Incidence of bronchiectasis in patients with acromegaly: a cohort study.

Objective: Associations between acromegaly and several respiratory diseases, such as obstructive lung disease or sleep apnea, have been suggested, but the relationship between bronchiectasis and acromegaly is unclear. We investigated whether acromegaly is related to the development of bronchiectasis. Materials and methods: Using the Korean National Health Insurance System database between 2006 and 2016, we studied the relationship between acromegaly and bronchiectasis in patients with acromegaly (n=2593) and controls (1:5 age- and sex-matched subjects without acromegaly, n=12965) with a mean follow-up period of 8.9 years. Cox proportional hazards regression analysis was used to assess the risk of bronchiectasis in patients with acromegaly compared with controls after adjusting for age, sex, household income, place, type 2 diabetes, hypertension, and dyslipidemia. Results: The mean age of the participants was 47.65 years, and male subjects comprised 45.62% of the cohort. The incidence rate of bronchiectasis in patients with acromegaly was 3.64 per 1,000 person-years and was significantly higher than that in controls (2.47 per 1,000 person-years) (log-rank test p = 0.002). In multivariable Cox proportional hazards regression modeling, the risk of bronchiectasis was significantly higher in patients with acromegaly than that in controls (HR: 1.49; 95% CI: 1.15-1.94, p = 0.0025) after adjusting for age, sex, household income, place, type 2 diabetes, hypertension, and dyslipidemia. Conclusions: Our results suggest that acromegaly may be associated with bronchiectasis.

New-onset obstructive airway disease following COVID-19: a multicenter retrospective cohort study.

BACKGROUND: The study assessed the association between COVID-19 and new-onset obstructive airway diseases, including asthma, chronic obstructive pulmonary disease, and bronchiectasis among vaccinated individuals recovering from COVID-19 during the Omicron wave. METHODS: This multicenter retrospective cohort study comprised 549,606 individuals from the U.S. Collaborative Network of TriNetX database, from January 8, 2022, to January 17, 2024. The hazard of new-onset obstructive airway diseases between COVID-19 and no-COVID-19 groups were compared following propensity score matching using the Kaplan-Meier method and Cox proportional hazards model. RESULTS: After propensity score matching, each group contained 274,803 participants. Patients with COVID-19 exhibited a higher risk of developing new-onset asthma than that of individuals without COVID-19 (adjusted hazard ratio (aHR), 1.27; 95% CI, 1.22-1.33; p < 0.001). Stratified analyses by age, SARS-CoV-2 variant, vaccination status, and infection status consistently supported this association. Non-hospitalized individuals with COVID-19 demonstrated a higher risk of new-onset asthma (aHR, 1.27; 95% CI, 1.22-1.33; p < 0.001); however, no significant differences were observed in hospitalized and critically ill groups. The study also identified an increased risk of subsequent bronchiectasis following COVID-19 (aHR, 1.30; 95% CI, 1.13-1.50; p < 0.001). In contrast, there was no significant difference in the hazard of chronic obstructive pulmonary disease between the groups (aHR, 1.00; 95% CI, 0.95-1.06; p = 0.994). CONCLUSION: This study offers convincing evidence of the association between COVID-19 and the subsequent onset of asthma and bronchiectasis. It underscores the need for a multidisciplinary approach to post-COVID-19 care, with a particular focus on respiratory health.

A systematic literature review of the clinical and socioeconomic burden of bronchiectasis.

