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Elevated granulocyte colony-stimulating factor levels predict treatment failure in patients with Kawasaki disease
Jun Abe1, Ryota Ebata, Toshiaki Jibiki
1Department of Allergy and Immunology, National Research Institute for Child Health and Development, Tokyo, Japan. jabe@nch.go.jp
Insights
Kawasaki disease (KD) treatment with intravenous immunoglobulin (IVIG) effectiveness varies. Gene expression profiling identified biomarkers, including Polycythemia rubra vera 1 and granulocyte colony-stimulating factor, to predict IVIG response in children with KD.
Area of Science:
- Pediatric Rheumatology
- Immunology
- Genomics
Background:
- Kawasaki disease (KD) is a critical pediatric vasculitis linked to coronary artery aneurysms.
- Intravenous immunoglobulin (IVIG) therapy mitigates KD inflammation and coronary issues, but its mechanisms remain unclear.
Purpose of the Study:
- Investigate IVIG mechanisms in KD using gene expression profiling.
- Identify biomarkers predicting IVIG unresponsiveness in KD patients.
Main Methods:
- Compared whole blood cell transcript abundance pre- and post-IVIG, and with febrile controls.
- Analyzed mRNA and protein levels in KD patients responsive versus nonresponsive to IVIG.
Main Results:
- Identified 298 differentially expressed transcripts between pre-IVIG and post-IVIG/control groups.
- Found 15 transcripts differed between IVIG-responsive and nonresponsive KD patients pre-treatment.
- Elevated Polycythemia rubra vera 1 and granulocyte colony-stimulating factor (G-CSF) in nonresponsive patients pre-IVIG.
Conclusions:
- Variable gene expression profiles correlate with KD patient response to IVIG.
- Polycythemia rubra vera 1 and G-CSF show potential as biomarkers for predicting IVIG response in KD.
Background:
Kawasaki disease (KD) is an acute vasculitis in young children, frequently associated with coronary artery aneurysms. The intravenous infusion of high-dose IgG (IVIG) effectively reduces the systemic inflammation and the incidence of coronary artery lesions, although the precise underlying mechanisms are unknown.
Objective:
We performed expression profiling of whole blood cells to investigate the mechanisms underlying the effect of IVIG and to identify biomarkers associated with unresponsiveness to IVIG.
Methods:
We compared the transcript abundance among pre-IVIG and post-IVIG patients and febrile control patients. Then we analyzed the mRNA levels and the protein levels among the different cohort of patients with KD who were either responsive or nonresponsive to the initial IVIG.
Results:
A total of 298 transcripts were overrepresented or underrepresented in the pre-IVIG patients compared with post-IVIG patients and febrile controls, of which 15 transcripts were differentially expressed in nonresponsive patients with KD compared with responsive patients before IVIG. The protein levels of polycythemia rubra vera 1, which was one of the most variably expressed transcripts in pre-IVIG patients, and the serum granulocyte colony-stimulating factor levels were significantly higher in nonresponsive patients than in responsive patients before the initial IVIG administration.
Conclusion:
These findings suggest that the variable gene expression profiles were correlated to the responses of patients with KD to IVIG administration. Polycythemia rubra vera 1 and granulocyte colony-stimulating factor levels may be good biomarkers for predicting response to IVIG in patients with KD.
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