Abstract
Background: Kawasaki Disease (KD) is a pediatric vasculitis of which the pathogenesis is unclear. The hypothesis is that genetically pre-disposed children develop KD when they encounter a pathogen which remains most often unidentified or pathogen derived factors. Since age is a dominant factor, prior immune status in children could influence their reactivity and hence the acquisition of KD. We hypothesized that systemic immune responses early in life could protect against developing KD. With this study we tested whether the incidence of previous systemic cytomegalovirus (CMV) or Epstein-Barr virus (EBV) infection is lower in children with KD compared to healthy age-matched controls.Methods and Results: We compared 86 KD patients with an age-matched control group regarding CMV and EBV VCA IgG measurements (taken before or 9 months after IVIG treatment). We found that both CMV and EBV had an almost 2-fold lower seroprevalence in the KD population than in the control group.Conclusions: We suggest that an under-challenged immune system causes an altered immune reactivity which may affect the response to a pathological trigger causing KD in susceptible children.
Highlights
Kawasaki disease (KD) is a rare pediatric vasculitis of unknown etiology that can lead to coronary artery aneurysms (CAAs)
We found a lower prevalence of Epstein-Barr virus (EBV) VCA IgG in the KD group as compared to the control group i.e., 14.3% as compared to 28.0% in the age category 0.5–2 years; 20.7% compared to 43.9% in the 2–4 years old category; 17.6% compared to 51.9% in the 4–6 years old category and 25.0% compared to 59.7% in the 6–10 years old category
When combined we found a lower seroprevalence of CMV and EBV together, in the KD group compared to the control group, but these differences did not reach levels of statistical significance
Summary
Kawasaki disease (KD) is a rare pediatric vasculitis of unknown etiology that can lead to coronary artery aneurysms (CAAs). I.e., preferably within the first 10 days of illness with intravenous immunoglobulin (IVIG) and oral aspirin for a prolonged period of time, a decrease in the incidence of CAAs has been reported [3, 4] With this treatment still ∼2.4–27.8% of the KD patients develop CAAs [5,6,7,8,9,10,11]. The hypothesis is that genetically pre-disposed children develop KD when they encounter a pathogen which remains most often unidentified or pathogen derived factors. With this study we tested whether the incidence of previous systemic cytomegalovirus (CMV) or Epstein-Barr virus (EBV) infection is lower in children with KD compared to healthy age-matched controls
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