Defective antigen presentation by lavage cells from terminal patients with cystic fibrosis

R A Knight1, S Kollnberger, B Madden

  • 1Department of Cystic Fibrosis, Royal Brompton Hospital, London, UK.

Insights

Immune cells in the lungs of cystic fibrosis (CF) patients show impaired function. This deficiency in lung macrophages may contribute to the frequent infections seen in CF patients.

Area of Science:

  • Immunology
  • Pulmonology
  • Cell Biology

Background:

  • Cystic fibrosis (CF) is characterized by recurrent lung infections.
  • The role of local immune cell function in CF pathogenesis is not fully understood.

Purpose of the Study:

  • To compare the immune function of lung macrophages and circulating monocytes in CF patients.
  • To investigate antigen presentation and lymphocyte stimulation capabilities of these cells.

Main Methods:

  • Macrophages were isolated from lung lavage samples of CF and non-CF patients undergoing transplantation.
  • Circulating monocytes from the same patients were also analyzed.
  • Functional assays included allogeneic lymphocyte stimulation and antigen presentation to autologous lymph node cells.

Main Results:

  • Lung macrophages from CF patients demonstrated significantly reduced ability to stimulate allogeneic lymphocytes and present antigens.
  • In contrast, circulating monocytes from CF patients remained functional in these assays.
  • Lavage cells from non-CF patients were generally effective in both immune functions.

Conclusions:

  • Immune cells, specifically macrophages, within the lungs of CF patients exhibit functional compromise.
  • This local immune deficiency may be a contributing factor to the susceptibility to recurrent infections in cystic fibrosis.