Complement deficiency
From IDWiki
Background
- Group of primary or acquired immunodeficiencies affecting the complement system within the innate humoural immune system
- Usually autosomal recessive genetic diseases
- Commonly defined as early (C1-C4) or late (C5-C9)
Clinical Manifestations
| Deficiency | |
|---|---|
| Early (C1, C2, and C4) | Encapsulated bacterial infections like Streptococcus pneumoniae and Haemophilus influenzae type b. Higher risk of autoimmune diseases, particularly systemic lupus erythematosus) |
| Early (C3) | Severe recurrent pyogenic infections early in life |
| Late (C5-C9) | Recurrent Neisseria infections |
| Alternative (properdin) | Recurrent Neisseria infections |
| Mannan-binding lectin | Increase frequency of pyogenic infections, particularly in young children |
