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Inherited deficiencies of the terminal components of human complement
R Würzner1, A Orren, P J Lachmann
1Molecular Immunopathology Unit, Medical Research Council Centre, Cambridge, UK.
Immunodeficiency Reviews
|January 1, 1992
Summary
Terminal complement deficiencies are linked to Neisserial infections, but prevalence varies geographically. Further research is needed to fully understand these complement system defects and their disease associations.
Area of Science:
- Immunology
- Infectious Diseases
Background:
- Terminal complement deficiencies are frequently observed in patients with Neisserial infections.
- The complement system's cytolytic activity plays a role in resistance to Neisseria meningitidis.
Purpose of the Study:
- To explore the relationship between terminal complement deficiencies and Neisserial infections.
- To investigate geographical variations in terminal complement deficiency prevalence among patients with meningococcal disease.
- To clarify the association of terminal complement deficiencies with autoimmune and non-Neisserial infections.
Main Methods:
- Review of available data on terminal complement deficiencies and Neisserial infections.
- Discussion of clinical indicators for suspecting terminal complement deficiency.
- Highlighting the need for advanced diagnostic techniques like ELISA and molecular assays for accurate characterization of defects.
Main Results:
- Geographical differences exist in the prevalence of terminal complement deficiency in meningococcal disease patients.
- In Western countries, recurrent Neisserial infection or infection with uncommon serogroups warrants clinical suspicion.
- In high-risk areas, recurrent Neisserial disease is a key indicator.
- Association of terminal complement deficiencies with autoimmune diseases or non-Neisserial infections is uncertain.
- Two types of deficiencies (subtotal and total absence) exist for complement components like C6, C7, and C8.
- Subtotal deficiencies show a weaker association with Neisserial infections.
Conclusions:
- The cytolytic function of the complement system is crucial for Neisseria meningitidis resistance.
- Clinical suspicion for terminal complement deficiency should be guided by geographical context and infection patterns.
- Accurate characterization of complement defects using sensitive assays is essential for a better understanding of their role in disease.
- Subtotal deficiencies may have a reduced impact on Neisserial infection susceptibility, suggesting a broad safety margin for complement activity.