BACKGROUND: The overall burden of bronchiectasis on patients and healthcare systems has not been comprehensively described. Here, we present the findings of a systematic literature review that assessed the clinical and socioeconomic burden of bronchiectasis with subanalyses by aetiology (PROSPERO registration: CRD42023404162). METHODS: Embase, MEDLINE and the Cochrane Library were searched for publications relating to bronchiectasis disease burden (December 2017-December 2022). Journal articles and congress abstracts reporting on observational studies, randomised controlled trials and registry studies were included. Editorials, narrative reviews and systematic literature reviews were included to identify primary studies. PRISMA guidelines were followed. RESULTS: 1585 unique publications were identified, of which 587 full texts were screened and 149 were included. A further 189 citations were included from reference lists of editorials and reviews, resulting in 338 total publications. Commonly reported symptoms and complications included dyspnoea, cough, wheezing, sputum production, haemoptysis and exacerbations. Disease severity across several indices and increased mortality compared with the general population was reported. Bronchiectasis impacted quality of life across several patient-reported outcomes, with patients experiencing fatigue, anxiety and depression. Healthcare resource utilisation was considerable and substantial medical costs related to hospitalisations, treatments and emergency department and outpatient visits were accrued. Indirect costs included sick pay and lost income. CONCLUSIONS: Bronchiectasis causes significant clinical and socioeconomic burden. Disease-modifying therapies that reduce symptoms, improve quality of life and reduce both healthcare resource utilisation and overall costs are needed. Further systematic analyses of specific aetiologies and paediatric disease may provide more insight into unmet therapeutic needs.

Bronchiectatic Actinomycosis with Osseous Metaplasia Masquerading as Lung Cancer.

After the publication of the original article, the authors noticed an error in the departmental affiliation of one of the contributors, Don MASCARENHAS. The corrected version of the department is provided below, and the original article has been updated accordingly. Archana BHAT < sup > 1 < /sup > , Manjunath J < sup > 1 < /sup > , Don MASCARENHAS < sup > 2 < /sup > < br / > Department of < sup > 1 < /sup > Pathology and < sup > 2 < /sup > Pulmonology, Father Muller Medical College, MANGALORE, INDIA.

[The impact of diabetes on bronchiectasis].

Objective: To study the impact of diabetes on bronchiectasis. Methods: From January 1 2017 to December 31 2021, data of 1 310 inpatients with bronchiectasis were retrospectively collected from Shanghai Pulmonary Hospital, Tongji University. This cohort inclueded 78 patients with bronchiectasis and diabetes (bronchiectasis-diabetes group) and 1 232 patients of bronchiectasis without diabetes (simple bronchiectasis group). To mitigate confounding variables, 269 patients with bronchiectasis but without diabetes (bronchiectasis non-diabetes group) were matched with 78 patients of bronchiectasis with diabetes (bronchiectasis-diabetes group) using the propensity score method in a 1∶4 ratio. A comparative analysis of clinical characteristics, laboratory test results, pathogenic infections, and disease severity was performed between the two groups. Analysis of continuous variables was performed using either a t-test or non-parametric test, while categorical data was assessed using the chi-square test. Results: The mean age of individuals in the bronchiectasis-diabetes group (62.99±8.09 years) was significantly higher than that of subjects in the simple bronchiectasis group (57.05±13.07 years) (t=-6.012, P<0.001). After propensity score matching, the pre-albumin level in the bronchiectasis-diabetes group (188.44±71.65 g/L) was found to be lower than in the bronchiectasis non-diabetes group (208.17±62.50 g/L) (t=2.023, P=0.044). In addition, the hospitalization cost for the bronchiectasis-diabetes group [1.59 (1.34, 2.15) Ten thousand yuan] was higher than that in the bronchiectasis non-diabetes group [1.39 (1.23, 1.62) Ten thousand yuan] (U=-3.744, P<0.001).The severity of mMRC in the bronchiectasis-diabetes group was significantly higher than that in the bronchiectasis non-diabetes group (χ2=25.392, P<0.001), and the frequency of previous hospitalization due to aggravation and acute exacerbation within the previous year were higher than in bronchiectasis non-diabetes group (χ2=34.031, 40.841, respectively, P<0.001). In additional, the BSI score was significantly increased in patients with bronchiectasis-diabetes compared to those with bronchiectasis non-diabetes (8.07±4.07 vs. 10.44±3.82) (P<0.001). Furthermore, fasting blood glucose concentration, urine glucose level, and BSI score exhibited positive correlations, whereas pre-albumin concentration showed a negative correlation with the BSI score (all P<0.05). Conclusion: Compared to those without diabetes, patients of bronchiectasis with diabetes have poorer nutritional status, more severe symptoms, increased risk of acute exacerbation, higher BSI score severity, and greater financial burden.

