Complement deficiencies in selected groups of patients with meningococcal disease
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H E Nielsen1, C Koch, P Magnussen
1Complement Laboratory, Statens Seruminstitut, Copenhagen, Denmark.
Abstract:
We have examined 125 individuals who have earlier had meningococcal (mgc) disease. They belonged to one or more of the following groups: (1) 2 or more cases of mgc disease in the same family; (2) individuals with 2 episodes of mgc disease or with 1 episode of mgc disease and 1 or more episodes of purulent meningitis of another aetiology; and (3) infections with Neisseria meningitidis belonging to serogroups that are uncommon as causes of disease and presumably low-virulent (W-135, 29E, X, Y). Among these we found 15 complement (C)-deficient individuals (12%). The prevalence of C deficiency in the groups above was 7%, 41% and 19%, respectively. The first group (family cases), is very heterogeneous and may be further subdivided into 2 groups: families whose members fell ill within an interval of 30 days (in these the prevalence of C deficiency was 2%), and families in which the interval between mgc disease cases exceeded 30 days (in those the prevalence of C deficiency was 14%). We found a predominance of defects of the initiation pathways, with properdin deficiency being the most common.
Insights
Complement deficiency is linked to meningococcal disease. This study found 12% of individuals with recurrent or familial meningococcal disease had complement deficiencies, particularly properdin deficiency.
Area of Science:
- Immunology
- Infectious Diseases
- Genetics
Background:
- Recurrent meningococcal disease (mgc) and infections with uncommon serogroups suggest potential underlying immune defects.
- Complement system deficiencies are known risk factors for invasive meningococcal disease.
Purpose of the Study:
- To investigate the prevalence of complement (C) deficiencies in individuals with a history of meningococcal disease.
- To identify specific complement pathways and factors associated with increased susceptibility.
Main Methods:
- Analysis of 125 individuals with a history of meningococcal disease.
- Categorization of individuals into groups based on family history, recurrent disease, or uncommon serogroup infections.
- Screening for complement deficiencies.
Main Results:
- Fifteen (12%) individuals had complement deficiencies.
- Prevalence of C deficiency varied by group: 7% (familial cases), 41% (recurrent/multiple meningitis), and 19% (uncommon serogroups).
- Properdin deficiency, a defect in the complement initiation pathway, was the most common finding.
Conclusions:
- Complement deficiencies, particularly in the initiation pathway like properdin deficiency, are associated with meningococcal disease.
- Individuals with recurrent or familial meningococcal disease represent a high-risk group for identifying complement deficiencies.