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Related Experiment Videos

Circulating immune complexes and complement activation in primary biliary cirrhosis.

J R Wands, J L Dienstag, A K Bhan

    The New England Journal of Medicine
    |February 2, 1978
    PubMed
    Summary
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    Patients with primary biliary cirrhosis (PBC) have high concentrations of circulating immune complexes that activate the complement system. These findings suggest a role for these specific immune complexes in PBC pathogenesis.

    Area of Science:

    • Immunology
    • Hepatology
    • Clinical Medicine

    Background:

    • Primary biliary cirrhosis (PBC) is a chronic liver disease with an autoimmune component.
    • The role of immune complexes in PBC pathogenesis is not fully understood.

    Purpose of the Study:

    • To investigate the presence and characteristics of circulating immune complexes in patients with PBC.
    • To determine if these immune complexes activate the complement system.

    Main Methods:

    • Evaluated 20 PBC patients and 7 controls with extrahepatic biliary obstruction.
    • Isolated immune complexes by cryoprecipitation and measured them using the Raji-cell radioimmunoassay.
    • Assessed complement-pathway activation.

    Main Results:

    Related Experiment Videos

    • 95% of PBC patients had high concentrations of immune complexes (mean 474 µg/mL), absent in controls.
    • Cryoproteins, composed of IgM, IgG-IgM, and IgA-IgM, were found in 90% of PBC patients.
    • Alternate complement pathway activation was detected in 8 PBC patients.

    Conclusions:

    • Circulating immune complexes are significantly elevated in PBC patients.
    • These immune complexes are capable of activating the complement system in vitro.
    • The identified complement-fixing immune complexes may play a crucial role in the pathogenesis of primary biliary cirrhosis.