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Immunologic Abnormalitiesin Kawasaki Diseaseand the Effect ofIntravenousImmunoglobulin

Journal of the Korean Pediatric Society 1996;39(5):691-702.
Published online May 15, 1996.
Immunologic Abnormalitiesin Kawasaki Diseaseand the Effect ofIntravenousImmunoglobulin
Joon Sung Lee, Bin Cho, Sung Hoon Cho
Department of Pediatrics, Catholic University Medical College, Seoul, Korea
가와사끼병의 면역이상소견과 정맥용 면역글로불린의 효과
이준성, 조빈, 조성훈
가톨릭대학교 의과대학 소아과학교실
Abstract
Purpose
: It has been suggested that immunologic abnormalities play a role in the pathogenesis of vascular injury in Kawasaki disease and the immunologic abnormalities could be regulated by high-dose intravenous immunoglobulin(IVIG). We investigated the peripheral blood lymphocyte subsets, the serum levels of interleukin-6(IL-6) and tumor necrosis factor-α(TNF-α) and the effect of high-dose IVIG(400 mg/kg/day for 5 days) on these factors during acute stage of Kawasaki disease.
Methods
: Thirty patients, aged 4 months to 5 years, who met the diagnostic criteria for Kawasaki disease were enrolled in this study. Patients were divided into two groups, Kawasaki disease with coronary vascular lesion(KD-CVL) and without coronary vascular lesion (KD-NCVL). Percentages of peripheral blood lymphocyte subsets were measured by flow-cytometry and theserum levels of IL-6 and TNF-α were measured by ELISA on 1 day before and 1 day after IVIG administration and compared with normal control group(n=15).
Results
: The results were as follows 1) Before IVIG administration, the percentages of CD3+ lymphocyte and CD8+ lymphocyte in both KD-CVL and KD-NCVL were significantly lower than those in control group. The percentages of CD19+ lymphocyte and CD4+/CD8+ ratio in both groups were significantly higher than those in control group. There was no significant differences between the percentages of CD4+ lymphocyte in both groups and that in control group. 2) After IVIG administration, the percentages of CD3+ lymphocyte and CD8+ lymphocyte in both KD-CVL and KD-NCVL were significantly increased. The percentages of CD19+ lymphocyte and CD4+/CD8+ ratio in both KD-CVL and KD-NCVL were significantly decreased. The percentages of CD4+ lymphocyte were not changed. 3) Before IVIG administration, the detection rate of serum IL-6 and TNF-α in both groups was significantly higher than that in control group. After IVIG administration, the detection rate of serum IL-6 in both groups was still higher than that in control group. In KD-CVL, the detection rate of serum TNF-α was still higher than that in control group but there was no significant difference between the detection rate of TNF-α in KD-NCVL and that incontrol group. 4) Before IVIG administration, the serum levels of IL-6 in Kawasaki disease were significantly higher than thatin control group but there was no significant difference between those in KD-CVL and KD-NCVL. After IVIG administration, the serum level of IL-6 in KD-CVL was not decreased. In KD-NCVL, it was decreased significantly but it was still higher than that in control group after IVIG administration. Before IVIG administration, the serum levels of TNF-α in Kawasaki diseases were significantly higher than that in control group and that in KD-CVL was significantly higher than that in KD-NCVL. After IVIG administration, the serum level of TNF-α in KD-CVL was not decreased butin KD-NCVL, it was decreased significantly to the level of control group.
Conclusion
: Immunologic abnormalities including decreased percentage of CD3+ lymphocyte, CD8+ lymphocyte, increased percentage of CD19+ lymphocyte and CD4+/ CD8+ ratio and increased levels of IL-6 and TNF-α were noted in the acute stage of Kawasaki diseases. But there were no difference of above immunologic abnormalities between KD-CVL and KD-NCVL. After IVIG administration, the abnormal percentages of peripheral blood lymphocyte subsets were changed to normal but in the cases of IL-6 and TNF-α, the levels were continuously elevated except TNF-α in KD-NCVL. Therefore the immunologic abnormalities including abnormal percentages of peripheral blood lymphocyte subsets and increased production of IL-6 and TNF-α might be involved in the pathogenesis of Kawasaki disease. And IVIG has some effects on the change of these kinds of immunologic abnormalities.
Key Words: Kawasaki disease, Coronary vascular lesion, Intravenous immunoglobulin(IVIG), Immunologic abnormalities


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