Abstract
BACKGROUND: Microscopic evaluation of arteritis in Kawasaki disease has demonstrated infiltration of the intima, muscularis and adventitia by predominantly mononuclear leukocytes; accompanied by edema, and, in severe lesions, by fibrinoid necrosis. There is limited published information on the phenotype of infiltrating leukocytes. The purpose of this study was to perform immunophenotyping of the leukocytes causing arteritis in kidneys from patients with Kawasaki disease. METHODS: Seven postmortem kidney specimens from children with Kawasaki disease were examined by light microscopy. Three of the seven specimens were selected for leukocyte immunophenotyping because they had a range of acute inflammation in arteries (mild, moderate, and severe arteritis). Only the specimen with severe arteritis had fibrinoid necrosis. Formalin-fixed sections were stained for immunoenzyme microscopy using antibodies specific for CD3 (T lymphocytes), CD4 (helper T lymphocytes), CD8 (cytotoxic T lymphocytes), CD20 (B lymphocytes), and CD68 (monocytes and macrophages). RESULTS: Arteritis was confined to the interlobar arteries. The arteritic infiltrates had an extremely high proportion of CD68 positive cells, most with the morphology of macrophages. Apparently early intimal lesions had almost exclusively CD68 positive cells beneath the endothelium. Most infiltrates in the intima, muscularis, and adventitia had approximately 90% CD68 positive cells. Most of the remaining cells were positive for CD3 (CD8CD4). CD20 positive cells were rare. Another interesting finding was increased numbers of CD68 positive cells in glomeruli. The severe arteritis specimen had 1.38 CD68 cells/glomerulus, moderate arteritis specimen 2.33 CD68 cells/ glomerulus, and mild arteritis specimen 0.04 CD68 cells/ glomerulus. CONCLUSIONS: The mural and perivascular leukocytes in early as well as advanced acute Kawasaki disease arteritis are predominantly monocytes/macrophages. Although there is no overt glomerulonephritis, patients with moderate and severe renal arteritis have increased monocytes/macrophages in glomeruli. These data suggest that the pathogenesis of Kawasaki disease involves extensive monocyte activation.
Article PDF
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Jennette, J., Sciarrotta, J., Takahashi, K. et al. Predominance of Monocytes and Macrophages in the Inflammatory Infiltrates of Acute Kawasaki Disease Arteritis. Pediatr Res 53, 173 (2003). https://doi.org/10.1203/00006450-200301000-00120
Issue Date:
DOI: https://doi.org/10.1203/00006450-200301000-00120