Individuals with interstitial lung disease (ILD) often experience worsening symptoms and activity avoidance. Limited data exists on outcome measures for assessing functional status (capacity and performance), as well as on the effectiveness of pulmonary rehabilitation (PR) in improving these outcomes in ILD.

Although smoking-related interstitial lung diseases (SR-ILD) are a relatively rare group of entities, they are a relevant public health problem of growing importance, both because they affect young adults and because of their increasing prevalence in recent years due to increased tobacco consumption. In patients who smoke and have non-specific respiratory symptoms, SR-ILD should be ruled out, a term that encompasses a group of different entities in which the basis for diagnosis is the smoking history together with compatible respiratory functional findings, radiology and/or histology. An association has been established between tobacco smoke and a group of diseases that include respiratory bronchiolitis-associated interstitial lung disease (2%-3% of all ILD), desquamative interstitial pneumonia (<1%), Langerhans cell histiocytosis (3%-5%) and acute eosinophilic pneumonia. Smoking is considered a risk factor for idiopathic pulmonary fibrosis which has also been called combined fibroemphysema (5%-10% of all ILD); however, the role and impact of smoking in its development, remains to be determined. The likely interconnection between the mechanisms involved in inflammation and pulmonary fibrosis in all these processes often results in an overlapping of clinical, radiological, and histological features. In the absence of robust scientific evidence on its management, smoking cessation is the first measure to be taken into account. Although most diseases have a benign clinical course after smoking cessation, some cases may progress to chronic respiratory failure.

Pulmonary rehabilitation (PR) is highly effective but underutilised. Pathways to home-based PR (HBPR) from general practice could improve utilisation, but program fidelity in this setting is unknown. This study aimed to explore the fidelity of HBPR in people referred from general practice. Secondary analysis of intervention-group data from two-arm cluster RCT (RADICALS-interdisciplinary intervention for people with COPD including smoking cessation support, home medicine reviews and 8-weeks HBPR). HBPR fidelity assessed by the extent to which exercise training was prescribed according to protocol. Completion of HBPR and contributing factors were determined. 107 participants (68% of intervention group) were referred to HBPR, with = 75 (70%) commencing the program (mean age 68 years, FEV 65% predicted, median mMRC 1). Aerobic training was prescribed according to protocol for 74% of participants in week one, and on average 89% of participants in weeks 2-8. Resistance training was prescribed according to protocol for 98% and 88% of participants (Week 1 and Weeks 2-8, respectively). Rehabilitation completers ( = 57, 76%) were 26 times more likely to have attended the Week 2 phone call (95% CI 2-352). Clinically meaningful improvements were achieved in health-related quality of life (SGRQ) and health status (CAT) following rehabilitation. PR program fidelity can be maintained when delivering HBPR to people with COPD referred directly from general practice. Early engagement with PR may be key to supporting rehabilitation completion.

Idiopathic pulmonary fibrosis (IPF) is characterized by progressive fibrosis of the lung parenchyma, resulting in respiratory failure. This study analysed differences in patient characteristics and antifibrotic treatment strategies during the first years after IPF diagnosis.

COVID-19 patients experience respiratory muscle damage, leading to reduced respiratory function and functional capacity often requiring mechanical ventilation which further increases susceptibility to muscle weakness. Inspiratory muscle training (IMT) may help mitigate this damage and improve respiratory function and functional capacity. We studied the effects of IMT on muscle damage biomarkers, respiratory function, and functional capacity in COVID-19 recovered young adults, successfully weaned from mechanical ventilation. Participants were randomly allocated to either an IMT ( = 11) or control (CON; = 11) intervention for 4 weeks. The IMT group performed 30 dynamic inspiratory efforts twice daily, at 50% of their maximal inspiratory mouth pressure (P) while the CON group performed 60 inspiratory efforts at 10% of daily. Serum was collected at baseline, week two, and week four to measure creatine kinase muscle-type (CKM), fast skeletal troponin-I (sTnI) and slow sTnI. Time × group interaction effects were observed for CKM and slow sTnI, but not for fast sTnI. Both were lower at two and 4 weeks for the IMT compared to the CON group, respectively. Time × group interaction effects were observed for forced expiratory volume in 1s, forced vital capacity, P and right- and left-hand grip strength. These were higher for the IMT compared to the CON group. Four weeks of IMT decreased muscle damage biomarkers and increased respiratory function and grip strength in recovered COVID-19 patients after weaning from mechanical ventilation.

