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[Clinical aspects of the complement system].

Hiroshi Tsukamoto1, Takahiko Horiuchi

  • 1Department of Medicine and Biosystemic Science, Kyushu University Graduate School of Medical Sciences, Fukuoka.

Rinsho Byori. the Japanese Journal of Clinical Pathology
|August 18, 2006
PubMed
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The complement system, crucial for immunity, involves three pathways and aids pathogen elimination. Defects in its early components are linked to autoimmune diseases like lupus, highlighting its therapeutic potential.

Area of Science:

  • The complement system is a vital part of innate immunity and antibody-dependent pathogen clearance.
  • It comprises over 30 proteins with classical, lectin, and alternative activation pathways.

Context:

  • Serum levels of CH50, C3, and C4 are key biomarkers for immune-mediated diseases.
  • Decreased CH50 with normal/low C3/C4 suggests specific autoimmune conditions, while elevated CH50 indicates general inflammation.
  • Defective clearance of immune complexes and apoptotic cells is increasingly linked to autoimmune disorders.

Purpose:

  • To explore the role of the complement system in immunity and disease pathogenesis.
  • To highlight the diagnostic utility of complement component levels in immune-mediated diseases.
  • To discuss the complement system as a therapeutic target for inflammatory and autoimmune conditions.

Summary:

Related Experiment Videos

  • Mice lacking Clq exhibit lupus-like symptoms, including autoantibodies and glomerulonephritis, due to impaired immune complex and apoptotic cell clearance.
  • This finding supports the link between early complement component deficiencies and systemic lupus erythematosus (SLE) in humans.
  • Mutations in Factor H, MCP, and Factor I genes are associated with atypical hemolytic uremic syndrome.

    • Impact:

    • Understanding complement pathways and defects is crucial for diagnosing and managing autoimmune and inflammatory diseases.
    • Targeting the complement system, particularly C5, offers promising therapeutic strategies.
    • Anti-C5 monoclonal antibodies like eculizumab show efficacy in treating conditions such as paroxysmal nocturnal hemoglobinuria and glomerulonephritis.