{"title":"Safety of Pulmonary Rehabilitation in Acute Exacerbations of Interstitial Lung Disease: A Multicentre Study.","authors":"Mitsutoshi Akiho, Yuta Takahashi, Takuya Miyahara, Shuken Kobayashi, Daisuke Minamishima, Yasunari Sakai, Yoshifumi Abe, Takaaki Watanabe, Masahiko Kimura, Naoto Matsumoto","doi":"10.1002/resp.70291","DOIUrl":"https://doi.org/10.1002/resp.70291","url":null,"abstract":"<p><strong>Background and objective: </strong>The safety of pulmonary rehabilitation (PR) during acute exacerbations of interstitial lung disease (AE-ILD) remains uncertain. Utilising a real-world, multicentre clinical context, this study investigated the incidence of PR associated adverse events in hospitalised patients with AE-ILD.</p><p><strong>Methods: </strong>This multicentre, prospective observational study was conducted over 82 months at five hospitals in Japan. A total of 295 patients with AE-ILD who received PR during hospitalisation were enrolled. Seven types of adverse events were predefined, and their incidence during daily PR sessions was recorded. Patient characteristics, PR content, and clinical outcomes were analysed.</p><p><strong>Results: </strong>A total of 3986 PR sessions were performed. Twenty-four adverse events (0.6%) were reported, the most common of which was transient hypoxaemia (SpO<sub>2</sub> < 85% for > 3 min; 79.2%), followed by hypotension (12.5%) and arrhythmia (8.3%). None of the adverse events required additional treatment or prolonged hospitalisation. Patients who experienced adverse events had significantly lower baseline functional status and underwent more PR sessions than those without adverse events.</p><p><strong>Conclusion: </strong>PR during AE-ILD was found to be safe when delivered by trained physiotherapists following appropriate risk management procedures. Study findings provide novel evidence supporting the implementation of PR as part of comprehensive care, including during AE-ILD.</p>","PeriodicalId":21129,"journal":{"name":"Respirology","volume":" ","pages":""},"PeriodicalIF":7.2,"publicationDate":"2026-07-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148607030","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":"Clinical Outcomes and Acute Exacerbation Incidence in Combined Pulmonary Fibrosis and Emphysema.","authors":"Kazuya Tsubouchi, Yasuaki Kotetsu, Tomotsugu Takano, Hiroaki Ogata, Fumiaki Kiyomi, Katsuyuki Ichiki, Shohei Takata, Yasuhiko Kitasato, Hiroshi Ishii, Kazuhiro Yatera, Kei Yamasaki, Masaki Okamoto, Makoto Yoshida, Masayuki Kawasaki, Masaki Fujita, Tomohiro Ogawa, Kazunori Tobino, Eiji Harada, Noriaki Nakagaki, Masashi Komori, Taishi Harada, Hiroshi Wataya, Yoichi Nakanishi, Isamu Okamoto","doi":"10.1002/resp.70285","DOIUrl":"10.1002/resp.70285","url":null,"abstract":"<p><strong>Background and objective: </strong>Combined pulmonary fibrosis and emphysema (CPFE) is characterized by coexisting upper-zone emphysema and lower-zone fibrosis, but standardized diagnostic criteria and clinical outcomes remain poorly defined. This study aimed to evaluate survival, acute exacerbation incidence, and disease progression in CPFE patients using international classification criteria with visual assessment of emphysema extent.</p><p><strong>Methods: </strong>This multicentre prospective cohort study at 29 Japanese hospitals enrolled 1016 patients with idiopathic interstitial pneumonias (IIPs) or chronic obstructive pulmonary disease (COPD) between 2013 and 2016. CPFE was defined as ≥ 5% emphysema determined by visual assessment of CT scans for IIP patients, according to international classification criteria. Patients were followed for 5 years.