Early and late outcomes of video thoracoscopic versus open approach for bronchiectasis lung resections.

OBJECTIVES: To evaluate the early and late results of surgical treatment of patients with bronchiectasis, comparing the Video-Assisted Thoracic Surgery (VATS) vs. the open thoracotomy (OT). METHODS: Observational retrospective study of patients who underwent surgery for bronchiectasis. Patients were divided into two groups according to surgical access OT/VATS. Variables collected included gender, age, preoperative symptoms, etiology, segments involved, FVC and FEV1, type of surgical resection, complications, mortality, and length of hospital stay. Late surgical results were classified as excellent, complete remission of symptoms; good, significative improvement; and poor, little/no improvement. RESULTS: 108 surgical resections (103 patients). OT group 54 patients (52.4%) vs. VATS 49 (47.6%). A high percentage of complications was observed, but no difference between the OT (29.6%) and VATS (24.5%) groups was found. Post-operative hospital stay was shorter in the VATS group (5.4 days) vs. the OT group (8.7 days (p=0.029). 75% of the patients had a late follow-up; the results were considered excellent in 71.4%, good in 26%, and poor in 2.6%. Regarding bronchiectasis distribution, an excellent percentage was obtained at 82.1% in patients with localized bronchiectasis and 47.5% with non-localized bronchiectasis, p=0.003. CONCLUSIONS: VATS leads to similar results regarding morbidity, compared to OT. However, VATS was related to shorter hospital stays, reflecting the early recovery. Late results were excellent in most patients, being better in patients with localized bronchiectasis. VATS should be considered a preferable approach for bronchiectasis lung resection whenever possible.

Nontuberculous mycobacterial pulmonary disease presenting as bronchiolitis pattern on CT without cavity or bronchiectasis.

BACKGROUND: This study aimed to investigate the radiological changes in patients with nontuberculous mycobacterial pulmonary disease (NTM-PD) having bronchiolitis patterns on computed tomography (CT). METHODS: We retrospectively reviewed the final diagnosis and radiologic changes of patients suspected of having NTM-PD without cavity or bronchiectasis on CT image, between January 1, 2005 and March 31, 2021. NTM-PD was diagnosed based on the American Thoracic Society and Infectious Diseases Society of America criteria. The initial and final CT findings (bronchiectasis, cellular bronchiolitis, cavity formation, nodules, and consolidation) were compared between patients diagnosed with and without NTM-PD. RESULTS: This study included 96 patients and 515 CT images. The median CT follow-up duration was 1510.5 (interquartile range: 862.2-3005) days. NTM-PD was recognized in 43 patients. The clinical variables were not significantly different between patients with and without NTM-PD, except for underlying chronic airway disease (P < 0.001). Nodule and consolidation were more frequently observed on the initial CT scans of patients with NTM-PD compared with those without (P < 0.05). On the final follow-up CT scan, bronchiectasis (P < 0.001), cavity (P < 0.05), nodule (P < 0.05), and consolidation (P < 0.05) were more frequently observed in patients with NTM-PD. Among the 43 patients with NTM-PD, 30 showed a radiological progression on CT, with bronchiectasis (n = 22) being the most common finding. The incidence of bronchiectasis increased over time. CONCLUSION: The bronchiolitis pattern on CT images of patients with NTM-PD showed frequent radiological progression during the follow-up period.

Current physiotherapy practice for adults with bronchiectasis: Data from the Australian bronchiectasis registry.

