Pulmonology最新文献

{"title":"Adulthood interstitial lung disease due to variants in surfactant related genes: A systematic review.","authors":"Claudio Tirelli, Stefano Sanduzzi Zamparelli, Camille Louvrier, Sabrina Mira, Marta Italia, Paola Rebecca Iovine, Fausta Alfano, Stefano Centanni, Michele Mondoni, Nadia Nathan, Marialuisa Bocchino","doi":"10.1080/25310429.2026.2736380","DOIUrl":"10.1080/25310429.2026.2736380","url":null,"abstract":"<p><p><b>Background:</b> Surfactant-related gene (SRG) variants are an emerging cause of interstitial lung disease/pulmonary fibrosis (ILD/PF). Accurate recognition of these disorders is relevant for diagnosis, genetic counselling, and clinical management.<b>Reaserch question:</b> How do SRG-associated ILD/PF present in adults, and what clinical, radiological, and histopathological features characterize their phenotype?<b>Study design and methods:</b> We performed a systematic review (PROSPERO CRD42024517610) to describe the clinical features, management, and outcomes of adults with SRG-related ILD/PF. PubMed, Embase, and Web of Science were searched from January 2000 to December 2024 for studies reporting adults (≥18 years) with genetically confirmed SRG variants and ILD/PF. Demographic, clinical, radiological, histological, functional, treatment, and lung transplantation (LT) data were extracted.<b>Results:</b> Thirty-eight studies included 162 adults carrying class 3-5 variants in <i>SFTPA1/</i>2 (61), <i>SFTPC</i> (52), <i>ABCA3</i> (31), <i>NKX2-1</i> (16), and <i>SFTPB</i> (2), including 35 novel variants. Age at diagnosis ranged from 19 to 79 years. Familial ILD predominated in SFTPA1/2, frequently associated with personal or familial lung cancer, whereas <i>NKX2-1</i> variants showed thyroid and neurological involvement. Lung function typically demonstrated mild-to-moderate restriction with impaired diffusing capacity. Chest CT findings were heterogeneous and often indeterminate, while usual interstitial pneumonia was the predominant histological pattern except in <i>ABCA3</i>-related disease. Treatment approaches varied considerably; LT provided favourable outcomes in selected patients.<b>Conclusion:</b> Adult SRG-related ILD/PF is likely under-recognised because of variable penetrance, wide age at onset, and non-specific imaging features. International registries are needed to improve genotype-phenotype correlations and guide personalised management.</p>","PeriodicalId":54237,"journal":{"name":"Pulmonology","volume":"32 1","pages":"2736380"},"PeriodicalIF":5.3,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149041854","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Mobile cone-beam computed tomography combined with electromagnetic navigation bronchoscopy for the diagnosis of peripheral pulmonary nodules.","authors":"Lin Ye, Siyuan Hong, Xiaoxuan Zheng, Junxiang Chen, Fangfang Xie, Shuaiyang Liu, Houteng Xi, Jiajia Ge, Xiaoshu Cai, Jiayuan Sun","doi":"10.1080/25310429.2026.2730871","DOIUrl":"10.1080/25310429.2026.2730871","url":null,"abstract":"<p><strong>Background: </strong>Electromagnetic navigation bronchoscopy (ENB) is widely used for diagnosing peripheral pulmonary nodules (PPNs), but its diagnostic yield remains variable. Mobile cone-beam computed tomography (mCBCT) enables intraprocedural assessment of the tool-lesion relationship and navigation correction; however, prospective data on its incremental clinical value remain limited.</p><p><strong>Research question: </strong>Clinical value of mCBCT-guided navigation correction on the tool-lesion relationship and diagnostic yield during ENB for PPNs.</p><p><strong>Study design and methods: </strong>This prospective, single-centre, single-arm trial evaluated the diagnostic performance of mCBCT-assisted ENB for PPNs and the incremental clinical value of mCBCT-guided navigation correction. Intraoperative mCBCT assessed the tool-lesion relationship and guided correction before sampling. The primary endpoint was overall diagnostic yield after mCBCT assessment and adjustment. The key secondary endpoint was the tool-in-lesion improvement rate.</p><p><strong>Results: </strong>A total of 108 patients were enrolled. Median nodule size was 21.0 (IQR, 17.0-25.0) mm, and a CT 'bronchus sign' was present in 77.8%. Nodules were peripheral (47.2%), intermediate (33.3%), or central (19.4%) and solid (83.3%), mixed ground-glass opacities (13.9%), or pure ground-glass opacities (2.8%). The overall diagnostic yield was 79.6% (86/108). Navigation correction was required in 42.6% (46/108). Following mCBCT-guided navigation correction, the tool-in-lesion rate increased from 54.6% (59/108) to 87.0% (94/108), an absolute improvement of 32.4%. Procedure-related atelectasis was identified by mCBCT in 8 (7.4%) patients. No serious adverse events occurred.</p><p><strong>Conclusion: </strong>mCBCT-guided navigation correction improved the tool-lesion relationship and provided incremental clinical value beyond conventional ENB, particularly for smaller lesions and those without a CT bronchus sign, while maintaining an acceptable safety profile.