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 |
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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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