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Complement components and activation in primary biliary cirrhosis.

S Lindgren, A B Laurell, S Eriksson

    Hepatology (Baltimore, Md.)
    |January 1, 1984
    PubMed
    Summary
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    Primary biliary cirrhosis commonly activates the classical complement pathway, evidenced by specific protein complexes and elevated complement factors. This activation appears linked to cholestasis rather than immune complexes.

    Area of Science:

    • Immunology
    • Hepatology
    • Biochemistry

    Background:

    • Primary biliary cirrhosis (PBC) is a chronic liver disease with an autoimmune component.
    • The role of the complement system in PBC pathogenesis is not fully understood.
    • Understanding complement activation pathways may reveal therapeutic targets.

    Purpose of the Study:

    • To investigate complement system activation in patients with primary biliary cirrhosis.
    • To determine whether classical or alternative complement pathways are involved.
    • To explore the relationship between complement component levels and disease characteristics.

    Main Methods:

    • Analysis of total complement activity using two hemolytic assays.
    • Detection of complement pathway activation through specific protein complex formation (C1r, C1s, C1 inactivator).

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  • Quantification of complement components (C1q, C4, C1 inactivator, C2, C3, factor B, factor H) and ceruloplasmin levels.

    • Main Results:

    • Total complement activity was normal, but classical pathway activation was evident in all patients.
    • Elevated levels of free C1q and several complement components (C1 inactivator, C2, C3, factor B, factor H) were observed.
    • Increased complement factors correlated with ceruloplasmin, suggesting a link to cholestasis, not immune complexes.

    Conclusions:

    • Classical complement pathway activation is a common feature in primary biliary cirrhosis.
    • Elevated complement component levels may be secondary to cholestasis.
    • Further research into complement-mediated mechanisms in PBC is warranted.