Alveolar inflammation in cystic fibrosis

Research output: Contribution to journal › Journal article › Research › peer-review

Standard

Alveolar inflammation in cystic fibrosis. / Ulrich, Martina; Worlitzsch, Dieter; Viglio, Simona; Siegmann, Nanna; Iadarola, Paolo; Shute, Janis K; Geiser, Marianne; Pier, Gerald B; Friedel, Godehard; Barr, Mark L; Schuster, Antje; Meyer, Keith C; Ratjen, Felix; Bjarnsholt, Thomas; Gulbins, Erich; Döring, Gerd.

In: Journal of Cystic Fibrosis, Vol. 9, No. 3, 2010, p. 217-27.

Research output: Contribution to journal › Journal article › Research › peer-review

Harvard

Ulrich, M, Worlitzsch, D, Viglio, S, Siegmann, N, Iadarola, P, Shute, JK, Geiser, M, Pier, GB, Friedel, G, Barr, ML, Schuster, A, Meyer, KC, Ratjen, F, Bjarnsholt, T, Gulbins, E & Döring, G 2010, 'Alveolar inflammation in cystic fibrosis', Journal of Cystic Fibrosis, vol. 9, no. 3, pp. 217-27. https://doi.org/10.1016/j.jcf.2010.03.001

APA

Ulrich, M., Worlitzsch, D., Viglio, S., Siegmann, N., Iadarola, P., Shute, J. K., Geiser, M., Pier, G. B., Friedel, G., Barr, M. L., Schuster, A., Meyer, K. C., Ratjen, F., Bjarnsholt, T., Gulbins, E., & Döring, G. (2010). Alveolar inflammation in cystic fibrosis. Journal of Cystic Fibrosis, 9(3), 217-27. https://doi.org/10.1016/j.jcf.2010.03.001

Vancouver

Ulrich M, Worlitzsch D, Viglio S, Siegmann N, Iadarola P, Shute JK et al. Alveolar inflammation in cystic fibrosis. Journal of Cystic Fibrosis. 2010;9(3):217-27. https://doi.org/10.1016/j.jcf.2010.03.001

Author

Ulrich, Martina ; Worlitzsch, Dieter ; Viglio, Simona ; Siegmann, Nanna ; Iadarola, Paolo ; Shute, Janis K ; Geiser, Marianne ; Pier, Gerald B ; Friedel, Godehard ; Barr, Mark L ; Schuster, Antje ; Meyer, Keith C ; Ratjen, Felix ; Bjarnsholt, Thomas ; Gulbins, Erich ; Döring, Gerd. / Alveolar inflammation in cystic fibrosis. In: Journal of Cystic Fibrosis. 2010 ; Vol. 9, No. 3. pp. 217-27.

Bibtex

@article{29707600a2e911df928f000ea68e967b,
title = "Alveolar inflammation in cystic fibrosis",
abstract = "BACKGROUND: In infected lungs of the cystic fibrosis (CF) patients, opportunistic pathogens and mutated cystic fibrosis transmembrane conductance regulator protein (CFTR) contribute to chronic airway inflammation that is characterized by neutrophil/macrophage infiltration, cytokine release and ceramide accumulation. We sought to investigate CF lung inflammation in the alveoli. METHODS: Lung tissue from 14 CF patients and four healthy individuals was analyzed for numbers of effector cells, elastin and collagen concentrations, inflammatory markers and density of Pseudomonas aeruginosa. Additionally, desmosine and isodesmosine concentrations were determined in 52 urine specimens from CF patients to estimate the burden of elastase activities in respiratory secretions. RESULTS: Elastin concentration was significantly decreased and collagen significantly increased in CF alveolar tissues as compared to age-matched, healthy individuals. Elastin split products were significantly increased in urine samples from patients with CF and correlated inversely with age, indicating local tissue remodelling due to elastin degradation by unopposed proteolytic enzymes. Alveolar inflammation was also characterized by a significant cell infiltration of neutrophils, macrophages and T cells, extensive nuclear factor-kappaB and insulin-like growth factor-1 activation in various cell types and increased intercellular adhesion molecule-1 expression, and increased numbers of myofibroblasts. Additionally, ceramide accumulated in type II alveolar epithelial cells, lacking CFTR. P. aeruginosa organisms were rarely present in inflamed alveoli. CONCLUSIONS: Chronic inflammation and remodeling is present in alveolar tissues of the CF lung and needs to be addressed by anti-inflammatory therapies.",
author = "Martina Ulrich and Dieter Worlitzsch and Simona Viglio and Nanna Siegmann and Paolo Iadarola and Shute, {Janis K} and Marianne Geiser and Pier, {Gerald B} and Godehard Friedel and Barr, {Mark L} and Antje Schuster and Meyer, {Keith C} and Felix Ratjen and Thomas Bjarnsholt and Erich Gulbins and Gerd D{\"o}ring",
year = "2010",
doi = "10.1016/j.jcf.2010.03.001",
language = "English",
volume = "9",
pages = "217--27",
journal = "Journal of Cystic Fibrosis",
issn = "1569-1993",
publisher = "Elsevier",
number = "3",

