CTPs) for IVIG-refractory KD.
Methods: In March 2022, the CARRA KD Workgroup developed a 34-item web-based survey with clinical cases and sent it to 102 randomly selected CARRA members. The anonymous survey addressed use of intensified initial treatment, when patients should be treated for refractory KD, medication choices, and factors influencing choices. Respondents who had not treated refractory KD in the previous 3 years were excluded. (Figure1)
Results: The response rate was 82%: 57 pediatric rheumatologists completing the survey section on refractory KD treatment (Figure1). Intensified initial therapy was used for MAS, KD shock, and high-risk for CAA by 84%, 76% and 52% respectively, with corticosteroids (CS) used most frequently, followed by anakinra and infliximab (Figure2a-2b). Temperature > 100.4 F (38C) 36 hours after the first dose IVIG was considered refractory KD by 54% of respondents; 33% considered refractory KD at 24 hours post IVIG, and 11% at 48 hours. Therapeutic choices varied, particularly based on the presence of CAA:
No CAA: 84% would give a second dose of IVIG; 58% would give IVIG alone and 23% would give IVIG with CS. 7% would give only daily CS and 4% added Infliximab.
Non-giant CAA: 72% would give a second dose of IVIG; 14% would give IVIG alone, 40% would give IVIG and CS, and 14% would give IVIG, CS and Infliximab. 16% would give Infliximab and CS without additional IVIG.
Giant CAA: 67% would give a second dose of IVIG; 5% would treat with IVIG alone, 23% IVIG and CS, and 30% would give IVIG, CS and Infliximab. 21% would give Infliximab and CS, but not IVIG. There were 32 different treatment regimens among the respondents, primarily because of varying CS doses/routes. Other factors most commonly influencing treatment choices included MAS, COVID-19 related KD, increasing CA dimensions and age < 6 months.
Conclusions: Treatment of IVIG-refractory KD varies widely among North American pediatric rheumatologists, particularly in the presence of CAA. Our results support the need to conduct comparative effectiveness research to identify the most effective therapy for this KD subgroup. The current use of intensified therapy will need to be considered as CTPs are developed.