</p><p><strong>Results: </strong>Among 528 IIP patients, 92 (17.4%) had CPFE. CPFE patients showed a significantly worse 5-year survival compared with non-CPFE IIP patients (51.4% vs. 63.1%, p = 0.026) and COPD patients (51.4% vs. 84.0%, p < 0.001). After adjustment for covariates including IIP subtype, CPFE remained independently associated with increased mortality among IIP patients (HR of 1.85 [95% CI, 1.27-2.69], p < 0.001). CPFE patients also had a significantly higher acute exacerbation rate than did non-CPFE IIP patients (p < 0.001). The prognostic impact of CPFE was most pronounced in patients with idiopathic pulmonary fibrosis (IPF), with CPFE/IPF patients showing the worst outcomes.</p><p><strong>Conclusion: </strong>Visual assessment with a ≥ 5% emphysema threshold effectively identified CPFE patients with a distinct clinical phenotype characterized by poor survival and an increased acute exacerbation incidence. These findings validate international classification criteria and emphasize the need for enhanced surveillance and tailored management strategies for this high-risk population.</p>","PeriodicalId":21129,"journal":{"name":"Respirology","volume":" ","pages":""},"PeriodicalIF":7.2,"publicationDate":"2026-07-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148405749","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}
RespirologyPub Date : 2026-07-01Epub Date: 2026-04-01DOI: 10.1002/resp.70246
Caitlin C Fermoyle, John A Mackintosh, Vidya Navaratnam, Samantha J Ellis, Wendy A Cooper, Nicole S L Goh, Yuben Moodley, Paul N Reynolds, Christopher J Zappala, Peter Hopkins, Ian N Glaspole, Tamera J Corte, Simon L F Walsh
{"title":"Deep-Learning Algorithm Diagnostic Support for Usual Interstitial Pneumonia Pattern Recognition in Fibrotic Interstitial Lung Disease.","authors":"Caitlin C Fermoyle, John A Mackintosh, Vidya Navaratnam, Samantha J Ellis, Wendy A Cooper, Nicole S L Goh, Yuben Moodley, Paul N Reynolds, Christopher J Zappala, Peter Hopkins, Ian N Glaspole, Tamera J Corte, Simon L F Walsh","doi":"10.1002/resp.70246","DOIUrl":"10.1002/resp.70246","url":null,"abstract":"<p><strong>Background and objective: </strong>High resolution computed tomography (HRCT) scan diagnostic classification for usual interstitial pneumonia (UIP) plays a critical role in therapeutic decision-making and clinical trial eligibility for interstitial lung disease (ILD) patients, but is subject to variability. A deep learning algorithm, the Systematic Objective Fibrotic Imaging Analysis Algorithm (SOFIA), has been validated to assist classification of HRCTs based on current guidelines. In this study, we evaluate the impact of SOFIA on inter-observer agreement for UIP classification and prognostic accuracy of clinicians' assessment of ILD HRCTs.</p><p><strong>Methods: </strong>Radiologists and pulmonologists (reviewers) were invited to evaluate 203 HRCTs from a national fibrotic ILD registry, scoring each of four UIP categories (definite UIP, probable UIP, indeterminate, or alternative diagnosis). SOFIA outputs were then provided, and reviewers were able to reevaluate their scores. Changes in interobserver agreement for UIP classification and prognostic accuracy were calculated.</p><p><strong>Results: </strong>Three hundred twelve reviewers (120 radiologists, 192 pulmonologists) from 49 countries evaluated 203 HRCT scans. Following SOFIA, inter-observer diagnostic agreement improved for definite UIP from moderate to good (ICC<sub>pre</sub> = 0.54[0.50-0.60]; ICC<sub>post</sub> = 0.70[0.66-0.74]), and for probable UIP from fair to moderate (ICC<sub>pre</sub> = 0.30[0.27-0.35]; ICC<sub>post</sub> = 0.53[0.49-0.58]). Following SOFIA, there was improved prognostic accuracy for reviewers' definite UIP, probable UIP, and indeterminate scores (significant change in c-index), and the proportion of reviewers whose probable UIP scores were significantly predictive of transplant-free survival increased by 42%.</p><p><strong>Conclusion: </strong>Providing SOFIA algorithm output to clinicians reviewing HRCT scans improved diagnostic agreement and prognostic accuracy for fibrotic ILD. SOFIA may be a useful automated assistive tool to support improved diagnostic consistency.</p>","PeriodicalId":21129,"journal":{"name":"Respirology","volume":" ","pages":"711-720"},"PeriodicalIF":7.2,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13342441/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147594149","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