The use of non-invasive ventilation (NIV) in patients with advanced cystic fibrosis (CF) has increased in recent years. Research evidence supports its clinical benefits, but less is known about the patients' experience of its long-term use in a domiciliary setting. To investigate patients' lived experience of using long-term domiciliary NIV. Semi-structured, qualitative interviews were conducted with adults with CF using long-term domiciliary NIV for respiratory failure. The data collected were subject to thematic analysis. Nine adults (6 female), 5 of whom were awaiting lung transplantation, with a mean age of 39 years and mean FEV per cent predicted of 28%, were recruited. Data analysis revealed 2 themes: gratitude, and determination despite challenges. Patients identified some troubling side effects from NIV but were grateful for its symptomatic relief and were determined to continue using it to improve their quality of life. Participants reported experiences of NIV to be generally positive in terms of symptom relief and quality of life. These findings provide an initial insight into patients' experience of NIV and have the potential to help guide and improve care.

A 57-year-old female presented with a chief complaint of cough, with productive yellow sputum particularly severe in the morning. Bronchoscopy revealed inflammatory changes in both main bronchi, with abundant white purulent secretions and necrotic material adhering to the luminal surface. Histopathological examination showed chronic inflammatory changes in the mucosal tissue, with mild hyperplasia of the local squamous epithelium and evidence of keratinization in the surrounding area, consistent with a diagnosis of tracheal mucosal keratosis. The patient was treated with anti-infection therapy, combined with symptomatic supportive treatments such as cough suppression, expectorants, and bronchodilation, resulting in an improvement of symptoms. Analysis of previous literature revealed that although this condition lacks specific clinical manifestations and signs, it exhibits a typical pattern of tracheal wall lesions, and bronchoscopy and histopathological examination have high diagnostic value for this disease. Due to impaired airway mucosal barrier function, the patient is prone to bacterial infection or colonization in the airways. Symptomatic supportive treatment based on the actual condition can effectively alleviate the patient's clinical symptoms. Currently, there is no definitive curative treatment for tracheal mucosal keratosis. However, treatments such as high-frequency electrocautery, carbon dioxide cryotherapy, and repeated lavage with epidermal growth factors may be helpful in curing this condition.

This study aimed to understand factors that health professionals, from a variety of healthcare contexts and countries, believed support remote delivery of pulmonary rehabilitation (PR); and to develop a targeted intervention to support implementation of remote PR. A 3-phase participatory action-research process was employed, across three study hubs in three countries (NZ, India, USA), representing diverse healthcare delivery contexts. Phase 1 employed focus groups of health professionals working in PR; data were analysed qualitatively with transcripts coded against two implementation frameworks (Theoretical Domains Framework (TDF) and Consolidated Framework for Implementation Research (CFIR)). Findings informed development of an online toolbox to support delivery of remote PR (Phase 2), which was evaluated using semi-structured interviews (Phase 3). 20 health professionals participated across all study phases. Factors considered to influence implementation of remote PR were consistent across diverse healthcare contexts and related to staffing availability, skills and confidence, and equipment and technology accessibility. An online toolbox provided support for enhancing knowledge and confidence, but was not able to address all implementation barriers. Key factors to support clinicians deliver remote PR are common across different healthcare contexts, suggesting broader telerehabilitation implementation strategies may be applicable across healthcare environments.

Health inequalities can affect access and uptake to pulmonary rehabilitation (PR). An individual's protected characteristics (age, disability, gender reassignment, marriage and civil partnership, pregnancy and maternity, race, religion or belief, sex and sexual orientation) may contribute to health inequalities. Healthcare professionals (HCPs) experiences of the inclusivity and representativeness of PR services and knowledge of protected characteristics are unknown, however are vital for the identification and resolution of health inequalities. This qualitative study explored HCPs understanding of protected characteristics and their perception of the inclusivity, representativeness and equitable benefit of their PR services.

