Classical pathway complement activation in Kawasaki syndrome.
In this study the complement breakdown products C3d, C4d, Bb and membrane attack complex were measured in plasma of patients with Kawasaki syndrome. The results suggested strong activation of the classical activation pathway. However, there was no significant decrease in hemolytic titer or in the concentrations of the intact proteins C3, C4, and B. The relationship between the serum concentrations of cytokines and complement components was examined; increased interleukin-6 concentration on the fifth day after the onset of fever was found to correlate well with the C3 and B concentrations in serum obtained 5 days later. We conclude that complement activation occurred in Kawasaki syndrome via the classical pathway but that the inflammatory reaction was accompanied by increased production of complement components. As a result, there was increased formation of activation products without changes in the serum complement levels.
Kohsaka
T
T
National Childrens' Hospital, Childrens' Medical Research Center, Department of Immunology, Tokyo, Japan.
Abe
J
J
Asahina
T
T
Kobayashi
N
N
D002675:Child, Preschool; D051561:Complement C3 Convertase, Alternative Pathway; D003179:Complement C3b / Q000378:metabolism; D015933:Complement C3d / Q000378:metabolism; D003181:Complement C4 / Q000378:metabolism; D015935:Complement C4b; D015938:Complement Membrane Attack Complex / Q000378:metabolism; D003171:Complement Pathway, Classical; D005334:Fever / Q000276:immunology; D006801:Humans; D007223:Infant; D015850:Interleukin-6 / Q000097:blood; D009080:Mucocutaneous Lymph Node Syndrome / Q000209:etiology / Q000276:immunology*; D010446:Peptide Fragments / Q000378:metabolism; D013997:Time Factors
D003181:Complement C4; D015938:Complement Membrane Attack Complex; D015850:Interleukin-6; D010446:Peptide Fragments; D003179:Complement C3b; D015933:Complement C3d; D015935:Complement C4b; C032261:complement C4d; D051561:Complement C3 Convertase, Alternative Pathway