RespirologyPub Date : 2026-07-01Epub Date: 2026-04-15DOI: 10.1002/resp.70248
Can Xia, Wanlong Wu, Saisai Lu, Yan Ye, Jingjing Li, Runci Wang, Wenwen Xu, Zhiwei Chen, Yakai Fu, Xia Lyu, Kaiwen Wang, Wei Fan, Jia Li, Bingpeng Guo, Qun Luo, Qian Han, Yu Xue, Weiguo Wan, Huaxiang Wu, Wenjia Sun, Li Sun, Xiaodong Wang, Shuang Ye, Dan Chen, Qiong Fu
{"title":"Upadacitinib Versus Tofacitinib in Anti-MDA5-Positive Dermatomyositis With Interstitial Lung Disease: A Multi-Centre Cohort Study Emulating a Target Trial.","authors":"Can Xia, Wanlong Wu, Saisai Lu, Yan Ye, Jingjing Li, Runci Wang, Wenwen Xu, Zhiwei Chen, Yakai Fu, Xia Lyu, Kaiwen Wang, Wei Fan, Jia Li, Bingpeng Guo, Qun Luo, Qian Han, Yu Xue, Weiguo Wan, Huaxiang Wu, Wenjia Sun, Li Sun, Xiaodong Wang, Shuang Ye, Dan Chen, Qiong Fu","doi":"10.1002/resp.70248","DOIUrl":"10.1002/resp.70248","url":null,"abstract":"<p><strong>Background and objective: </strong>The treatment of anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis with interstitial lung disease (MDA5<sup>+</sup>DM-ILD) remains a significant clinical challenge. To compare the effectiveness and safety of upadacitinib (UPA) versus tofacitinib (TOFA) upon 6-month lung transplantation-free survival in newly diagnosed MDA5<sup>+</sup>DM-ILD patients.</p><p><strong>Methods: </strong>This multi-centre cohort study in China enrolled MDA5<sup>+</sup>DM-ILD patients treated from January 2020 to February 2025. Prevalent/incident new users and non-inferior design along with inverse probability treatment weighting (IPTW) were implemented to emulate randomized controlled trial.</p><p><strong>Results: </strong>In the eligible cohort, a total of 106 patients were treated with UPA and 328 with TOFA. The crude 6-month lung transplantation-free survival rates were 71.7% (76/106) in the UPA group and 67.4% (221/328) in the TOFA group. In the IPTW-adjusted cohort, UPA showed a trend toward higher survival than TOFA (p = 0.067), with a weighted risk difference of 10.3% (95% confidence interval -0.4% to 20.2%) meeting the non-inferiority margin (non-inferiority p = 0.003). Sensitivity and subgroup analyses showed consistent results, with potentially greater benefit of UPA in incident JAK inhibitor users (as first-line treatment), monotherapy recipients (without combination with other immunosuppressants except steroid), and those with rapidly progressive ILD. Safety profiles were comparable between groups.</p><p><strong>Conclusion: </strong>UPA was found to be non-inferior to TOFA in improving 6-month lung transplantation-free survival among patients with MDA5<sup>+</sup>DM-ILD, with comparable safety.</p>","PeriodicalId":21129,"journal":{"name":"Respirology","volume":" ","pages":"721-731"},"PeriodicalIF":7.2,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147691647","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}
RespirologyPub Date : 2026-07-01Epub Date: 2026-05-24DOI: 10.1002/resp.70263
Thomas J Altree, Robert J Adams
{"title":"New Obstructive Sleep Apnoea Multi-Night Diagnostic Devices. Is a Single Night of Measurement Now Considered Enough?","authors":"Thomas J Altree, Robert J Adams","doi":"10.1002/resp.70263","DOIUrl":"10.1002/resp.70263","url":null,"abstract":"","PeriodicalId":21129,"journal":{"name":"Respirology","volume":" ","pages":"669-671"},"PeriodicalIF":7.2,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13342434/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148017011","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
RespirologyPub Date : 2026-07-01Epub Date: 2026-06-10DOI: 10.1002/resp.70252
Angela O Suen, Lauren R Pollack, C Adrian Austin, Leah J Witt
