Kawasaki disease is an acute multisystem vasculitic syndrome of unknown etiology occurring mostly in infants and children younger than 5 years of age. In developed countries, Kawasaki disease is currently the leading cause of acquired heart diseases in children. However, it is still a mysterious disease. In this article, we reviewed and summarized from the aspects based on infection agents, host immune dysregulation and genetic background intended to establish a feasible infection-immunogenetic pathogenesis for this mysterious disease and also provided the rational strategy to explore optimal treatment of this disease.
ABSTRACT. 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 agematched febrile children with various diseases were studied in parallel as controls. CD40L expression on Tcells 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. Pediatrics 2003;111:e140 -e147. URL: http://www. pediatrics.org/cgi/content/full/111/2/e140; 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. K awasaki disease (KD) is an acute multisystem vasculitic syndrome of unknown cause that occurs in infants and children. 1 Evidence increasingly suggests that immunoregulatory activation with vascular endothelial inflammation may be involved in the immunopathogenesis of KD. 2 The acute stage of KD is associated with overactivation of numerous immunologic parameters, such as immune-competent cell activation, 3-5 cytokines, 6 nitric oxide production, 7 autoantibody production, 8 and adhesion molecule expression. 9 Pathologic examination of acute coronary arteritis in the acute stage of KD showed that KD vascular lesion formation is an activated T-lymphocyte-dependent process characterized by transmural infiltration of activated T-lymphocytes, with CD8ϩ T...
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