title: Lung disease, T-cells and inflammation in common variable immunodeficiency disorders.
abstract: BACKGROUND
  Besides hypogammaglobulinemia and recurrent infections, abnormalities of T-cells might contribute to lung damage in common variable immunodeficiency disorders (CVID).
  
  
  METHODS
  In 16 adult patients, the majority of whom had pulmonary abnormalities, we studied T-cell subsets and markers of inflammation in bronchoalveolar lavage fluid (BALF) and blood and their relations with pulmonary function and high resolution computed tomography (HRCT).
  
  
  RESULTS
  We demonstrated that some of the lymphocyte abnormalities previously demonstrated in peripheral blood from CVID patients, such as low CD4/CD8 T-cell ratio, were also present in BALF. Moreover, low BALF CD4/CD8 ratio (≤ 1), found in seven patients, was significantly associated with higher blood CD8⁺ cell count and to lower values of the lung function variables; forced expiratory volume (FVC), total lung capacity (TLC), vital capacity (VC) and residual volume (RV) in % of predicted. The expression of the inflammatory markers HLA-DR and CCR5 on T-cells was significantly higher, and the expression of CCR7 significantly lower, in BALF compared to blood, possibly reflecting an inflammatory/cytotoxic T-cell phenotype within pulmonary tissue in CVID. Furthermore, patients with bronchiectasis had higher concentrations of the pro-inflammatory cytokine TNFα in plasma, compared to those without.
  
  
  CONCLUSIONS
  Our findings suggest that inflammation and T-cell activation may be involved in the immunopathogenesis of pulmonary complications in CVID.
authors: Department of Respiratory Medicine.
authors: Stina
authors: S
authors: Gregersen
authors: Are M
authors: AM
authors: Holm
authors: Børre
authors: B
authors: Fevang
authors: Thor
authors: T
authors: Ueland
authors: Liv I B
authors: LI
authors: Sikkeland
authors: Trond M
authors: TM
authors: Aaløkken
authors: Georg
authors: G
authors: Mynarek
authors: Johny
authors: J
authors: Kongerud
authors: Pål
authors: P
authors: Aukrust
authors: Bjørn
authors: B
authors: Johansen
authors: Stig S
authors: SS
authors: Frøland
mesh_terms: D000328:Adult; D001992:Bronchoalveolar Lavage Fluid; D016516:CD4-CD8 Ratio; D016022:Case-Control Studies; D054415:Chemokine CCL19 / Q000378:metabolism; D017074:Common Variable Immunodeficiency / Q000150:complications / Q000276:immunology*; D005260:Female; D006801:Humans; D018836:Inflammation Mediators / Q000378:metabolism; D008168:Lung / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology; D017563:Lung Diseases, Interstitial / Q000000981:diagnostic imaging / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D011859:Radiography; D013601:T-Lymphocytes / Q000276:immunology*; D055815:Young Adult
chemical_list: C515460:CCL19 protein, human; D054415:Chemokine CCL19; D018836:Inflammation Mediators