title: Cytokine expression in bronchial biopsies of cystic fibrosis patients with and without acute exacerbation.
abstract: In patients with cystic fibrosis (CF), the progression of pulmonary disease differs considerably, even in identical cystic fibrosis transmembrane conductance regulator-genotypes which could reflect an additional influence of the host's immune response. This study therefore measured cytokine expression patterns in CF patients with different clinical presentation. Expression of interleukin (IL)-8, interferon gamma (IFN-gamma), IL-4, IL-10, and transforming growth factor (TGF)beta(I) was assessed in bronchial mucosal biopsies of eight CF patients with acute exacerbation (age 6.0-14.2 yrs), eight CF patients with chronic stable disease (age 7.3-17.4 yrs), and in five normal control subjects by semiquantitative and quantitative reverse transcriptase polymerase chain reaction combined with histopathological assessment and immunohistochemical staining. All CF patients expressed IL-8. In acute exacerbation, expression of TGF-beta1 and IFN-gamma was either absent or extremely low. In contrast, all patients with stable disease strongly expressed TGF-beta1. The highest expression of TGF-beta1 and IFN-gamma was found in CF patients with mild disease and a history of infrequent exacerbations. No correlation was found between the expression of IL-4 and IL-10 and patient history. In normal control subjects, only a weak expression of TGF-beta1 was observed. These results show a remarkable correlation between cytokine pattern and the clinical course of cystic fibrosis. High expression of transforming growth factor-beta1 and interferon gamma was associated with mild disease, whereas no or very weak expression of these cytokines was typical for patients with acute disease and frequent exacerbations suggesting a contribution of the immune response to the progression of pulmonary disease in cystic fibrosis.
authors.affiliation: Dept of Paediatrics, University Hospital Vienna, Austria.
authors.forename: C
authors.initials: C
authors.lastname: Wojnarowski
authors.forename: T
authors.initials: T
authors.lastname: Frischer
authors.forename: E
authors.initials: E
authors.lastname: Hofbauer
authors.forename: C
authors.initials: C
authors.lastname: Grabner
authors.forename: W
authors.initials: W
authors.lastname: Mosgoeller
authors.forename: I
authors.initials: I
authors.lastname: Eichler
authors.forename: R
authors.initials: R
authors.lastname: Ziesche
mesh_terms: D001706:Biopsy; D001980:Bronchi / Q000737:chemistry* / Q000473:pathology; D016022:Case-Control Studies; D002648:Child; D003550:Cystic Fibrosis / Q000378:metabolism*; D016207:Cytokines / Q000032:analysis*; D005260:Female; D006801:Humans; D018836:Inflammation Mediators / Q000032:analysis; D007371:Interferon-gamma / Q000032:analysis; D008297:Male; D020545:Respiratory Mucosa / Q000737:chemistry / Q000473:pathology; D020133:Reverse Transcriptase Polymerase Chain Reaction; D016212:Transforming Growth Factor beta / Q000032:analysis
chemical_list: D016207:Cytokines; D018836:Inflammation Mediators; D016212:Transforming Growth Factor beta; D007371:Interferon-gamma