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Published on: April 9, 2018
Classical pathway complement activation in Kawasaki syndrome
1National Childrens' Hospital, Childrens' Medical Research Center, Department of Immunology, Tokyo, Japan.
Insights
Complement activation via the classical pathway occurs in Kawasaki syndrome, indicated by breakdown products. Increased production of complement components compensates for activation, maintaining normal serum levels during inflammation.
Area of Science:
- Immunology
- Pediatric Medicine
- Complement System
Background:
- Kawasaki syndrome is an acute febrile illness affecting young children.
- The complement system plays a crucial role in innate and adaptive immunity.
- Understanding complement activation in Kawasaki syndrome is vital for disease management.
Purpose of the Study:
- To investigate complement activation pathways in patients with Kawasaki syndrome.
- To examine the relationship between complement components and cytokine levels.
- To determine if complement consumption occurs during the inflammatory process.
Main Methods:
- Measurement of complement breakdown products (C3d, C4d, Bb, MAC) in patient plasma.
- Assessment of hemolytic titers and intact complement protein concentrations (C3, C4, B).
- Correlation analysis between serum cytokine levels (IL-6) and complement components.
Main Results:
- Strong evidence of classical complement pathway activation was observed.
- No significant decrease in hemolytic titer or intact complement proteins (C3, C4, B) was detected.
- Elevated interleukin-6 levels correlated with C3 and B concentrations 5 days later.
Conclusions:
- Complement activation via the classical pathway is implicated in Kawasaki syndrome.
- Increased production of complement components accompanies the inflammatory response.
- Activation products increase without a net decrease in serum complement levels due to compensatory production.
Abstract:
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.
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