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Immune complexes and complement abnormalities in patients with cystic fibrosis. Increased mortality associated with

J J Wisnieski, E W Todd, R K Fuller

    The American Review of Respiratory Disease
    |October 1, 1985
    PubMed

    Insights

    Circulating immune complexes (CIC) and decreased alternative complement pathway (ACP) activity are linked to higher mortality in cystic fibrosis (CF) patients. Identifying these immune markers may improve CF patient outcomes.

    Area of Science:

    • Immunology
    • Pulmonology
    • Genetics

    Background:

    • Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs, primarily the lungs.
    • Immune system dysregulation, including complement abnormalities and immune complex formation, is increasingly recognized in CF pathogenesis.
    • The role of specific complement pathways and circulating immune complexes (CIC) in CF morbidity and mortality requires further elucidation.

    Purpose of the Study:

    • To investigate the prevalence of complement abnormalities and CIC in cystic fibrosis patients.
    • To determine the association between CIC, alternative complement pathway (ACP) activity, and patient mortality.
    • To identify potential humoral immune mechanisms contributing to CF progression.

    Main Methods:

    • Serum samples from 139 CF patients were analyzed for whole complement activity, ACP activity, and CIC.
    • Patients were monitored over a 5-year period for mortality.
    • Statistical analyses were performed to assess the correlation between immune markers and survival, including independent risk factor analysis.

    Main Results:

    • No consistent changes in whole complement activity were observed.
    • Circulating immune complexes (CIC) were detected in 29% of patients, and decreased ACP activity in 36%.
    • Mortality was significantly higher in patients with CIC (p<0.001) or decreased ACP activity (p<0.01). Patients with both had a 31% mortality rate, while those with neither had no deaths (p<0.001). CIC was an independent risk factor for death.

    Conclusions:

    • Humoral immune mechanisms, specifically the presence of CIC, are associated with increased mortality in CF patients.
    • Decreased ACP activity, while prevalent, was not an independent risk factor for death.
    • These findings suggest that immune complex formation may play a significant role in CF morbidity and mortality, offering potential therapeutic targets.

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