Deficiency of complement components

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Primary immunodeficiency

• Disorders of all individual components of complement,
• rare disorders,
• C1, C2, C3 and C4 disorders manifest as SLE-like immunocomplex diseases; combined with purulent infections,
• C1 inhibitor defects clinically severe → hereditary angioedema: uncontrolled complement activation occurs after various stimuli → local edema; airway swelling can be fatal,
• disorders in receptors for complement components include LAD I syndrome (affects CR3),
• in children with recurrent bacterial and fungal infections, a deficiency of mannose-binding protein (lectin) (MBL), which initiates the lectin pathway of complement activation, has been identified; in adults it is not manifested (compensated by other mechanisms).

Secondary immunodeficiency

• Reduction of complement components during its depletion, in case of synthesis failure,
• complement consumption in immunocomplex diseases, septic conditions,
• in severe liver disease,
  • normalization of liver functions → normalization of complement,
• transient consumption of C4 in attacks of hereditary angioedema.

Sources

• HOŘEJŠÍ, Václav – BARTŮŇKOVÁ, Jiřina. Základy imunologie. 3. edition. Praha : Triton, 2008. 280 pp. ISBN 80-7254-686-4.