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In vitro complement activation by intercellular antibodies

T Hashimoto, M Sugiura, S Kurihara

    The Journal of Investigative Dermatology
    |April 1, 1982
    PubMed
    Summary
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    Pemphigus antibodies activate the complement system via the classical pathway, leading to C3 amplification. This study clarifies the in vitro complement fixation mechanisms in pemphigus sera.

    Area of Science:

    • Immunology
    • Autoimmune Diseases
    • Complement System

    Background:

    • Pemphigus is an autoimmune blistering disease characterized by autoantibodies against desmosomal proteins.
    • The complement system plays a role in the pathogenesis of autoimmune diseases, but its activation pathway in pemphigus is not fully elucidated.

    Purpose of the Study:

    • To investigate the in vitro complement fixation profile of pemphigus sera.
    • To determine the complement activation pathway initiated by intercellular antibodies in pemphigus.

    Main Methods:

    • In vitro complement immunofluorescence assays were performed on 6 pemphigus sera.
    • The fixation of complement components C1q, C4, C3, and properdin was assessed.
    • Experiments using C2-deficient serum were conducted to differentiate between classical and alternative complement pathways.

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    Main Results:

    • All 6 pemphigus sera showed positive C3 staining.
    • Positive staining for C1q, C4, and properdin was observed in 3, 5, and 3 sera, respectively.
    • C3 and properdin staining were inhibited by C2-deficient serum, while C1q and C4 staining remained positive.

    Conclusions:

    • Complement activation by intercellular antibodies in pemphigus occurs primarily through the classical pathway.
    • The classical pathway activation is followed by the assembly of the C3 amplification mechanism.
    • These findings contribute to understanding the immunopathogenesis of pemphigus.