title: End-stage cystic fibrosis lung disease is characterised by a diverse inflammatory pattern: an immunohistochemical analysis.
abstract: Cystic fibrosis (CF) lung disease is characterised by vigorous airway inflammation eventually resulting in severe lung damage. This study aimed to describe the diversity of the inflammatory pattern in end-stage CF lungs by evaluating and quantifying which components of the innate and adaptive immunity are involved, and by assessing whether this is gender-specific.
  
  
  
  CF explant lung tissue (n = 20) collected at time of transplantation and control tissue (n = 22) was sectioned (9 μm) and stained for neutrophils, eosinophils, mast cells, dendritic cells, macrophages, CD4 T cells, cytotoxic T cells and B cells. Quantification with special attention for immune cell location was performed.
  
  
  
  Neutrophils, mast cells, dendritic cells, macrophages, CD4 T and cytotoxic T cells were significantly increased in CF compared to controls and there was a disproportionate increase of neutrophils around the airways in CF. Large amounts of lymphoid follicles were found in the CF lung and they had a skewed B cell/T cell composition. Upon subdividing the CF patients into a male and female population, eosinophils, mast cells and CD4 T cells were increased specifically in CF females. In this subpopulation, lymphoid follicles had less B cells and more CD8 T cells.
  
  
  
  These data demonstrate a diverse inflammatory response in the CF lung, reflected by an increase of both myeloid and lymphoid immune cells. Inflammation in the CF lung appeared to be gender-specific in our population, as the significant increase of eosinophils, mast cells and CD4 T cells was especially notable in the female subpopulation.
authors.affiliation: Laboratory of Respiratory Diseases, Department of Clinical and Experimental Medicine, University of Leuven, UZ Herestraat 49, Box 706, 3000, Leuven, Belgium. Elise.lammertyn@kuleuven.be.
authors.forename: Elise J
authors.initials: EJ
authors.lastname: Lammertyn
authors.affiliation: Laboratory of Respiratory Diseases, Department of Clinical and Experimental Medicine, University of Leuven, UZ Herestraat 49, Box 706, 3000, Leuven, Belgium.
authors.forename: Elly
authors.initials: E
authors.lastname: Vandermeulen
authors.affiliation: Laboratory of Respiratory Diseases, Department of Clinical and Experimental Medicine, University of Leuven, UZ Herestraat 49, Box 706, 3000, Leuven, Belgium.
authors.forename: Hannelore
authors.initials: H
authors.lastname: Bellon
authors.affiliation: Laboratory of Respiratory Diseases, Department of Clinical and Experimental Medicine, University of Leuven, UZ Herestraat 49, Box 706, 3000, Leuven, Belgium.
authors.forename: Stephanie
authors.initials: S
authors.lastname: Everaerts
authors.affiliation: Laboratory of Respiratory Diseases, Department of Clinical and Experimental Medicine, University of Leuven, UZ Herestraat 49, Box 706, 3000, Leuven, Belgium.
authors.forename: Stijn E
authors.initials: SE
authors.lastname: Verleden
authors.affiliation: Translational Cell and Tissue Research Unit, Department of Imaging and Pathology, University of Leuven, Leuven, Belgium.
authors.forename: Kathleen
authors.initials: K
authors.lastname: Van Den Eynde
authors.affiliation: Department of Respiratory Medicine, Ghent University Hospital, Ghent, Belgium.
authors.forename: Ken R
authors.initials: KR
authors.lastname: Bracke
authors.affiliation: Department of Respiratory Medicine, Ghent University Hospital, Ghent, Belgium.
authors.forename: Guy G
authors.initials: GG
authors.lastname: Brusselle
authors.affiliation: Laboratory of Respiratory Diseases, Department of Clinical and Experimental Medicine, University of Leuven, UZ Herestraat 49, Box 706, 3000, Leuven, Belgium.
authors.forename: Pieter C
authors.initials: PC
authors.lastname: Goeminne
authors.affiliation: Translational Cell and Tissue Research Unit, Department of Imaging and Pathology, University of Leuven, Leuven, Belgium.
authors.forename: Erik K
authors.initials: EK
authors.lastname: Verbeken
authors.affiliation: Laboratory of Respiratory Diseases, Department of Clinical and Experimental Medicine, University of Leuven, UZ Herestraat 49, Box 706, 3000, Leuven, Belgium.
authors.forename: Bart M
authors.initials: BM
authors.lastname: Vanaudenaerde
authors.affiliation: Cystic Fibrosis Unit, Department of Respiratory Diseases, University Hospitals Leuven, Leuven, Belgium.
authors.forename: Lieven J
authors.initials: LJ
authors.lastname: Dupont
mesh_terms: D003550:Cystic Fibrosis / Q000276:immunology* / Q000473:pathology; D005260:Female; D006801:Humans; D018836:Inflammation Mediators / Q000276:immunology*; D008168:Lung / Q000276:immunology* / Q000473:pathology; D008264:Macrophages / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D011014:Pneumonia / Q000276:immunology* / Q000473:pathology; D012727:Sex Characteristics; D013601:T-Lymphocytes / Q000276:immunology*
chemical_list: D018836:Inflammation Mediators
keywords: Cellular immune system; Histopathology; Lung immunopathology