| Summary: | 博士 === 長庚大學 === 臨床醫學研究所 === 99 === Kawasaki disease (KD) is a systemic vasculitis syndrome mainly affects children less than 5 year-old. The treatment with high dose intravenous immunoglobulin (IVIG) and aspirin is the best treatment for KD but the mechanism is still under investigation. Appearance of allergic features and eosinophilia in KD was reported in the literature. This study was conducted to study whether T helper (Th) 2 immune response was involved in KD.
Results showed that peripheral blood eosinophils increased in acute phase of KD and surplus after IVIG treatment. The higher the eosinophil the lower the IVIG treatment failure rate and coronary artery lesions (CAL) formation in KD was found in this study. Further studies on the eosinophil related Th2 immune response including cytokines (IL-4, IL-5 and eotaxin) and the eosinophil activation marker (eosinophil cationic protein, ECP) changes in KD before and after IVIG treatment, we first reported an increase in eosinophilia-related Th2 cytokines, including IL-4, IL-5 and eotaxin in KD patients in comparison to controls. After IVIG treatment, the Th2 cytokines were even greater than before IVIG treatment and associated with CAL formation. This result suggests that a higher Th2 (IL-5 and eosinophil) response may protect KD patients from CAL formation.
We also studied the genetics association of immune modulator gene (cytotoxic T lymphocyte 4, CTLA-4) (+49 A/G and -318 C/T) polymorphism with Th2 immune response in KD patients with/without CAL formation. Polymorphisms of CTLA-4 (+49 A/G) and (-318 C/T) were not significantly different between normal children and patients with KD. The CTLA-4 (+49, GG genotype), however, was significantly associated with the protection of CAL formation, especially in female patients. Female KD patients with protective allele had higher Th2 immune response (IL-4, IL-5 and eosinophil).
In conclusion, we found that Th2 immune response played a role to protect KD patients from CAL formation, possibly through CTLA-4 gene polymorphism, that mediates higher IL-5, IL-4 and eosinophil cells.
|
|---|
