Abstract
Introduction: Inadequate handling or clearance of microbial antigens is one of the explanations for the beneficial effect of IVIG in Kawasaki Disease. In case of antigen clearance by IgG, Fcγ-receptor (FcγR)-dependent mechanisms were logical targets for study. There are various FcγR types, each with a different expression pattern among leukocytes. Relevant amino acid substitutions or polymorphisms are known in the FcγRIIa, FcγRIIIA and FcγRIIIB genes, which define receptor affinity for IgG. Materials and Methods: Gene polymorphisms were determined by PCR techniques using specific primers for the FcγRIIA, FcγRIIIA and FcγRIIIB alleles. Results: In a cohort of Caucasian KD patients (n=106), FcγR polymorphisms showed differences in genotype distribution compared to a group of 87 healthy controls. A clear shift towards FcγRIIIA-158F/F was seen in our group compared to the control (41% versus 32%, p=0.08). No such differences in the genotype distribution of FcγRIIA-131R/H and FcγRIIIB-NA (1,2) were detected. A relation to IVIG efficacy and the occurrence of CAA was not found. The FcγRIIIA is expressed on Natural Killer cells and macrophages. The FcγRIIIA-158F isoform is known to have a lower affinity for IgG (1, 3, 4 subclasses) than the FcγRIIIA-158V isoform. Overexpression of this isoform might therefore result in a less effective handling of endogenous IgG or IVIG and a higher risk for the development of KD.
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Biezeveld, M., Kuipers, I., Geissler, J. et al. Association of FC Γ Receptor Polymorphisms in Kawasaki Disease. Pediatr Res 53, 165 (2003). https://doi.org/10.1203/00006450-200301000-00072
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DOI: https://doi.org/10.1203/00006450-200301000-00072