</p>","PeriodicalId":54237,"journal":{"name":"Pulmonology","volume":"32 1","pages":"2730871"},"PeriodicalIF":5.3,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148921849","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Non-eligibility for highly effective modulator therapy with elexacaftor/tezacaftor/ivacaftor in cystic fibrosis: What about Portugal?","authors":"Elsa Fragoso, Pilar Azevedo, Luísa Pereira, Telma Barbosa, Ana Lúcia Cardoso, Célia Maia Cruz, Adelina Amorim, Inês Azevedo, Leonor Almeida, Susana Castanhinha, Ana Sofia Santos, Fernanda Gamboa, Teresa Reis Silva, Cristina Freitas, Vitor Teixeira, Alexandra Carreiro, Juan Gonçalves","doi":"10.1080/25310429.2026.2742057","DOIUrl":"10.1080/25310429.2026.2742057","url":null,"abstract":"<p><strong>Background: </strong>Cystic fibrosis (CF) is an autosomal recessive disease caused by mutations in cystic fibrosis transmembrane conductance regulator (CFTR) gene. CFTR modulators are drugs developed to target the defective protein. All over the world, there are patients considered non-eligible for this therapy according to local regulatory policies.</p><p><strong>Methods: </strong>To characterise the Portuguese CF patients defined as non-eligible according to genotype for highly effective modulator therapy (HEMT) with elexacaftor/tezacaftor/ivacaftor (ETI), the authors conducted a multicentre observational study based on a retrospective analysis of the Portuguese CF population.</p><p><strong>Results: </strong>Based on the current prescribing policy in Portugal, the prevalence of non-eligible patients in Portugal in 2023 was 18.7%, similar to that reported in Europe. However, the regulatory acceleration in the CFTR modulator landscape in 2025 will most likely decrease the expected prevalence to 1.2% under national regulatory adoption of EMA criteria. R334W, G85E, N1303K, G542X, A561E, R1066C and P205S are the most prevalent variants. Most patients are young adults with established pulmonary disease. CF therapies impose a considerable burden on this population.</p><p><strong>Conclusion: </strong>This study provides a deeper understanding of the Portuguese CF population currently ineligible for ETI and enables the projection of the potential impact that forthcoming changes in HEMT eligibility criteria may have across Portugal. With the anticipated expansion of these criteria, the number of patients remaining ineligible for HEMT is expected to become residual. Furthermore, the present work offers a characterisation of patients likely to remain ineligible for HEMT in the future, who could be suitable candidates for clinical trials exploring alternative therapeutic approaches.</p>","PeriodicalId":54237,"journal":{"name":"Pulmonology","volume":"32 1","pages":"2742057"},"PeriodicalIF":5.3,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149225381","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Dyspnoea-referenced minimal clinically important difference in the resting inspiratory capacity response to short-acting bronchodilators in COPD patients.","authors":"Pierantonio Laveneziana, David Hajage, Nicolas Verger, Clara Bianquis, François-Xavier Blanc, Christian Straus, Thomas Similowski","doi":"10.1080/25310429.2025.2611184","DOIUrl":"10.1080/25310429.2025.2611184","url":null,"abstract":"<p><strong>Background: </strong>In COPD, the clinical significance of bronchodilator-induced changes in inspiratory capacity (IC) at rest remains uncertain. Unlike forced expiratory volume in one second (FEV₁), IC may better reflect symptom improvement, but no minimal clinically important difference (MCID) has been formally established.</p><p><strong>Research question: </strong>To relate short-acting bronchodilator (SABD)-induced dyspnoea relief in stable COPD patients at rest with the concomitant IC changes and determine the MCID of the latter.</p><p><strong>Study design and methods: </strong>In 106 stable COPD outpatients undergoing routine lung function testing, spirometry and plethysmography were performed before and after SABD administration. Dyspnoea was assessed using a visual analogue scale (VAS) and a five-point Likert scale. Three approaches were used to estimate MCID, namely two anchored methods (receiver operating characteristics (ROC)-based and difference-based) and a distribution-based method.</p><p><strong>Results: </strong>Dyspnoea improvement was reported in 64% of patients. IC changes correlated strongly with symptom relief, while FEV₁ changes did not. ROC analysis identified an optimal IC threshold of 0.125 L (5% of baseline) with excellent predictive accuracy. The distribution-based estimate was 0.187 L, and the lowest difference-based estimate was 0.27 L. Averaging the ROC and distribution thresholds yielded a proposed MCID of 0.150 L.