Primary Ciliary Dyskinesia (PCD) is a rare genetic disorder requiring airway clearance techniques for mucus removal. We aimed to evaluate the feasibility and the effect of the active cycle of breathing technique (ACBT) versus oscillating positive expiratory pressure therapy (OPEP) in improving lung function and functional exercise capacity among children with PCD in Palestine. 32 PCD children (6-18 years) were included in a 12-week home-based feasibility study. They were assigned randomly into two groups: ACBT and OPEP. Data collection included spirometry measurements, and the six-minute walk test (6MWT). After 12 weeks of regular airway clearance techniques (ACT), the FEV, MEF, and the 6MWT demonstrated statistically significant differences ( = .02, = .04, and = .05 respectively) between the two groups, in favor of the OPEP group with the effect size of Cohen's d (0.86, 0.76, and 0.71) respectively. However, there was no significant difference ( > .05) between the two groups in FVC and FEV/FVC. Additionally, only in the OPEP group, significant differences were recorded between pre and post-tests for FEV1 and 6MWT ( < .05). The randomized study design comparing ACBT and OPEP was feasible and acceptable to patients. OPEP demonstrates potential for managing respiratory health; however, treatments should be individualized to address each patient's specific needs. Further research with larger cohorts is needed to assess the effectiveness of both methods.

Pulmonary rehabilitation (PR) services are increasingly using alternative programme delivery modes, for example telerehabilitation strategies including videoconferencing, to improve patient choice and accessibility. Although telerehabilitation results in improvements in core outcomes, the effect on knowledge attainment is not known. To observe the real-world responses of patients choosing to undergo videoconference PR to a matched control group choosing to undergo in-person PR, in terms of knowledge attainment. Using propensity score matching, 25 people with COPD who completed videoconference PR were matched 1:1 with a control group of 25 people with COPD who completed in-person PR. Knowledge attainment was measured using the Lung Information Needs Questionnaire (LINQ). There was a statistically and clinically significant improvement in LINQ score in both groups (mean (95%CI): videoconference -3.2 (-4.7 to -1.6); in-person -3.0 (-4.5 to -1.4)), with no significant between-group difference (mean (95%CI): 0.2 (-2.0 to -2.4)). 76% and 80% of participants achieved the minimal important difference of the LINQ in the videoconference and in-person PR groups respectively. In conclusion, this real-world service evaluation indicates that videoconference PR may be associated with similar improvements in knowledge attainment as in-person PR, but this requires corroboration due to the small sample size.

Individuals with Preserved Ratio Impaired Spirometry (PRISm), defined as FEV/FVC ≥0.7 and FEV1 <80% predicted, are at higher risk of developing COPD. However, data for Australian adults are limited. We aimed to describe prevalence of PRISm and its relationship with clinical characteristics in Australia. Data from the Burden of Lung Disease (BOLD) Australia study of randomly selected adults aged ≥40 years from six sites was classified into airflow limitation, PRISm, or normal spirometry groups. Demographic, clinical characteristics, and lung function were compared between groups. Of the study sample ( = 3518), 387 (11%) had PRISm, 549 (15.6%) had airflow limitation, and 2582 (73.4%) had normal spirometry. PRISm was more common in Indigenous Australian adults. Adults with PRISm had more frequent respiratory symptoms, more comorbidities, greater health burden and poorer quality of life than those with normal spirometry. Pre- and post-bronchodilator FEV and FVC were lower in adults with PRISm than those with airflow limitation. Adults with PRISm were less likely to use respiratory medicine than those with airflow limitation (OR = 0.56, 95% CI 0.38-0.81). PRISm was present in 11% of adults in this study and they had similar respiratory symptoms and health burden as adults with airflow limitation.

The efficacy and safety of ensifentrine, a novel PDE3/PDE4 inhibitor, were previously evaluated in the ENHANCE-1 (NCT04535986) and ENHANCE-2 (NCT04542057) trials. Here, we present a pooled post-hoc subgroup analysis of patients according to background chronic obstructive pulmonary disease (COPD) maintenance medication regimens.

