A role for cathepsin S in the pathogenesis of cystic fibrosis lung disease

Weldon, Sinéad; Elborn, J.S.; Scott, Christopher J.; Taggart, Clifford C.

doi:10.1016/s1569-1993(10)60173-7

Cited by 4 publications

(8 citation statements)

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“…Given that epithelial cells have been reported to express CTSS (21,24,25), we investigated the hypothesis that CF airway epithelial cells contribute to the protease burden of the CF lung by releasing increased levels of CTSS compared with non-CF control cells, and explore the mechanism responsible for our observations. Some of the results of these studies have been previously reported in the form of abstracts (26,27).…”

mentioning

confidence: 95%

miR-31 Dysregulation in Cystic Fibrosis Airways Contributes to Increased Pulmonary Cathepsin S Production

Weldon

McNally

McAuley

et al. 2014

Am J Respir Crit Care Med

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mentioning

confidence: 95%

miR-31 Dysregulation in Cystic Fibrosis Airways Contributes to Increased Pulmonary Cathepsin S Production

Weldon

McNally

McAuley

et al. 2014

Am J Respir Crit Care Med

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“…8 ACTs, used in isolation or in conjunction with other therapies such as inhaled osmotic agents and mucolytics, attempt to reduce the degree of mucus obstruction within the airways by promoting increased MCC, ultimately aiming to reduce the rate of progressive airway damage seen in pwCF. 1 56 57 58…”

Section: Airway Clearance Strategies In Cystic Fibrosismentioning

confidence: 99%

“…This can lead to bronchiectasis and eventually respiratory failure, resulting in death or the need for lung transplantation. 1 In addition to respiratory disease, mutations in the CF gene have a direct and/or indirect effect on digestion, bone mineral density, and skeletal muscle function. 1 2 Exercise and airway clearance techniques (ACTs) have been recommended as important components of the management of the respiratory and nonrespiratory sequalae of CF for many decades.…”

mentioning

confidence: 99%

“…1 In addition to respiratory disease, mutations in the CF gene have a direct and/or indirect effect on digestion, bone mineral density, and skeletal muscle function. 1 2 Exercise and airway clearance techniques (ACTs) have been recommended as important components of the management of the respiratory and nonrespiratory sequalae of CF for many decades. 3 4 However, the evolution of clinical practice over the years, particularly the increased number of pharmacological and nonpharmacological therapies available to people with CF (pwCF), has resulted in an increased life expectancy and more pwCF having normal or only mildly impaired respiratory function.…”

mentioning

confidence: 99%

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Exercise and Airway Clearance Techniques in Cystic Fibrosis

Ward

Stiller

Holland

2022

Semin Respir Crit Care Med

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Exercise and airway clearance techniques (ACTs) have been a cornerstone of treatment for people with cystic fibrosis (pwCF) for many decades. Exercise may confer both respiratory and nonrespiratory benefits for pwCF, with greater exercise capacity associated with improved survival. A wide variety of exercise interventions for pwCF have been investigated. ACTs may assist in reducing respiratory symptoms for pwCF and are currently recommended to be performed daily, with the types of ACTs used varying globally. While recommended components of care, both exercise and ACTs are time-intensive and maintaining adherence to the recommendations over the longer term can be challenging. It has been proposed that with advances in the therapeutic options for pwCF, a rationalization of the therapeutic regimen may be possible. We summarize the current evidence for the use of exercise and ACTs by pwCF, discuss the implications of the introduction of cystic fibrosis transmembrane conductance regulator modulators on both exercise and ACTs, and highlight areas for further research.

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“…This is associated with an altered airway microbiome that fosters chronic infection causing morbidity, progressive deterioration in lung function, and premature death. 98 The crucial importance of acquisition of key pathogenic bacteria in CF has long been acknowledged, but the CF lung is also colonized by complex polymicrobial communities including fungi and viruses. 99 It is likely that the interactions between these communities have a large part in exacerbations in CF.…”

Section: Respiratory Viruses and The Cystic Fibrosis Lung Microbiomementioning

confidence: 99%

Respiratory Viruses and Cystic Fibrosis

Brackenborough

Ellis

Flight

2022

Semin Respir Crit Care Med

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The threat of respiratory virus infection to human health and well-being has been clearly highlighted by the coronavirus disease 2019 (COVID-19) pandemic. For people with cystic fibrosis (CF), the clinical significance of viral infections long predated the emergence of severe acute respiratory syndrome coronavirus 2. This article reviews the epidemiology, diagnosis, and treatment of respiratory virus infection in the context of CF as well as the current understanding of interactions between viruses and other microorganisms in the CF lung. The incidence of respiratory virus infection in CF varies by age with young children typically experiencing more frequent episodes than adolescents and adults. At all ages, respiratory viruses are very common in CF and are associated with pulmonary exacerbations. Respiratory viruses are identified at up to 69% of exacerbations, while viruses are also frequently detected during clinical stability. The full impact of COVID-19 in CF is yet to be established. Early studies found that rates of COVID-19 were lower in CF cohorts than in the general population. The reasons for this are unclear but may be related to the effects of shielding, infection control practices, maintenance CF therapies, or the inflammatory milieu in the CF lung. Observational studies have consistently identified that prior solid organ transplantation is a key risk factor for poor outcomes from COVID-19 in CF. Several key priorities for future research are highlighted. First, the impact of highly effective CFTR modulator therapy on the epidemiology and pathophysiology of viral infections in CF requires investigation. Second, the impact of respiratory viruses on the development and dynamics of the CF lung microbiota is poorly understood and viral infection may have important interactions with bacteria and fungi in the airway. Finally, bacteriophages represent a key focus of future investigation both for their role in transmission of antimicrobial resistance and as a promising treatment modality for multiresistant pathogens.

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A role for cathepsin S in the pathogenesis of cystic fibrosis lung disease

Cited by 4 publications

References 0 publications

miR-31 Dysregulation in Cystic Fibrosis Airways Contributes to Increased Pulmonary Cathepsin S Production

miR-31 Dysregulation in Cystic Fibrosis Airways Contributes to Increased Pulmonary Cathepsin S Production

Exercise and Airway Clearance Techniques in Cystic Fibrosis

Respiratory Viruses and Cystic Fibrosis

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