BACKGROUND: Although airway clearance techniques (ACTs) and physical exercise are recommended for adults with bronchiectasis, there is little data on current practice and limited guidance predicting clinical approach. OBJECTIVE: This study aimed to describe current ACT and exercise practice recorded by patients, and identify predictors of regular ACTs, ACT modalities and exercise. METHODS: Physiotherapy-specific interventions, quality of life (Quality-of-Life Bronchiectasis questionnaire, QOL-B), demographics and disease severity were extracted from the Australian Bronchiectasis Registry. Multivariate analyses were undertaken to identify predictors of undertaking ACTs or exercise. RESULTS: We included 461 patients; median age of 72 years (interquartile range 64-78 years). Regular ACT use was recorded by 266 (58 %) patients; the active cycle of breathing technique (n = 175, 74 %) was the most common technique. Regular exercise use was recorded by 213 (46 %) patients, with walking the most common form of exercise. A pulmonary rehabilitation referral was made for 90 (19.5 %) of patients. Regular ACT use was associated with a higher treatment burden on QOL-B (Odds ratio (OR) = 0.97, 95 % confidence interval (CI) 0.96 to 0.99). Regular exercise was more likely amongst patients with severe bronchiectasis compared to those with mild disease (OR = 9.46, 95 % CI 1.94 to 67.83) and in those with greater physical function on the QOL-B (OR = 1.02, 95 % CI 1.01 to 1.04). CONCLUSION: Approximately half the adults in the registry report regular ACT or exercise; QOL and disease severity predict this engagement. This knowledge may guide the tailoring of ACTs and exercise prescription to optimise physiotherapy management in adults with bronchiectasis.

Real-world treatment patterns, health care resource utilization, and costs in a US Medicare population with bronchiectasis.

BACKGROUND: Bronchiectasis carries a significant economic burden with high health care expenditures associated with frequent hospitalizations, physician visits, and treatments, including oral and intravenous antibiotics for repeated lung infections, airway-clearance therapy, and oxygen administration. Bronchiectasis exacerbations can contribute to this burden. OBJECTIVE: To estimate US health care resource utilization (HCRU) and costs associated with bronchiectasis and with bronchiectasis exacerbations. METHODS: This retrospective study used the 100% Medicare Fee-for-Service database (January 2014 to December 2020) to compare HCRU and costs among patients with bronchiectasis with those of patients without bronchiectasis (controls). For patients with bronchiectasis, the index date was a randomly selected bronchiectasis claim after more than 1 year of disease history and, for controls, a claim closest to their matched bronchiectasis patient's index date. All patients had continuous enrollment for at least 12 months pre-index (baseline) and at least 12 months post-index. Primary outcomes were all-cause, respiratory-related, and bronchiectasis-related HCRU and health care costs, which were presented by the overall sample and by segmented patient cohorts based on the number of exacerbations during baseline (0, 1, or ≥2). RESULTS: 92,529 patients with bronchiectasis (mean [SD] age, 76.7 [8.8] years; 72.3% female) and 92,529 matched controls qualified for the study. Compared with controls, patients with bronchiectasis presented greater mean (SD) all-cause physician visits (15.4 [10.0] vs 13.2 [9.7]; P < 0.001) and respiratory-related physician visits (5.2 [4.3] vs 1.9 [3.1]), pulmonologist visits (1.9 [2.2] vs 0.3 [1.0]), hospitalizations (0.4 [0.9] vs 0.3 [0.8]), emergency department visits (0.33 [1.0] vs 0.26 [1.0]), and total health care costs ($10,224 [$23,263] vs $6,704 [$19,593]). Respiratory-related HCRU was also greater in patients with more baseline exacerbations, with total health care costs of $8,506, $10,365, and $14,790 for patients with 0, 1, and at least 2 exacerbations, respectively (P < 0.01). CONCLUSIONS: This real-world study demonstrates the high disease burden associated with bronchiectasis and with exacerbations, highlighting the need to improve management and reduce exacerbations.

Novel cathepsin C inhibitor, BI 1291583, intended for treatment of bronchiectasis: Phase I characterization in healthy volunteers.