}

RIS

TY - JOUR

T1 - Alveolar inflammation in cystic fibrosis

AU - Ulrich, Martina

AU - Worlitzsch, Dieter

AU - Viglio, Simona

AU - Siegmann, Nanna

AU - Iadarola, Paolo

AU - Shute, Janis K

AU - Geiser, Marianne

AU - Pier, Gerald B

AU - Friedel, Godehard

AU - Barr, Mark L

AU - Schuster, Antje

AU - Meyer, Keith C

AU - Ratjen, Felix

AU - Bjarnsholt, Thomas

AU - Gulbins, Erich

AU - Döring, Gerd

PY - 2010

Y1 - 2010

N2 - BACKGROUND: In infected lungs of the cystic fibrosis (CF) patients, opportunistic pathogens and mutated cystic fibrosis transmembrane conductance regulator protein (CFTR) contribute to chronic airway inflammation that is characterized by neutrophil/macrophage infiltration, cytokine release and ceramide accumulation. We sought to investigate CF lung inflammation in the alveoli. METHODS: Lung tissue from 14 CF patients and four healthy individuals was analyzed for numbers of effector cells, elastin and collagen concentrations, inflammatory markers and density of Pseudomonas aeruginosa. Additionally, desmosine and isodesmosine concentrations were determined in 52 urine specimens from CF patients to estimate the burden of elastase activities in respiratory secretions. RESULTS: Elastin concentration was significantly decreased and collagen significantly increased in CF alveolar tissues as compared to age-matched, healthy individuals. Elastin split products were significantly increased in urine samples from patients with CF and correlated inversely with age, indicating local tissue remodelling due to elastin degradation by unopposed proteolytic enzymes. Alveolar inflammation was also characterized by a significant cell infiltration of neutrophils, macrophages and T cells, extensive nuclear factor-kappaB and insulin-like growth factor-1 activation in various cell types and increased intercellular adhesion molecule-1 expression, and increased numbers of myofibroblasts. Additionally, ceramide accumulated in type II alveolar epithelial cells, lacking CFTR. P. aeruginosa organisms were rarely present in inflamed alveoli. CONCLUSIONS: Chronic inflammation and remodeling is present in alveolar tissues of the CF lung and needs to be addressed by anti-inflammatory therapies.

AB - BACKGROUND: In infected lungs of the cystic fibrosis (CF) patients, opportunistic pathogens and mutated cystic fibrosis transmembrane conductance regulator protein (CFTR) contribute to chronic airway inflammation that is characterized by neutrophil/macrophage infiltration, cytokine release and ceramide accumulation. We sought to investigate CF lung inflammation in the alveoli. METHODS: Lung tissue from 14 CF patients and four healthy individuals was analyzed for numbers of effector cells, elastin and collagen concentrations, inflammatory markers and density of Pseudomonas aeruginosa. Additionally, desmosine and isodesmosine concentrations were determined in 52 urine specimens from CF patients to estimate the burden of elastase activities in respiratory secretions. RESULTS: Elastin concentration was significantly decreased and collagen significantly increased in CF alveolar tissues as compared to age-matched, healthy individuals. Elastin split products were significantly increased in urine samples from patients with CF and correlated inversely with age, indicating local tissue remodelling due to elastin degradation by unopposed proteolytic enzymes. Alveolar inflammation was also characterized by a significant cell infiltration of neutrophils, macrophages and T cells, extensive nuclear factor-kappaB and insulin-like growth factor-1 activation in various cell types and increased intercellular adhesion molecule-1 expression, and increased numbers of myofibroblasts. Additionally, ceramide accumulated in type II alveolar epithelial cells, lacking CFTR. P. aeruginosa organisms were rarely present in inflamed alveoli. CONCLUSIONS: Chronic inflammation and remodeling is present in alveolar tissues of the CF lung and needs to be addressed by anti-inflammatory therapies.

U2 - 10.1016/j.jcf.2010.03.001

DO - 10.1016/j.jcf.2010.03.001

M3 - Journal article

C2 - 20347403

VL - 9

SP - 217

EP - 227

JO - Journal of Cystic Fibrosis

JF - Journal of Cystic Fibrosis

SN - 1569-1993

IS - 3

ER -

ID: 21257741