[Recurrent meningitis and inherited complement deficiency].

Area of Science:

• Immunology
• Genetics

Background:

• Complement deficiency accounts for 5% of primary immunodeficiencies globally.
• Classical complement pathway deficiencies are rare, with only seven cases reported in the Czech Republic by 2015.
• Typical symptoms include recurrent meningitis, bacterial infections, autoimmunity, and kidney disease.

Purpose of the Study:

• To report two cases of molecularly confirmed complement deficiencies (C7 and C8).
• To highlight the clinical manifestations and management of these rare conditions.

Main Methods:

• Molecular confirmation of C7 deficiency (compound heterozygote) and C8 deficiency (homozygous).
• Review of patient histories detailing infection episodes and management strategies.
• Assessment of vaccination and antibiotic prophylaxis efficacy.

Main Results:

• Patient 1 (C7 deficiency) experienced four episodes of meningococcal meningitis and pneumonia.
• Patient 2 (C8 deficiency) had six meningitis attacks and recurrent ENT infections, which decreased with antibiotic prophylaxis.
• Neither patient developed autoimmune disease.

Conclusions:

• Complement deficiency should be suspected in patients with recurrent meningococcal infections, especially with concurrent encapsulated bacterial infections or autoimmunity.
• Conjugate polysaccharide vaccines are recommended, and antibiotic prophylaxis may be beneficial in select cases.