Novel treatments are needed to reduce inflammation, improve symptoms, address exacerbations, and slow disease progression in bronchiectasis. Cathepsin C (CatC) inhibition promises to achieve this through reduction of neutrophil-derived serine protease (including neutrophil elastase [NE] and proteinase 3 [PR3]) activation. Here, we present the phase I characterization of the novel CatC inhibitor, BI 1291583. Five phase I trials of BI 1291583 in healthy subjects are presented: a single-rising-dose study (NCT03414008) and two multiple-rising-dose studies (NCT03868540 and NCT04866160) assessing the safety, tolerability, pharmacodynamics, and pharmacokinetics of BI 1291583; a food effect study (NCT03837964); and a drug-drug interaction study (NCT03890887) of BI 1291583 and itraconazole. BI 1291583 was safe and well tolerated across the doses tested in these trials. Most adverse events (AEs) were mild or moderate in intensity, with no serious AEs, AEs of special interest or deaths reported in any trial. Drug-related skin exfoliation was not reported more frequently in subjects treated with BI 1291583 compared with placebo. BI 1291583 was readily absorbed, and pharmacokinetics were supra-proportional over the dose ranges assessed. Additionally, BI 1291583 inhibited CatC in a dose-dependent manner, inhibited downstream NE activity, and decreased PR3 levels. No food effect was observed. Co-administration of multiple doses of itraconazole increased BI 1291583 exposure approximately twofold. Due to these promising phase I results, a multinational phase II program of BI 1291583 in adults with bronchiectasis is ongoing (Airleaf™ [NCT05238675], Clairafly™ [NCT05865886], and Clairleaf™ [NCT05846230]).

Causal associations of obstructive sleep apnea with Chronic Respiratory Diseases: a Mendelian Randomization study.

PURPOSE: This study aimed to elucidate the causal relationship between Obstructive Sleep Apnea (OSA) and Chronic Respiratory Diseases (CRDs), employing Mendelian Randomization (MR) to overcome limitations inherent in observational studies. METHODS: Utilizing a two-sample MR approach, this study analyzed genetic variants as instrumental variables to investigate the causal link between OSA and various CRDs, including chronic obstructive pulmonary disease (COPD), asthma, bronchiectasis, and idiopathic pulmonary fibrosis (IPF). Data were sourced from the FinnGen Consortium (OSA, n = 375,657) and UK Biobank, focusing on genome-wide associations between single-nucleotide polymorphisms (SNPs) and the diseases. Instrumental variables were selected based on strict criteria, and analyses included a random-effects inverse-variance weighted method supplemented by several sensitivity analyses. RESULTS: The study suggests a protective effect of OSA against COPD (OR = 0.819, 95% CI 0.722-0.929, P-value = 0.002), which becomes non-significant after adjusting for BMI, indicating a potential mediating role of BMI in the OSA-COPD nexus. No significant causal links were found between OSA and other CRDs (asthma, IPF, bronchiectasis) or between COPD, asthma, and OSA. CONCLUSIONS: Our findings reveal a BMI-mediated protective effect of OSA on COPD, with no causal connections identified between OSA and other CRDs. These results emphasize the complex relationship between OSA, BMI, and COPD, guiding future clinical strategies and research directions, particularly in light of the study's genetic analysis limitations.

Clinical perspectives in bronchiectasis management.

Non-cystic fibrosis bronchiectasis is a chronic inflammatory airway disease that results in permanent lung damage and can correlate with considerable clinical and economic burden. There are gaps in knowledge surrounding bronchiectasis, for which there are no published US-based treatment guidelines or FDA-approved therapies. Given the current challenges and gaps in care, the authors of this article convened for an AJMC® roundtable in March 2024. This publication summarizes the main findings of that roundtable and situates them in a scholarly context. Panelists agreed that patients with unexplained chronic cough or fatigue, purulent sputum production, hemoptysis, or repeated infection should undergo CT scanning to assess the presence of bronchiectasis, which has been estimated to affect approximately 364,000 to 558,000 individuals at least 18 years of age. They noted that disease symptoms and treatment burden can considerably diminish patient health-related quality of life (HRQOL) and that an exacerbation uniformly signifies deteriorating health and substantially impacts disease progression, hospitalization rates, and mortality. Absent an FDA-approved therapy, panelists' top management priorities were preventing or reducing exacerbations and maintaining or improving HRQOL. Panelists concluded that providers are ill-equipped to change the course of this heterogenous disease and that there is a real need for options to manage symptoms, for US-based guidelines, and for more research into epidemiology, etiology, and treatment.

