title: Common variable immunodeficiency and the complement system; low mannose-binding lectin levels are associated with bronchiectasis.
abstract: The importance of the innate immune system, including mannose-binding lectin and the complement system, in common variable immunodeficiency is unclear. The objective of this study was to evaluate mannose-binding lectin and the complement system in relation to clinical and immunological parameters in patients with common variable immunodeficiency. Circulating levels of mannose-binding lectin, complement components, complement activation products and functional capacity of complement pathways were correlated to clinical features within 71 patients and compared with 30 healthy controls. The main findings were; the patients had signs of increased complement activation significantly associated with signs of autoimmunity and immunological hyperactivity; there were no signs of deficiencies of the classical and alternative complement pathways in the patient group; the prevalence of lectin pathway deficiency was the same in patients and controls, but patients with increased frequency of lower respiratory tract infections or bronchiectasis had lower capacity of the lectin pathway than patients without these features (P = 0.002 and 0.004, respectively); the serum concentration of mannose-binding lectin was inversely correlated to the frequency of lower respiratory tract infections (P = 0.002) and bronchiectasis (P = 0.01). We conclude that patients with common variable immunodeficiency have no increased frequency of complement deficiencies but signs of increased complement activation. Our findings suggest that mannose-binding lectin and the lectin complement pathway may protect against lower respiratory tract infection and bronhiectasis in patients with common variable immunodeficiency.
authors.affiliation: Research Institute for Internal Medicine, University of Oslo, Rikshospitalet University Hospital, N-0027 Oslo, Norway. Borre.Fevang@medisin.uio.no
authors.forename: B
authors.initials: B
authors.lastname: Fevang
authors.forename: T E
authors.initials: TE
authors.lastname: Mollnes
authors.forename: A M
authors.initials: AM
authors.lastname: Holm
authors.forename: T
authors.initials: T
authors.lastname: Ueland
authors.forename: L
authors.initials: L
authors.lastname: Heggelund
authors.forename: J K
authors.initials: JK
authors.lastname: Damås
authors.forename: P
authors.initials: P
authors.lastname: Aukrust
authors.forename: S S
authors.initials: SS
authors.lastname: Frøland
mesh_terms: D000328:Adult; D001987:Bronchiectasis / Q000276:immunology* / Q000378:metabolism; D002097:C-Reactive Protein / Q000032:analysis / Q000276:immunology; D017074:Common Variable Immunodeficiency / Q000276:immunology*; D003167:Complement Activation / Q000276:immunology; D015926:Complement C3a / Q000032:analysis / Q000276:immunology; D015934:Complement C4a / Q000032:analysis / Q000276:immunology; D015936:Complement C5a / Q000032:analysis / Q000276:immunology; D003170:Complement Pathway, Alternative / Q000276:immunology; D003171:Complement Pathway, Classical / Q000276:immunology; D003165:Complement System Proteins / Q000032:analysis / Q000276:immunology*; D005260:Female; D006801:Humans; D037102:Lectins / Q000097:blood / Q000276:immunology* / Q000378:metabolism; D008297:Male; D008358:Mannose / Q000378:metabolism*; D008875:Middle Aged; D012141:Respiratory Tract Infections / Q000276:immunology / Q000378:metabolism
chemical_list: D037102:Lectins; D015926:Complement C3a; D015934:Complement C4a; D015936:Complement C5a; D003165:Complement System Proteins; D002097:C-Reactive Protein; D008358:Mannose