</p><p><strong>Conclusion: </strong>IC changes after SABD administration at rest are more closely associated with dyspnoea relief than FEV₁ changes. An IC increase of 0.150 L may serve as a practical benchmark in future large-scale studies aiming at evaluation of the practical utility of IC changes in clinical practice, e.g. to inform therapeutic strategies, such as guiding the use of rescue SABD.</p>","PeriodicalId":54237,"journal":{"name":"Pulmonology","volume":"32 1","pages":"2611184"},"PeriodicalIF":5.3,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145960832","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Dual-correction robotic bronchoscopy system with ultra-thin catheter for diagnosing challenging peripheral pulmonary nodules: A multi-centre prospective trial.","authors":"Zhending You, Junfeng Huang, Feng Wang, Li Xu, Jing Liu, Hongjia Li, Jiaxin Tang, Wan Li, Zhaohui Li, Yuhua Liu, Yanfei Bai, Shiyue Li, Changhao Zhong","doi":"10.1080/25310429.2026.2621546","DOIUrl":"10.1080/25310429.2026.2621546","url":null,"abstract":"<p><strong>Background: </strong>Conventional bronchoscopy has imited diagnostic yield for challenging peripheral pulmonary nodules(C-PPNs), particularly for small (≤20 mm), pleural-contact, or reverse-angle (bifurcation angle ≤90°) nodules. This prospective multi-centre study evaluates the novel robotic bronchoscopy system (RBS) with ultra-thin catheters in C-PPNs under radiation-free conditions.</p><p><strong>Methods: </strong>This multi-centre, prospective study enrolled 89 patients with C-PPN (characterised by a diameter ≤20 mm, pleural-contact, or an angle ≤90°) from three centres, who underwent RBS biopsy between 2022 and 2024. Diagnostic yield were conducted based on specific nodule characteristics. Univariate and multivariate logistic regressions were performed to evaluate the association between nodular-related factors and diagnostic yield.</p><p><strong>Results: </strong>The average nodule size was 19.54 mm (53.9%, ≤20 mm). Pleural-contact was observed in 71.9% of nodules, and 64.0% exhibited a reverse-angle (≤90°). The novel system achieved 100% navigation and sampling success. Importantly, the diagnostic yield reached 84.2%, with 88.3% sensitivity for malignancy. Diagnostic performance were comparable across Single-criteria, Dual-criteria, and Triple-criteria groups (<i>p</i> = 0.416). Nodules ≤20 mm had lower yield (75.0%) compared to pleura-contact (90.6%, <i>p</i> = 0.013) or reverse-angle (89.5%, <i>p</i> = 0.028). Logistic regression confirmed nodule size >20 mm as a significant diagnostic yield predictor. Notably, no pneumothorax occurred, and only two patients experienced minor bleeding.</p><p><strong>Conclusion: </strong>This prospective multi-centre study introduced the concept of C-PPN, establishing a valuable reference for subsequent research. Moreover, the novel system featuring ultra-thin catheters demonstrated superior performance, achieving 100% navigation success, 84.2% diagnostic yield and 0 pneumothorax, in such nodules without radiation guidance.</p>","PeriodicalId":54237,"journal":{"name":"Pulmonology","volume":"32 1","pages":"2621546"},"PeriodicalIF":5.3,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146115109","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Building competency in navigation bronchoscopy: Development and evaluation of a structured training programme.","authors":"Ferhat Beyaz, Julie E M Swillens, Renée M Geraats, Roel L J Verhoeven, Rosella P M G Hermens, Erik H F M van der Heijden","doi":"10.1080/25310429.2026.2722773","DOIUrl":"https://doi.org/10.1080/25310429.2026.2722773","url":null,"abstract":"<p><strong>Background: </strong>Cone-beam CT-guided navigation bronchoscopy (CBCT-NB) is a minimally invasive technique for diagnosing pulmonary nodules combining endoscopic navigation with real-time 3D imaging. The procedure requires advanced bronchoscopic skills and interpretation of multiple imaging modalities. Despite evidence that simulation-based education enhances procedural safety and performance, no standardised training programmes for CBCT-NB have yet been established. This study describes the development and evaluation of a structured training programme for pulmonologists new to CBCT-NB.</p><p><strong>Methods: </strong>A multidisciplinary expert panel developed a stepwise CBCT-NB training programme comprising online theoretical e-learning, live case observation at an expert centre, a structured one-day hands-on simulation session, and on-site supervision during the initial clinical procedures. Pulmonologists from Dutch hospitals preparing to implement CBCT-NB were enrolled. Participants' expectations, perceived learning needs, and post-training experiences were explored through semi-structured interviews and analysed using thematic analysis.