Swyer-James-Macleod syndrome mimicking pulmonary embolism in adults: a case report.

BACKGROUND: Swyer-James-MacLeod syndrome (SJMS) is a rare lung condition characterized by a unilateral lung hyperlucency and reduction in the pulmonary vasculature, with or without the presence of bronchiectasis. In the 1950s, Swyer, James, and Macleod simultaneously characterized the syndrome for the first time. It is typically diagnosed in childhood. Adult-onset cases are extremely rare, with little literature available on its clinical presentation and diagnostic challenges. Swyer-James-MacLeod syndrome can mimic other lung disorders, resulting in misdiagnosis and improper treatment. CASE PRESENTATION: A 49- year-old woman from Debre Berhan, Ethiopia, presented to the emergency department of Hakim Gizaw Teaching Hospital with symptoms and radiographic findings mimicking acute pulmonary embolism. On the basis of the clinical presentation and radiographic findings, the patient was first treated as a probable case of pulmonary embolism. Anticoagulant therapy and oxygen support were initiated. Nevertheless, additional testing using a chest computed tomography angiography revealed left lung hyperlucency, decreased vascularity, bronchiectasis, and a negative result for pulmonary embolism. As a result, Swyer-James-MacLeod syndrome was diagnosed. CONCLUSION: The symptoms of Swyer-James-MacLeod syndrome can be mistaken for pulmonary embolism, which could lead to ineffective treatment and needless expenses. In individuals presenting with symptoms suggestive of pulmonary embolism, this case emphasizes the significance of considering Swyer-James-MacLeod syndrome as a differential diagnosis, especially in the absence of established risk factors for pulmonary embolism.

Development and validation of a novel questionnaire to describe and assess sensations and triggers associated with refractory and unexplained chronic cough.

INTRODUCTION: Refractory or unexplained chronic cough (RUCC) is a common clinical problem with no effective diagnostic tools. The Sensations and Triggers Provoking Cough questionnaire (TOPIC) was developed to characterise cough in RUCC versus cough in other conditions. METHODS: Content analysis of participant interviews discussing the sensations and triggers of chronic cough informed TOPIC development. Participants with chronic cough completed the draft-TOPIC (a subset repeating 5-7 days later), St George's Respiratory Questionnaire (SGRQ), Cough Severity Diary (CSD) and Global Rating of Change Scale. The draft-TOPIC item list was reduced in hierarchical and Rasch analysis to refine the questionnaire to the TOPIC. RESULTS: 49 items describing the triggers and sensations of cough were generated from participant interviews (RUCC n=14, chronic obstructive pulmonary disease (COPD) n=11, interstitial lung disease (ILD) n=10, asthma n=11, bronchiectasis n=3, cystic fibrosis n=7). 140 participants (median age 60.0 (19.0-88.0), female 56.4%; RUCC n=39, ILD n=38, asthma n=45, COPD n=6, bronchiectasis n=12) completed draft-TOPIC, where items with poor 'fit' for RUCC were removed to create TOPIC (8 trigger items, 7 sensation items). Median TOPIC score was significantly higher in RUCC (37.0) vs ILD (24.5, p=0.009) and asthma (7.0, p<0.001), but not bronchiectasis (20.0, p=0.318) or COPD (18.5, p=0.238), likely due to small sample sizes. The Rasch model demonstrated excellent fit in RUCC (χ2=22.04, p=0.85; PSI=0.88); as expected. When all participant groups were included, fit was no longer demonstrated (χ2=66.43, p=0.0001, PSI=0.89) due to the increased heterogeneity (CI=0.077). TOPIC correlated positively with SGRQ (r=0.47, p<0.001) and CSD (r=0.63, p<0.001). The test-retest reliability of TOPIC (intraclass correlation coefficient) was excellent (r=0.90, p<0.001). CONCLUSIONS: High TOPIC scores in the RUCC patients suggest their cough is characterised by specific sensations and triggers. Validation of TOPIC in cough clinics may demonstrate value as an aid to identify features of RUCC versus cough in other conditions.

