Abstract

Kawasaki disease (KD) is a febrile acute systemic vasculitis in childhood, primarily affecting coronary arteries. Different causes are discussed including infectious agents, cofactors, and genetic predisposition. We tested the hypothesis that polymorphisms of TL-2 and TLR4, key mediators of the innate immune system influence coronary artery involvement in KD patients. 15 patients with KD in childhood were revaluated in young adulthood including the analysis of TLR2 and TLR4 regarding expression pattern, stimulation effects and polymorphisms (SNP) compared to an ageand sex related control group.Heterozygote Genotype for TLR2 SNP was found in two KD patients, one also positive for TLR4 SNP, both had no acute or late cardiac symptoms. The expression pattern on the surface of monocytes showed no difference between the KD and control group, nor in the KD patients with or without cardiac pathology. Stimulation via TLR2 and TLR4 also showed no significant influence on p38 and ERK phosphorylation and cytokine induction.We can conclude that there is no disturbance in the innate immune system of previous patients with KD, but a modest positive effect of TLR2 and TLR4 SNPs on coronary involvement can be discussed.

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