To explore the feasibility and effect of a nurse-led integrated care intervention on health-related quality of life (QoL) and unplanned 90-day rehospitalisation in patients hospitalised due to acute exacerbation of COPD (AECOPD).

The paradigm of bronchiectasis is shifting away from its exclusive characterization as a neutrophilic condition. Patients with bronchiectasis and high eosinophil levels have been found to have a specific phenotype, but the clinical effect of eosinopenia remains unclear.

Chronic obstructive pulmonary disease (COPD) is associated with a reduced exercise capacity. Although several field tests for exercise capacity have been modified for non-standard settings, i.e. outside the hospital clinic or pulmonary rehabilitation center, their uptake remains limited. To assess the test-retest reliability, constuct validity and responsiveness of the one-minute sit-to-stand test (1'STST) adopted in clinical practice among patients with COPD and to confirm the earlier established minimal important difference (MID) of three repetitions. Patients with COPD performed two 1'STSTs, two 6-minute walk tests (6MWT), an isometric quadriceps force (QF) measurement, a cardiopulmonary exercise test (CPET), and a seven-day physical activity (PA) measurement before and after three months of pulmonary rehabilitation (PR). An Intraclass Correlation Coefficient (ICC) evaluated the agreement between two 1'STSTs. Pearson Correlation examined the association between the 1'STST and other physical measurements, and their changes following PR. A receiver operating characteristic (ROC) curve was constructed using a 30-meter increment in the 6MWT as cut-off to identify responders. The 1'STST demonstrated good reliability (Δ0.9 ± 4.0 repetitions, = .13; ICC = 0.79). The 1'STST was moderately correlated with the 6MWT (r = 0.57, < .0001), VOmax (r = 0.50, = .0006) and maximal work rate (r = 0.52, = .0003). Weak correlations were observed with QF (r = 0.33, = .03) and step count (r = 0.38, = .013). The 1'STST improved after PR (∆ = 3.6 ± 6.4 repetitions, = .0013) and changes correlated moderately with changes in the 6MWT (r = 0.57, = .002), QF (r = 0.48, = .003) and VOmax (r = 0.41, = .014). A cut-off of three repetitions demonstrated a 71% accuracy in identifying responders to a rehabilitation program. The 1'STST is a valuable alternative to evaluate exercise capacity in patients with COPD when more expensive and time-consuming tests are unavailable.

Increasing uptake and completion of Pulmonary Rehabilitation in people with COPD has the potential to deliver health benefit and reduce health inequalities. We have quantified the cost-effectiveness of enhancing PR access and completion by reviewing the cost-effectiveness literature for PR in COPD. A literature review identified studies that provided cost-effectiveness evidence for PR compared to no PR. The key metrics of interest were healthcare resource use and cost savings, and quality adjusted life year (QALY) gains. Healthcare resource use data were valued using the UK NHS National Tariff 2022/23. From the literature search we identified the QALY gain resulting from completion of PR. The value of the QALY gain resulting from PR completion was calculated using the standard willingness-to-pay threshold of £20,000 considered by the UK National Institute for Health and care Excellence (NICE). We estimated a QALY gain resulting from completion of PR of 0.065 and value of the QALY gain was therefore calculated to be £1300 per person completing PR. We estimated the 12 month reduction in hospitalisation following completion of PR to be 8.2% giving a total cost reduction per patient of £245. We therefore calculated that up to £1545 could be spent per person with COPD to deliver PR cost-effectively. Our analysis provides commissioners with the information they need to make informed decisions about planning and provision of PR. The data allows estimation of additional resources that could be deployed in addressing inequitable access to PR among disadvantaged and underserved populations whilst retaining cost effectiveness of the intervention.