Construction of a panoramic mRNA map of adult noncystic fibrosis bronchiectasis and a preliminary study of the underlying molecular mechanisms.

BACKGROUND: The pathogenesis of noncystic fibrosis bronchiectasis in adults is complex, and the relevant molecular mechanisms remain unclear. In this study, we constructed a panoramic map of bronchiectasis mRNA, explored the potential molecular mechanisms, and identified potential therapeutic targets, thus providing a new clinical perspective for the preventive management of bronchiectasis and its acute exacerbation. METHODS: The mRNA profiles of peripheral blood and bronchiectasis tissues were obtained through transcriptome sequencing and public databases, and bioinformatics methods were used to screen for differentially expressed genes (DEGs). The DEGs were then subjected to biological function and pathway analyses. Some DEGs were validated using a real-time quantitative polymerase chain reaction (RT-qPCR) in peripheral blood. Spearman's correlation analysis was used to analyse the correlation between DEGs and clinical indicators. RESULTS: Based on transcriptome sequencing and public databases, the mRNA profile of bronchiectasis was determined. DEGs were obtained from the peripheral blood sequencing dataset (985 DEGs), tissue sequencing dataset (2919 DEGs), and GSE97258 dataset (1083 DEGs). Bioinformatics analysis showed that upregulated DEGs had enriched neutrophil-related pathways, and downregulated DEGs had enriched ribosome-related pathways. RT-qPCR testing confirmed the upregulated expression of VCAN, SESTD1, SLC12A1, CD177, IFI44L, SIGLEC1, and RSAD2 in bronchiectasis. These genes were related to many clinical parameters, such as neutrophils, C-reactive protein, and procalcitonin (P < 0.05). CONCLUSIONS: Transcriptomic methods were used to construct a panoramic map of bronchiectasis mRNA expression. The findings showed that neutrophil activation, chronic inflammation, immune regulation, impaired ribosomal function, oxidative phosphorylation, and energy metabolism disorders are important factors in the development of bronchiectasis. VCAN, SESTD1, SLC12A1, CD177, IFI44L, SIGLEC1, and RSAD2 may play important roles in the pathogenesis of bronchiectasis and are potential therapeutic targets.

Patient pathways for four major chronic respiratory diseases in England between 2008 and 2021.

BACKGROUND: Not all chronic diseases have clear pathways and time targets for diagnosis. We explored pathways and timings for four major chronic respiratory diseases in England. METHODS: Using deidentified electronic healthcare records from Clinical Practice Research Datalink Aurum linked to Hospital Episode Statistics, we derived cohorts of patients diagnosed with asthma, chronic obstructive pulmonary disease (COPD), ILD or bronchiectasis at three time periods (2008/2009, 2018/2019 and 2020/2021). We followed people 2 years before and 2 years after diagnosis, calculating the proportion of people who presented with symptoms, underwent diagnostic tests, were treated and consulted healthcare (primary or secondary) and calculated time intervals between events. We repeated analyses by socioeconomic status and geographical region. RESULTS: We descriptively studied patient pathways for 429 619 individuals across all time frames and diseases. Most people (>87%) had first evidence of diagnosis in primary care. The proportion of people reporting symptoms prior to diagnosis was similar for asthma, COPD and ILD (41.0%-57.9%) and higher in bronchiectasis (67.9%-71.8%). The proportion undergoing diagnostic tests was high for COPD and bronchiectasis (77.6%-89.2%) and lower for asthma (14%-32.7%) and ILD (2.6%-3.3%). The proportion of people undergoing diagnostic tests decreased in 2020/2021 for all diseases, mostly COPD. Time (months) (median (IQR)) between symptoms and diagnosis, averaged over three time periods, was lowest in asthma (~7.5 (1.3-16.0)), followed by COPD (~8.6 (1.8-17.2)), ILD (~10.1 (3.6-18.0)) and bronchiectasis (~13.5 (5.9-19.8)). Time from symptoms to diagnosis increased by ~2 months in asthma and COPD over the three time periods. Although most patients were symptomatically treated prior to diagnosis, time between diagnosis and postdiagnostic treatment was around 4 months for ILD, 3 months for bronchiectasis and instantaneous for asthma and COPD. Socioeconomic status and regional trends showed little disparity. CONCLUSION: Current pathways demonstrate missed opportunities to diagnose and manage disease and to improve disease coding.