</p><p><strong>Results: </strong>Nine pulmonologists from five hospitals participated. Participants expressed a strong need for structured, practical instructions, particularly regarding procedural workflow, multimodal image interpretation, equipment handling, and use of augmented fluoroscopy software. Following completion of the programme, all reported increased self-confidence and improved perceived competency. Hands-on simulation was consistently identified as a key component for developing technical and cognitive skills and was considered essential preparation before independent clinical practice.</p><p><strong>Conclusion: </strong>The structured multimodal CBCT-NB training programme facilitates confidence and addresses learner-identified competency needs among novice navigation bronchoscopists. The findings offer practical guidance for the structured implementation and training of novel medical technological interventions. Future research should evaluate long-term skill retention, objective competency outcomes, and clinical effectiveness.</p>","PeriodicalId":54237,"journal":{"name":"Pulmonology","volume":"32 1","pages":"2722773"},"PeriodicalIF":5.3,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148867946","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Letter to the editor: Urinary incontinence is common among people attending pulmonary rehabilitation, yet pulmonary rehabilitation has a small effect on urinary symptoms: A multicenter prospective cohort study.","authors":"Trishala Rangare, Shweta Sharma","doi":"10.1080/25310429.2026.2671501","DOIUrl":"10.1080/25310429.2026.2671501","url":null,"abstract":"","PeriodicalId":54237,"journal":{"name":"Pulmonology","volume":"32 1","pages":"2671501"},"PeriodicalIF":5.3,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147846009","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Letter to editor 'dyspnoea-referenced minimal clinically important difference in the resting inspiratory capacity response to short-acting bronchodilators in COPD patients'.","authors":"Shivi Bajpai, Mousumi Saha, Subhasish Chatterjee, Shanvi Priya","doi":"10.1080/25310429.2026.2630534","DOIUrl":"10.1080/25310429.2026.2630534","url":null,"abstract":"","PeriodicalId":54237,"journal":{"name":"Pulmonology","volume":"32 1","pages":"2630534"},"PeriodicalIF":5.3,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146208545","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A pilot study to assess the role of 12-week roflumilast treatment in non-CF bronchiectasis.","authors":"Wang Chun Kwok, Kelvin Kai Wang To, Frankie Chi Fat Ko, Herbert Pang, Isaac Sze Him Leung, Wai On Tam, Chi Hung Chau, Anthony Shing Yiu Yu, Hon Cheung Fan, Charles Wong, Flora Pui Ling Miu, James Chung Man Ho","doi":"10.1080/25310429.2026.2742051","DOIUrl":"https://doi.org/10.1080/25310429.2026.2742051","url":null,"abstract":"<p><strong>Background: </strong>Roflumilast demonstrated anti-inflammatory activity in COPD. While bronchiectasis is also characterised by neutrophilic inflammation, there is a lack of evidence on the use of roflumilast in bronchiectasis.</p><p><strong>Methods: </strong>An open‑label observation study with an exploratory nature was conducted in Hong Kong. The primary outcome measure was 24-hour sputum volume. The extent of airway inflammation was indicated by sputum leucocyte density, pro-inflammatory cytokines and neutrophil elastase. All eligible patients were treated with roflumilast, starting at 250 µg daily with possible escalation to 500 µg daily if tolerated.</p><p><strong>Results: </strong>A total of 39 patients were started on roflumilast with 24 (61.5%) completed 12 weeks of treatment and 9 of them escalated to 500 µg daily. There were no significant changes in mean daily sputum volume. The median sputum leucocyte density and TNF-α significantly decreased from the recruitment visit to 12 weeks. In extrapolatory analyses, patients with a baseline daily sputum volume ≥ 20 mL might be more likely to respond with a reduction in sputum volume.</p><p><strong>Conclusion: </strong>Roflumilast treatment in non-CF bronchiectasis could provide anti-inflammatory effects with a significant reduction in sputum leucocyte density and TNF-α level over 12 weeks of treatment. Extrapolatory analyses suggested potential benefits in sputum volume reduction among the subgroup with baseline daily sputum volume ≥ 20 mL.</p>","PeriodicalId":54237,"journal":{"name":"Pulmonology","volume":"32 1","pages":"2742051"},"PeriodicalIF":5.3,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149118378","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Correspondence: 'Interpreting exercise's immunomodulatory effects in chronic obstructive pulmonary disease requires methodological scrutiny'.","authors":"Hongjuan Jing, Chao Yuan","doi":"10.1080/25310429.2026.2613997","DOIUrl":"10.1080/25310429.2026.2613997","url":null,"abstract":"","PeriodicalId":54237,"journal":{"name":"Pulmonology","volume":"32 1","pages":"2613997"},"PeriodicalIF":5.3,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145960843","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}