In this study the complement breakdown products C3d, C4d, Bb and membr
ane attack complex were measured in plasma of patients with Kawasaki s
yndrome. The results suggested strong activation of the classical acti
vation pathway. However, there was no significant decrease in hemolyti
c titer or in the concentrations of the intact proteins C3, C4, and B.
The relationship between the serum concentrations of cytokines and co
mplement components was examined; increased interleukin-6 concentratio
n on the fifth day after the onset of fever was found to correlate wel
l with the C3 and B concentrations in serum obtained 5 days later We c
onclude that complement activation occurred in Kawasaki syndrome via t
he classical pathway but that the inflammatory reaction was accompanie
d by increased production of complement components. As a result, there
was increased formation of activation products without changes in the
serum complement levels.