{"title":"An Age-Friendly Approach to Caring for Chronic Lung Disease in the Ambulatory Setting.","authors":"Angela O Suen, Lauren R Pollack, C Adrian Austin, Leah J Witt","doi":"10.1002/resp.70252","DOIUrl":"10.1002/resp.70252","url":null,"abstract":"<p><p>Many common chronic lung diseases, such as chronic obstructive pulmonary disease (COPD) and interstitial lung disease (ILD), have much greater prevalence among older adults and as a result, pose a substantial burden of morbidity, disability, and mortality. The care of older adults with chronic lung disease necessitates an age-friendly approach due to marked differences in physiology, clinical presentation, disease trajectories, and healthcare priorities as compared to younger adults. Pulmonary care models have historically prioritised disease-focused approaches over person-centered care. Disease-focused care fails to adequately address geriatric syndromes that complicate the management of chronic disease, such as multicomplexity, functional impairment, cognitive dysfunction, and psychosocial complexity. In this narrative review, we propose a deliberate, geriatrics-informed approach to ambulatory pulmonary care using the Age-Friendly 5Ms framework-What Matters, Medications, Mentation, Mobility, and Multicomplexity. This framework is an easy-to-use reference for chronic disease management and offers a practical roadmap to support clinical decision-making, inform health system improvement, and guide future research focused on outcomes that matter most to older adults living with chronic lung disease. We review how the 5 M framework helps to align care with patients' goals and values, minimize medication-related harms, address cognitive, psychological, and caregiving needs, preserve mobility and functional independence, and manage the intersecting medical and social challenges common in later life.</p>","PeriodicalId":21129,"journal":{"name":"Respirology","volume":" ","pages":"676-692"},"PeriodicalIF":7.2,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13342435/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148219555","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
RespirologyPub Date : 2026-07-01Epub Date: 2026-06-10DOI: 10.1002/resp.70272
Mark Lavercombe
{"title":"Professional Development for the Clinician-Educator in Respiratory and Sleep Medicine.","authors":"Mark Lavercombe","doi":"10.1002/resp.70272","DOIUrl":"10.1002/resp.70272","url":null,"abstract":"","PeriodicalId":21129,"journal":{"name":"Respirology","volume":" ","pages":"672-675"},"PeriodicalIF":7.2,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13342436/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148212377","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
RespirologyPub Date : 2026-07-01Epub Date: 2026-03-27DOI: 10.1002/resp.70242
Charles Wong, Yiu Wing Lam, Vinson Nelson Yew, Jeffrey Chi Chung Wong, Hei Shun Cheng, Pui Hing Chiu, Chun Wai Tong, Flora Pui Ling Miu
{"title":"Real-World Evaluation of a Rapid Multiplex PCR Panel in Adults Hospitalized With COPD Exacerbations.","authors":"Charles Wong, Yiu Wing Lam, Vinson Nelson Yew, Jeffrey Chi Chung Wong, Hei Shun Cheng, Pui Hing Chiu, Chun Wai Tong, Flora Pui Ling Miu","doi":"10.1002/resp.70242","DOIUrl":"10.1002/resp.70242","url":null,"abstract":"<p><strong>Background and objective: </strong>The clinical utility of rapid multiplex PCR panels in patients with exacerbation of COPD (ECOPD) is undefined. We aimed to assess the clinical relevance of the BioFire FilmArray pneumonia plus panel (FAPP) testing in ECOPD in a real-world hospital setting.</p><p><strong>Methods: </strong>A prospective observational study was conducted on 74 hospitalized ECOPD patients. Spontaneous sputum samples were analysed using FAPP alongside standard-of-care (SOC) tests. Microbial profiles were assessed, and patients were phenotyped based on FAPP findings.