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Hereditary complement (C2) deficiency with dermatomyositis.

J P Leddy, R C Griggs, M R Klemperer

    The American Journal of Medicine
    |January 1, 1975
    PubMed
    Summary
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    This study reports a dermatomyositis case in a patient lacking the second component of complement (C2). This highlights that inflammatory myopathy can occur despite a defective classic complement pathway, emphasizing the alternate pathway's role.

    Area of Science:

    • Immunology
    • Rheumatology
    • Genetics

    Background:

    • Dermatomyositis is an idiopathic inflammatory myopathy.
    • Hereditary complement deficiencies are increasingly associated with rheumatic diseases.
    • The classic complement pathway involves C1, C4, and C2 components.

    Purpose of the Study:

    • To investigate a case of dermatomyositis with an unusual complement defect.
    • To determine the functional consequences of C2 deficiency in this patient.
    • To explore the relationship between complement pathways and inflammatory myopathies.

    Main Methods:

    • Clinical assessment and muscle biopsy for dermatomyositis diagnosis.
    • Immunofluorescence assays for immunoglobulin and complement deposits.
    • Functional and immunoprecipitin assays to identify complement deficiency.

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  • Family studies to determine inheritance pattern.

    • Main Results:

    • The patient presented with typical dermatomyositis but lacked serum hemolytic complement activity.
    • Definitive diagnosis of total absence of the second component of complement (C2).
    • C2 deficiency was inherited in an autosomal recessive manner.
    • Normal chemotactic and bactericidal activity, suggesting reliance on the alternate complement pathway.

    Conclusions:

    • Dermatomyositis can manifest with a complete absence of C2, indicating the classic complement pathway is not essential for its development.
    • This case underscores the significance of the alternate complement pathway for immune functions like bacterial defense.
    • Associations between rheumatic diseases and hereditary complement deficiencies are important areas for further research.