Inherited complement deficiencies and bacterial infections

Abstract

A wide variety of bacteria are recognized by the complement system through the early components that trigger the three pathways of complement activation, leading to the release of biologically active products involved in opsonization, recruitment of phagocytes and bacterial killing. Deficiencies of complement components and regulators provide a model to understand the in vivo role of complement as a defense system against bacterial infections. An increased susceptibility to these types of infections is frequently seen in individuals with C2, C3, late component, properdin and factor I deficiencies. The identification of these deficiencies is essential for the adoption of preventive measures aimed to reduce the risk of bacterial infections. Vaccination represents the treatment of choice to protect these subjects, although further studies on a large number of C-deficient individuals are needed to prove the protective effect of vaccines.