To evaluate the impact of participant-selected music listening as an adjunct to pulmonary rehabilitation (PR) in people with COPD. Adults with COPD referred to PR were randomly assigned to participant-selected music listening (intervention group, [IG]) or usual care (control group [CG]) during an 8-weeks PR program. Prior to training, the IG completed an interview with a registered music therapist to identify music preferences. IG participants listened to an individualised playlist; CG participants had usual care. Primary outcomes included end-6-min walk test symptoms (dyspnoea and exertion) and dyspnoea (Multidimensional Dyspnoea Profile [MDP]), measured pre and post PR and 6-months follow-up. 58 participants, FEV1 52.4 (25.9)% pd) were recruited. There were no between-group differences following the intervention ( > .05 for all outcomes at all time points). Within-group differences following PR were significant for MDP sensory quality: IG mean difference [95% CI] -2.2 [-3.3 to -1.2]; CG -1.5 [-2.5 to -0.5] points; MDP emotional response: IG -3.2 [-4.2 to -2.3]; CG -2.2 [-3.2 to -1.3] points). Participant-selected music listening during PR offered no greater benefit to symptoms of dyspnoea or exertion compared to usual care. With the study limited by COVID-19 restrictions, the role of this adjunct remains to be clarified.

The diagnosis and management of common chronic respiratory diseases depend on various parameters obtained from pulmonary function tests (PFTs), such as spirometry, plethysmography, and carbon monoxide diffusion capacity (DLCO). These tests are interpreted following guidelines established by reputable scientific societies like the European Respiratory Society and the American Thoracic Society (ERS/ATS). This review aimed to offer a comprehensive framework for interpreting PFTs, incorporating the latest ERS/ATS update (i.e.; 2022), and to briefly explore some complex cases to shed light on their implications for understanding PFTs. The ERS/ATS update outlines a systematic approach to interpreting PFT results, which involves several steps. Initially, results are compared to those of a healthy reference population to determine normal, low, or high parameters. Then, potential ventilatory impairments (VIs), such as obstructive or restrictive VIs, are identified, which could indicate specific chronic respiratory or extra-respiratory diseases. The severity of identified VIs or reductions in DLCO is then assessed. If bronchodilator testing is performed, its response is evaluated. Lastly, any significant changes in PFT parameters over time are noted by comparing current results with previous ones, if available. Despite the clarity provided by the ERS/ATS update, certain uncertainties persist and require clarification, such as the identification of new patterns (e.g.; non-obstructive abnormal spirometry, isolated low forced expiratory volume in 1 s), and classifications of mixed VI or lung hyperinflation in terms of functional severity. This review is a comprehensive framework for interpreting PFTs. Since some issues pose uncertainty in clinical practice, it would be beneficial to the ERS/ATS to reconcile some inconsistencies and provide clearer guidance on different classifications and VIs.

Despite the fact that inhaled medications serve as the foundation of chronic obstructive pulmonary disease (COPD) treatment, patient adherence to inhaler therapy remains low, significantly impacting health outcomes in disease management. The Common Sense Model of Self-Regulation suggests that illness perception plays a crucial role in individual behavior. Nevertheless, the relationship between illness perception and inhaler adherence, as well as the underlying mechanisms, remains unclear in the elderly Chinese COPD population. This study aimed to explore the correlation between dimensions of illness perception and adherence to inhaler therapy in elderly Chinese patients with COPD. A cross-sectional study was conducted by recruiting 305 participants (mean age: 70.96 years; 69.8% male) using convenience sampling from a tertiary hospital in Anhui, China. The Chinese versions of the Test of Adherence to Inhalers (TAI) and Brief Illness Perception Questionnaire (B-IPQ) were used to evaluate adherence to inhalation and perception of their illness in patients with COPD. Binary logistic regression analyses were used to explore the relationship between inhaler adherence and illness perception in patients with COPD. 84.3% of participants showed poor adherence, and the mean (standard deviation) B-IPQ total score was 44.87 (6.36). The results indicated an essential correlation between illness perception and inhaler adherence. Specifically, personal control (AOR = 2.149, < 0.001), treatment control (AOR = 1.743, < 0.001), comprehension (AOR = 5.739, < 0.001) and emotions (AOR = 1.946, < 0.001) within illness perception emerged as significant positive predictors for inhaler adherence among patients with COPD. This study suggests that clinical practitioners should monitor the illness perception of patients with COPD and develop targeted intervention measures to improve patient adherence to inhaler therapy.

To assess the safety, reliability and acceptability of the modified incremental step test (MIST) supervised remotely via videoconferencing in adults with chronic respiratory disease.