Pain and its clinical associations in individuals with cystic fibrosis

Lee, Annemarie L.; Rawlings, Sarah; Bennett, Katharine A; Armstrong, David

doi:10.1177/1479972316631135

Cited by 28 publications

(24 citation statements)

References 43 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Chronic pain is common in patients with chronic lung disease [156,157] and CTSS activity has been linked to pain in several conditions. The inflammatory chemokine CX3CL1/fractalkine, associated with inflammatory lung diseases [158], is proteolytic cleaved by CTSS resulting in the promotion of pain signaling [50,159].…”

Section: Other Outcomesmentioning

confidence: 99%

Cathepsin S: investigating an old player in lung disease pathogenesis, comorbidities, and potential therapeutics

et al. 2020

View full text Add to dashboard Cite

Dysregulated expression and activity of cathepsin S (CTSS), a lysosomal protease and a member of the cysteine cathepsin protease family, is linked to the pathogenesis of multiple diseases, including a number of conditions affecting the lungs. Extracellular CTSS has potent elastase activity and by processing cytokines and host defense proteins, it also plays a role in the regulation of inflammation. CTSS has also been linked to G-coupled protein receptor activation and possesses an important intracellular role in major histocompatibility complex class II antigen presentation. Modulated CTSS activity is also associated with pulmonary disease comorbidities, such as cancer, cardiovascular disease, and diabetes. CTSS is expressed in a wide variety of immune cells and is biologically active at neutral pH. Herein, we review the significance of CTSS signaling in pulmonary diseases and associated comorbidities. We also discuss CTSS as a plausible therapeutic target and describe recent and current clinical trials examining CTSS inhibition as a means for treatment. Proteases in pulmonary diseases Research in the last 60 years has demonstrated that proteases are critical contributors to pulmonary disease pathophysiology. Initially known as protein-degrading enzymes with a restricted spectrum of substrates, recent studies have revealed that the diversity of protease substrates and biological effects triggered by their processing is vast [1, 2]. Proteases are primarily known for their matrix degradation capabilities, but also play significant roles in other biological mechanisms such as angiogenesis, growth factor bioavailability, cytokine processing, receptor shedding and activation, as well as cellular processes including migration, proliferation, invasion, and survival [3]. Importantly, protease activity requires tight regulation, and disruption of the close interplay between proteases, substrates and inhibitors may contribute to the pathogenesis and progression of a variety of pulmonary diseases, including muco-inflammatory diseases such as cystic fibrosis (CF) and chronic obstructive pulmonary disease (COPD), idiopathic pulmonary fibrosis (IPF), as well as infection [4]. In pulmonary diseases with a high neutrophil burden such as CF, a protease:antiprotease imbalance is frequently observed. The activity of proteases such as neutrophil elastase (NE) in the respiratory tract is regulated by antiproteases, such as α1-antitrypsin (A1AT) [5], secretory leukoprotease inhibitor (SLPI) [6] and elafin [7]. However, in diseases like CF, the antiproteases are overburdened by their cognate proteases and this imbalance can result in chronic airway inflammation, decreased mucociliary clearance, mucus obstruction, extracellular matrix (ECM) remodeling, increased susceptibility to infection and impaired immune responses [8].

show abstract

Section: Other Outcomesmentioning

confidence: 99%

Cathepsin S: investigating an old player in lung disease pathogenesis, comorbidities, and potential therapeutics

et al. 2020

View full text Add to dashboard Cite

show abstract

“…Some studies have utilized well-established assessment measures to evaluate pain in patients with CF [71][72][73][74]. Approximately 42-84% of patients with CF experience pain [75], with headache, abdominal, back, chest, and limb pain being the most commonly identified [71,73,75]. Pain is significantly related to decreased daily activity, altered quality of life, and interference with treatments [71][72][73]75].…”

Section: Cystic Fibrosismentioning

confidence: 99%

“…Approximately 42-84% of patients with CF experience pain [75], with headache, abdominal, back, chest, and limb pain being the most commonly identified [71,73,75]. Pain is significantly related to decreased daily activity, altered quality of life, and interference with treatments [71][72][73]75]. Chest pain, headaches, back, joint, and limb pain also occur in patients with CF [72,76,77].…”

Section: Cystic Fibrosismentioning

confidence: 99%

Sleep and Pain in Pediatric Illness: A Conceptual Review

et al. 2016

View full text Add to dashboard Cite

show abstract

“…Previous studies of acute, chronic, and/or procedural pain in CF have suggested that it occurs frequently in both children 13,16,17,21 and adults, 12,15,16,18,19 with prevalence rates ranging from 6% to 94%. 29 Although younger age has been identified as a risk factor to contribute to pain during medical procedures in children with chronic diseases other than CF, 30 we could not detect any age-dependent differences in pain 16 were unable to find an association between age and prevalence of pain perception.…”

Section: Respiratory Culture Sampling-related Painmentioning

confidence: 64%