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Inherited complement deficiencies.

P J Lachmann

    Philosophical Transactions of the Royal Society of London. Series B, Biological Sciences
    |September 6, 1984
    PubMed
    Summary
    This summary is machine-generated.

    Complement deficiencies increase susceptibility to bacterial infections, particularly Neisseria, and are linked to immune-complex diseases like lupus. The complement system is crucial for fighting infections and managing immune responses.

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    Area of Science:

    • Immunology
    • Genetics

    Background:

    • Genetic deficiencies in complement system components are known for classical pathway, membrane attack complex, Factor I, Factor H, and properdin.
    • Homozygous deficiencies for Factor B and Factor D of the alternative pathway remain uncharacterized.
    • Complement deficiency studies offer direct insights into the in vivo role of the complement system.

    Purpose of the Study:

    • To review the known complement deficiency states and their clinical implications.
    • To highlight the role of complement in resistance to bacterial infections, especially Neisseria.
    • To explore the unexpected association between complement deficiencies and immune-complex diseases.

    Main Methods:

    • Review of existing literature on complement deficiencies.
    • Analysis of in vitro and in vivo studies on complement function.

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  • Discussion of the pathogenesis of infections and immune-complex diseases in complement-deficient individuals.

    • Main Results:

    • Complement deficiencies are strongly associated with increased susceptibility to bacterial infections, particularly Neisseria, due to impaired phagocytosis and inflammation.
    • An unexpected link exists between complement deficiencies and immune-complex diseases, including systemic lupus erythematosus.
    • The complement system is vital for immune complex solubilization and clearance, and its deficiency can lead to disease persistence.

    Conclusions:

    • The complement system plays a critical role in host defense against bacterial pathogens.
    • Complement deficiencies contribute to the development of immune-complex diseases through impaired immune complex handling.
    • Understanding complement deficiencies provides crucial insights into immune system function and disease pathogenesis.