All issues > Volume 39(5); 1996

Original Article

J Korean Pediatr Soc. 1996;39(5):691-702. Published online May 15, 1996.

Immunologic Abnormalitiesin Kawasaki Diseaseand the Effect ofIntravenousImmunoglobulin

Joon Sung JS Lee¹, Bin B Cho¹, Sung Hoon SH Cho¹

¹Department of Pediatrics, Catholic University Medical College, Seoul, Korea

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.

Keywords :Kawasaki disease, Coronary vascular lesion, Intravenous immunoglobulin(IVIG), Immunologic abnormalities