</p><p><strong>Results: </strong>FAPP significantly increased the proportion of patients with identifiable pathogens from 36% by SOC tests to 78% (increase of 42%, p < 0.0001), primarily contributed by increased bacterial detection (increase of 47%, p < 0.0001), with a shorter turnaround time (median 6 h vs. 75 h, p < 0.0001). Staphylococcus aureus was the most prevalent bacteria, while Haemophilus influenzae predominated at high abundance (10<sup>6</sup>-≥ 10<sup>7</sup> copies/ml). Respiratory viruses were detected in 27%, most frequently Influenza A. All in-panel cultured bacteria were concordantly detected by FAPP at high abundance. High-abundance bacterial detection was associated with significantly higher host immune response markers. The bacterial-predominant phenotype was associated with higher exacerbation severity, while the purely eosinophilic phenotype had a lower 180-day treatment failure rate compared to the unclassified phenotype.</p><p><strong>Conclusion: </strong>FAPP significantly enhances microbial detection in patients with ECOPD. Appropriate utilization of FAPP might guide antibiotic decision-making and phenotyping in ECOPD. The potential of sputum multiplex PCR-based strategy to guide antibiotic decisions in ECOPD merits further research.</p>","PeriodicalId":21129,"journal":{"name":"Respirology","volume":" ","pages":"702-710"},"PeriodicalIF":7.2,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147521787","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}
RespirologyPub Date : 2026-07-01Epub Date: 2026-03-18DOI: 10.1002/resp.70241
Lixia Wang, Xinjie Hui, Rong Huang, Yi Xiao
{"title":"Differences in Endotypic Traits Between Obstructive Sleep Apnoea Patients With and Without Excessive Daytime Sleepiness.","authors":"Lixia Wang, Xinjie Hui, Rong Huang, Yi Xiao","doi":"10.1002/resp.70241","DOIUrl":"10.1002/resp.70241","url":null,"abstract":"<p><strong>Background and objective: </strong>Obstructive sleep apnoea (OSA) is a heterogeneous disorder. Although excessive daytime sleepiness (EDS) is a hallmark symptom of OSA, many patients remain asymptomatic, and a subset continues to experience EDS despite optimal therapy. The endotypic traits underlying EDS in OSA remain poorly defined.</p><p><strong>Methods: </strong>We conducted a cross-sectional study of 694 adults at a Beijing sleep center, including 542 participants with AHI ≥ 5 events/h. EDS was defined as an Epworth Sleepiness Scale score > 10. Four endotypic traits were derived from full-night polysomnography using the \"Phenotyping Using Polysomnography\" method. Associations with EDS were assessed by multivariable logistic regression, and relationships with OSA severity by generalized linear regression.</p><p><strong>Results: </strong>Compared with OSA without EDS, OSA with EDS exhibited significantly higher loop gain (LG), arousal threshold (ArTH), and upper airway collapsibility, and lower upper airway muscle compensation (Vcomp) (all p < 0.05). Multivariable logistic regression identified ArTH as an independent correlate of comorbid EDS, with an odds ratio of 3.12 (95% CI: 1.24-8.02; p = 0.016) for the highest quartile of ArTH. In the fully adjusted linear regression model, collapsibility, LG, and ArTH were significant determinants of OSA severity in both subgroups, whereas Vcomp was predictive predominantly among patients with EDS.</p><p><strong>Conclusions: </strong>This study suggests that elevated ArTH is an over-expressed trait in OSA patients with EDS, potentially amplifying the link between respiratory pathophysiology and daytime sleepiness. While LG and collapsibility remain key determinants of OSA severity, these findings highlight the value of ArTH-targeted strategies and endotype-informed stratification for personalized management.</p>","PeriodicalId":21129,"journal":{"name":"Respirology","volume":" ","pages":"756-767"},"PeriodicalIF":7.2,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147481075","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}