title: Complement deficiency states and associated infections.
abstract: A major function of the immune system is to protect the host from microbial infections. The complement system plays important roles in both the innate and the adaptive immune defense and also acts as a bridge between these arms of immunity. This is obvious from complement deficiencies which in varying degree, depending on which factor is missing, are associated with increased infection susceptibility and also increased risk for other, mainly autoimmune diseases. Genetically determined deficiencies are described for almost all complement proteins but the consequences show a wide variation. Here the genetic defects and molecular abnormalities in complement deficient persons, related clinically relevant infections and the options for prevention and therapy are reviewed. The roles of complement in host defense against common infections are also discussed.
authors.affiliation: Department of Laboratory Medicine, Section of Microbiology, Immunology and Glycobiology, Lund University, Lund, Sweden.
authors.forename: Lillemor
authors.initials: L
authors.lastname: Skattum
authors.forename: Marcel
authors.initials: M
authors.lastname: van Deuren
authors.forename: Tom
authors.initials: T
authors.lastname: van der Poll
authors.forename: Lennart
authors.initials: L
authors.lastname: Truedsson
mesh_terms: D056704:Adaptive Immunity; D000818:Animals; D050718:Complement C1 Inhibitor Protein / Q000008:administration & dosage; D015938:Complement Membrane Attack Complex / Q000172:deficiency; D003170:Complement Pathway, Alternative; D003171:Complement Pathway, Classical; D003165:Complement System Proteins / Q000008:administration & dosage / Q000172:deficiency* / Q000235:genetics; D006801:Humans; D007113:Immunity, Innate; D007239:Infections / Q000209:etiology* / Q000276:immunology* / Q000628:therapy; D037601:Mannose-Binding Lectin / Q000008:administration & dosage / Q000172:deficiency; D008586:Meningitis, Pneumococcal / Q000276:immunology; D018448:Models, Immunological; D016870:Neisseriaceae Infections / Q000276:immunology; D010951:Plasma Exchange; D011018:Pneumonia, Pneumococcal / Q000276:immunology; D011951:Receptors, Complement / Q000172:deficiency; D018805:Sepsis / Q000276:immunology; D014611:Vaccination
chemical_list: D050718:Complement C1 Inhibitor Protein; D015938:Complement Membrane Attack Complex; C445619:MBL2 protein, human; D037601:Mannose-Binding Lectin; D011951:Receptors, Complement; D003165:Complement System Proteins