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Kawasaki Disease (Mucocutaneous...
PEDIATRICS Vol. 111 No. 2 February 2003, pp. e140-e147

ELECTRONIC ARTICLE

Expression of CD40 Ligand on CD4+ T-Cells and Platelets Correlated to the Coronary Artery Lesion and Disease Progress in Kawasaki Disease

Objective.Kawasaki disease (KD) is an acute febrile vasculitic syndrome in children. CD40 ligand (CD40L) has been implicated in certain types of vasculitis. We proposed that CD40L expression might be correlated with coronary artery lesions in KD.

Methods.Blood samples were collected from 43 patients with KD before intravenous immunoglobulin (IVIG) treatment and 3 days afterward. Forty-three age-matched febrile children with various diseases were studied in parallel as controls. CD40L expression on T-cells and platelets were detected by flow cytometry, and soluble CD40L (sCD40L) levels were measured by enzyme-linked immunosorbent assay.

Results.We found that CD40L expression on CD4+ T-cells was significantly higher in patients with KD than in the febrile control (FC) group (28.69 ± 1.17% vs 4.37 ± 0.36%). CD40L expression decreased significantly 3 days after IVIG administration (28.69 ± 1.17% vs 13.53 ± 0.55%). CD40L expression on platelets from patients with KD was also significantly higher than in the FC group (8.20 ± 0.41% vs 1.26 ± 0.12%) and decreased after IVIG therapy. sCD40L levels were also significantly higher in KD patients with those of FC (9.69 ± 0.45 ng/mL vs 2.25 ± 0.19 ng/mL) but were not affected by IVIG treatment 3 days afterward (9.69 ± 0.45 ng/mL vs 9.03 ± 0.32 ng/mL). More interesting, we found that in KD patients, CD40L expression on CD4+ T-cells and platelets but not on CD8+ T-cells or sCD40L was correlated with the occurrence of coronary artery lesions.

Conclusions.CD40L might play a role in the immunopathogenesis of KD. IVIG therapy might downregulate CD40L expression, resulting in decrease of CD40L-mediated vascular damage in KD. This implicates that modulation of CD40L expression may benefit to treat KD vasculitis.

Chih-Lu Wang, MD*, Yu-Tsun Wu, MD{ddagger}, Chieh-An Liu, MD*, Mei-Wei Lin, BS*, Chia-Jung Lee, BS*, Li-Tung Huang, MD* and Kuender D. Yang, MD, PhD*

* Divisions of Allergy and Immunology
{ddagger} Cardiology, Chang-Gung Children’s Hospital at Kaohsiung, Chang-Gung University, Kaohsiung, Taiwan

Key Words: Kawasaki disease • intravenous immunoglobulin • CD40 ligand • soluble CD40L

Abbreviations: KD, Kawasaki disease • CD40L, CD40 ligand • IgG, immunoglobulin G • sCD40L, soluble CD40L • FC, febrile controls • IVIG, intravenous immunoglobulin • CAL, coronary artery lesions • FITC, fluorescein isothiocyanate • PE, phycoerythrin • PBS, phosphate-buffered saline • PRP, platelet-rich plasma • IL, interleukin • TSST-1, toxic shock syndrome toxin-1

Received for publication Jul 3, 2002; Accepted Oct 2, 2002.