Hypoxia and sterile inflammation in cystic fibrosis airways: Mechanisms and potential therapies
Copyright © ERS 2017. Cystic fibrosis is one of the most common autosomal recessive genetic diseases in Caucasian populations. Diagnosis via newborn screening and targeted nutritional and antibiotic therapy have improved outcomes, however respiratory failure remains the key cause of morbidity and m...
| Main Authors: | , , , |
|---|---|
| Format: | Journal Article |
| Language: | English |
| Published: |
EUROPEAN RESPIRATORY SOC JOURNALS LTD
2017
|
| Subjects: | |
| Online Access: | http://hdl.handle.net/20.500.11937/76811 |
| _version_ | 1848763766162849792 |
|---|---|
| author | Montgomery, S.T. Mall, M.A. Kicic, Anthony Stick, S.M. |
| author_facet | Montgomery, S.T. Mall, M.A. Kicic, Anthony Stick, S.M. |
| author_sort | Montgomery, S.T. |
| building | Curtin Institutional Repository |
| collection | Online Access |
| description | Copyright © ERS 2017. Cystic fibrosis is one of the most common autosomal recessive genetic diseases in Caucasian populations. Diagnosis via newborn screening and targeted nutritional and antibiotic therapy have improved outcomes, however respiratory failure remains the key cause of morbidity and mortality. Progressive respiratory disease in cystic fibrosis is characterised by chronic neutrophilic airway inflammation associated with structural airway damage leading to bronchiectasis and decreased lung function. Mucus obstruction is a characteristic early abnormality in the cystic fibrosis airway, associated with neutrophilic inflammation often in the absence of detectable infection. Recent studies have suggested a link between hypoxic cell death and sterile neutrophilic inflammation in cystic fibrosis and other diseases via the IL-1 signalling pathway. In this review, we consider recent evidence regarding the cellular responses to respiratory hypoxia as a potential driver of sterile neutrophilic inflammation in the lung, current knowledge on hypoxia as a pathogenic mechanism in cystic fibrosis and the potential for current and future therapies to alleviate hypoxia-driven sterile inflammation. |
| first_indexed | 2025-11-14T11:08:40Z |
| format | Journal Article |
| id | curtin-20.500.11937-76811 |
| institution | Curtin University Malaysia |
| institution_category | Local University |
| language | English |
| last_indexed | 2025-11-14T11:08:40Z |
| publishDate | 2017 |
| publisher | EUROPEAN RESPIRATORY SOC JOURNALS LTD |
| recordtype | eprints |
| repository_type | Digital Repository |
| spelling | curtin-20.500.11937-768112019-11-11T07:16:37Z Hypoxia and sterile inflammation in cystic fibrosis airways: Mechanisms and potential therapies Montgomery, S.T. Mall, M.A. Kicic, Anthony Stick, S.M. Science & Technology Life Sciences & Biomedicine Respiratory System NF-KAPPA-B TRANSMEMBRANE CONDUCTANCE REGULATOR INDUCIBLE FACTOR-I INTERLEUKIN-1 RECEPTOR ANTAGONIST ALVEOLAR EPITHELIAL-CELLS OXYGEN PARTIAL-PRESSURE LUNG-DISEASE PSEUDOMONAS-AERUGINOSA NLRP3 INFLAMMASOME HYPERTONIC SALINE Copyright © ERS 2017. Cystic fibrosis is one of the most common autosomal recessive genetic diseases in Caucasian populations. Diagnosis via newborn screening and targeted nutritional and antibiotic therapy have improved outcomes, however respiratory failure remains the key cause of morbidity and mortality. Progressive respiratory disease in cystic fibrosis is characterised by chronic neutrophilic airway inflammation associated with structural airway damage leading to bronchiectasis and decreased lung function. Mucus obstruction is a characteristic early abnormality in the cystic fibrosis airway, associated with neutrophilic inflammation often in the absence of detectable infection. Recent studies have suggested a link between hypoxic cell death and sterile neutrophilic inflammation in cystic fibrosis and other diseases via the IL-1 signalling pathway. In this review, we consider recent evidence regarding the cellular responses to respiratory hypoxia as a potential driver of sterile neutrophilic inflammation in the lung, current knowledge on hypoxia as a pathogenic mechanism in cystic fibrosis and the potential for current and future therapies to alleviate hypoxia-driven sterile inflammation. 2017 Journal Article http://hdl.handle.net/20.500.11937/76811 10.1183/13993003.00903-2016 English EUROPEAN RESPIRATORY SOC JOURNALS LTD restricted |
| spellingShingle | Science & Technology Life Sciences & Biomedicine Respiratory System NF-KAPPA-B TRANSMEMBRANE CONDUCTANCE REGULATOR INDUCIBLE FACTOR-I INTERLEUKIN-1 RECEPTOR ANTAGONIST ALVEOLAR EPITHELIAL-CELLS OXYGEN PARTIAL-PRESSURE LUNG-DISEASE PSEUDOMONAS-AERUGINOSA NLRP3 INFLAMMASOME HYPERTONIC SALINE Montgomery, S.T. Mall, M.A. Kicic, Anthony Stick, S.M. Hypoxia and sterile inflammation in cystic fibrosis airways: Mechanisms and potential therapies |
| title | Hypoxia and sterile inflammation in cystic fibrosis airways: Mechanisms and potential therapies |
| title_full | Hypoxia and sterile inflammation in cystic fibrosis airways: Mechanisms and potential therapies |
| title_fullStr | Hypoxia and sterile inflammation in cystic fibrosis airways: Mechanisms and potential therapies |
| title_full_unstemmed | Hypoxia and sterile inflammation in cystic fibrosis airways: Mechanisms and potential therapies |
| title_short | Hypoxia and sterile inflammation in cystic fibrosis airways: Mechanisms and potential therapies |
| title_sort | hypoxia and sterile inflammation in cystic fibrosis airways: mechanisms and potential therapies |
| topic | Science & Technology Life Sciences & Biomedicine Respiratory System NF-KAPPA-B TRANSMEMBRANE CONDUCTANCE REGULATOR INDUCIBLE FACTOR-I INTERLEUKIN-1 RECEPTOR ANTAGONIST ALVEOLAR EPITHELIAL-CELLS OXYGEN PARTIAL-PRESSURE LUNG-DISEASE PSEUDOMONAS-AERUGINOSA NLRP3 INFLAMMASOME HYPERTONIC SALINE |
| url | http://hdl.handle.net/20.500.11937/76811 |