The impact of bronchiectasis and its severity on long-term renal outcomes.

While bronchiectasis is associated with adverse cardiovascular outcomes, data regarding its impact on long-term renal outcomes is lacking.We reviewed bronchiectasis patients followed up at Queen Mary Hospital in 2017 and examined their clinical/renal outcomes in the subsequent five years. The relationships between the severity of bronchiectasis as defined by FACED (FEV1, Age, Chronic colonisation, Extension, Dyspnoea) scores and adverse renal outcomes were evaluated.A total of 315 bronchiectasis patients were included. Seventy-five patients (23.8%) showed renal progression. Baseline FACED score showed a positive correlation with renal progression over 5 years of follow-up (adjusted odds ratio [aOR] 1.30 (95% CI 1.083-1.559, P = 0.005). Patients with moderate-to-severe bronchiectasis (FACED score ≥3) showed an increased risk of renal progression (aOR 1.833, 95% CI 1.082-3.106; P = 0.024) and more rapid decline in estimated glomerular filtration rate than those with mild disease (-4.77 ± 4.19 mL/min/1.73 m²/year vs. -3.49 ± 3.94 mL/min/1.73 m²/year; P = 0.006). Patients who developed renal progression had a higher risk of death (adjusted hazard ratio [aHR] 3.056, 95% CI 1.505-6.206; P = 0.002) and subsequent rates of hospitalisation (1.56 ± 2.81 episodes/year vs. 0.60 ± 1.18 episodes/year; P < 0.001) compared to those without renal progression.Progressive renal function deterioration is prevalent among bronchiectasis patients, and the severity of bronchiectasis is a robust predictor of renal progression..

Severe bronchiectasis is associated with increased carotid intima-media thickness.

BACKGROUND: Although bronchiectasis has been shown to be associated with cardiovascular disease, there is limited evidence of an association with subclinical atherosclerosis, especially carotid intima-media thickness (CIMT). METHODS: This prospective study compared CIMT among patients with and without bronchiectasis, and among bronchiectatic patients classified according to disease severity using the FACED score. The study was carried out at a major regional hospital and tertiary respiratory referral centre in Hong Kong. RESULTS: Total 155 Chinese patients with non-cystic fibrosis (CF) bronchiectasis and 512 controls were recruited. The mean CIMT was 0.58 ± 0.10 mm, 0.63 ± 0.11 mm and 0.66 ± 0.08 mm respectively among controls, patients with mild-to-moderate bronchiectasis and patients with severe bronchiectasis. There was no statistically significant difference in CIMT between patients with mild-to-moderate bronchiectasis and controls. Multivariate linear regression revealed that CIMT was significantly increased in patients with severe bronchiectasis relative to controls. The same phenomenon was observed among patients without a history of cardiovascular disease or cardiovascular risk factors. CONCLUSIONS: CIMT was significantly increased in patients with severe bronchiectasis compared with controls without bronchiectasis, but not among patients with mild-to-moderate bronchiectasis, which suggested the subclinical atherosclerosis to be more prevalent among patients with severe bronchiectasis.