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Related Experiment Videos

Circulating immune complexes in cystic fibrosis.

M Berdischewsky, M Pollack, L S Young

    Pediatric Research
    |June 1, 1980
    PubMed
    Summary
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    Circulating immune complexes (CIC) are common in advanced cystic fibrosis patients with Pseudomonas aeruginosa infections. These complexes show significant enrichment of antibodies against lipopolysaccharide (LPS) and endotoxin-like activity.

    Area of Science:

    • Immunology
    • Microbiology
    • Pulmonology

    Background:

    • Cystic fibrosis (CF) patients often experience recurrent respiratory infections, particularly with mucoid Pseudomonas aeruginosa in advanced stages.
    • Chronic antigenic stimulation from persistent bacterial infections may lead to the formation of circulating immune complexes (CIC).

    Purpose of the Study:

    • To investigate the association between chronic antigenic stimulation and the presence of CIC in cystic fibrosis patients.
    • To characterize the antibody content and endotoxin-like activity within CIC found in advanced CF.

    Main Methods:

    • Sera from 20 hospitalized CF patients were analyzed for CIC levels using polyethylene glycol precipitation.
    • Antibody titers against Pseudomonas aeruginosa lipopolysaccharide (LPS) and exotoxin A were measured in serum, supernatant, and precipitates.

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  • Immune complex enrichment ratios and endotoxin-like activity (Limulus test) were assessed.

    • Main Results:

    • Elevated CIC levels (> 310 µg IgG/ml) were detected in 18 out of 20 patients.
    • Markedly elevated serum antibody titers against LPS and exotoxin A were observed.
    • CIC demonstrated significant enrichment (mean ratio > 13) for anti-LPS antibodies but not anti-exotoxin A antibodies.
    • Boiled precipitates showed positive endotoxin-like activity at dilutions > 1:8, while supernatants were negative.

    Conclusions:

    • Circulating immune complexes are prevalent in advanced cystic fibrosis.
    • CIC in CF patients are significantly enriched with antibodies targeting Pseudomonas aeruginosa LPS.
    • The presence of endotoxin-like activity within CIC suggests a role in the inflammatory process of advanced CF.