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Complement factor I deficiency associated with recurrent meningitis coinciding with menstruation.
C González-Rubio1, A Ferreira-Cerdán, I M Ponce
1Immunology Unit, Hospital Universitario, La Paz, Paseo de la Castellana, 261, 28046 Madrid, Spain.
Archives of Neurology
|November 16, 2001
Summary
Complement factor I deficiency, a rare immunodeficiency, can lead to recurrent meningitis, particularly in women during menstruation. This case highlights an unusual link between menstruation and meningitis in factor I deficiency.
Area of Science:
- Immunology
- Genetics
- Infectious Diseases
Background:
- Complement factor I deficiency is a rare immunodeficiency.
- It is associated with recurrent pyogenic infections and uncontrolled alternative pathway activation, leading to C3 consumption.
Observation:
- A 23-year-old woman experienced monthly meningitis episodes, often perimenstrual.
- She had a history of meningococcal sepsis at age 16, also coinciding with menstruation.
Findings:
- Total complement factor I deficiency was confirmed in the patient and her sister.
- The patient exhibited low levels of C3, factor B, factor H, properdin, C5, C7, and C8.
- High levels of immune complexes and various complement component complexes were detected in her serum.
Implications:
- This study presents a novel association between meningitis and menstruation in complement factor I deficiency.
- Understanding this link may improve diagnosis and management of similar rare immunodeficiencies.