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Complement deficiencies in patients over ten years old with meningococcal disease due to uncommon serogroups

C A Fijen1, E J Kuijper, A J Hannema

  • 1Department of Medical Microbiology, University of Amsterdam Academic Medical Centre, the Netherlands.

Lancet (London, England)
|September 9, 1989
PubMed

Insights

Half of patients with rare meningococcal disease serogroups had complement deficiencies. Terminal complement component deficiencies were linked to recurrent infections, highlighting the link between complement and meningococcal disease.

Area of Science:

  • Immunology
  • Infectious Diseases
  • Genetics

Background:

  • Meningococcal disease is typically caused by specific serogroups.
  • Infections with less common serogroups (X, Y, Z, W135, 29E) after age 10 are unusual.
  • Complement deficiencies are known risk factors for meningococcal disease.

Purpose of the Study:

  • To investigate the association between complement deficiencies and meningococcal disease caused by uncommon serogroups.
  • To identify specific complement deficiencies prevalent in patients with these infections.

Main Methods:

  • Retrospective investigation of 46 patients diagnosed with meningococcal disease due to serogroups X, Y, Z, W135, or 29E after age 10.
  • Analysis of complement component levels and function in affected patients.

Main Results:

  • Complement deficiency was identified in 50% of the patients studied.
  • Properdin deficiency was found in 9 patients.
  • C3 deficiency syndromes in 5 patients, and homozygous deficiencies of terminal components (C5-C8) in 9 patients.
  • Recurrent meningococcal infections occurred in 5 of the 9 patients with terminal complement component deficiencies.

Conclusions:

  • Meningococcal disease caused by uncommon serogroups is frequently associated with underlying complement deficiency.
  • Terminal complement component deficiencies represent a significant risk for recurrent meningococcal infections.
  • Screening for complement deficiencies should be considered in patients with invasive disease due to unusual meningococcal serogroups.

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