Annals of the American Thoracic Society最新文献

{"title":"Clinical Effectiveness of Elexacaftor/Tezacaftor/Ivacaftor in 6 to 11-Year-Olds with Cystic Fibrosis.","authors":"Jessica E Pittman, Sarah Morgan, Morgan McCreary, Phuong T Vu, Peter Jorth, Sonya Heltshe, Lucas R Hoffman, Andrea Kelly, Scott D Sagel, Pradeep K Singh, George M Solomon, Margaret Rosenfeld, Felix Ratjen","doi":"10.1513/AnnalsATS.202501-103OC","DOIUrl":"10.1513/AnnalsATS.202501-103OC","url":null,"abstract":"<p><p><b>Background</b>: Elexacaftor/tezacaftor/ivacaftor (ETI) provided substantial health benefits to children with cystic fibrosis (CF) in clinical trials; there is less information about its effectiveness in a \"real world\" setting. <b>Methods:</b> The pediatric PROMISE substudy enrolled CF children 6 to <12 years of age starting ETI. Outcomes measured at baseline (pre-ETI), 1, 3, 6 and 12 months after ETI initiation included the lung clearance index (LCI_2.5), % predicted forced expiratory volume in 1 second (ppFEV₁), the CFQ-R respiratory domain symptom score (CFQ-R RD), height, weight, oropharyngeal (OP) cultures, culture and DNA-based analysis of sputum microbiology (when sputum available). Sweat chloride was assessed at baseline, 1 and 6 months. <b>Results:</b> 125 participants were enrolled at 20 US CF centers. Lung function improvement post-ETI initiation was rapid and sustained through 12 months, with a mean decrease in LCI_2.5 of -0.79 (95% CI -1.04, - 0.55) and a mean increase in ppFEV₁ of 5.6 (95% CI 3.4, 7.7). Respiratory symptoms also improved significantly (mean change in CFQ-R RD of 4.1 (95% CI [1.94, 6.24]). Sweat chloride decreased significantly at 6 months (mean change -47.2 mmol/L (95% CI -51.99, -43.8)). Weight, BMI and height z scores were not different from baseline at 12 months. <i>S. aureus</i> prevalence in OP or sputum cultures did not change but its density in sputum cultures decreased a mean of 1.47 log₁₀ CFU/g (95% CI -2.37, -0.58) at 12 months. <b>Conclusions:</b> Initiation of ETI in a real-world setting was associated with clinically significant improvements in lung function and symptoms, and decreased <i>S. aureus</i> sputum density at one year; lung function improvements were smaller than those reported in clinical trials.</p>","PeriodicalId":93876,"journal":{"name":"Annals of the American Thoracic Society","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144337334","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Making Sense of Dyspnea in Interstitial Lung Disease.","authors":"Yet H Khor","doi":"10.1513/AnnalsATS.202506-588ED","DOIUrl":"10.1513/AnnalsATS.202506-588ED","url":null,"abstract":"","PeriodicalId":93876,"journal":{"name":"Annals of the American Thoracic Society","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144337336","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Identifying Clinical Research Priorities in Interventional Pulmonary: An Interventional Pulmonology Outcomes Group (IPOG) Working Group Report.","authors":"Jeffrey Thiboutot, Lonny B Yarmus, Fabien Maldonado, Gerard Silvestri, Anil Vachani, Momen Wahidi, Anne Gonzalez, Angela Christine Argento, Hans J Lee, Peter Mazzone, Felix Herth, Alexander Chen, Jason Akulian, Y C Gary Lee, Neal Navani, Christopher Kapp, David Feller-Kopman, Nick A Maskell, Pallav L Shah, Andrew DeMaio, Moishe Liberman, Fergus Gleeson, Alastair J Moore, Erik H F M van der Heijden, Eihab O Bedawi, Jason Beattie, Najib M Rahman, Christopher R Gilbert","doi":"10.1513/AnnalsATS.202502-242PS","DOIUrl":"https://doi.org/10.1513/AnnalsATS.202502-242PS","url":null,"abstract":"<p><p>The field of Interventional Pulmonology suffers from a paucity of methodologically robust studies to inform patient care, often relying on retrospective, single-center, non-comparative cohorts of commercialized products. The Interventional Pulmonary Outcomes Group (IPOG) was established to address the critical need for increased scientific rigor within the Interventional Pulmonology community. IPOG convened a meeting to assess the current state and future needs for minimally invasive lung cancer diagnostics and treatment. The goals of this meeting were to review the current landscape, and identify gaps and barriers in lung cancer diagnostics and therapeutics research. From this discussion short- and long-term research goals and priorities were identified. Nineteen international experts from various institutions and disciplines participated. The top short-term priorities identified were: 1) harmonization of core outcome measures in interventional pulmonology trials, 2) validation of a pathway/structure for the introduction of new technology, 3) establishment of a patient advisory board with focus on patient centered outcomes, 4) early engagement with industry partnerships during clinical trial design. The top long-term priorities identified were: 1) characterize the development, implementation, and role of bronchoscopic ablation, 2) validation and clinical utility of biomarker use and artificial intelligence, 3) implement research training skills for junior investigators in interventional pulmonology, 4) deliver 5 prospective, large clinical trials, with at least one adaptive trial, 5) develop a biorepository accessible to investigators. This perspective reviews the colloquium discussions, the identified priorities and the plans to help address those priorities as well as progress made in the year since its inception. Keywords: Interventional Pulmonology Outcomes Group; Interventional Pulmonology; Lung Cancer; Research; Outcomes.</p>","PeriodicalId":93876,"journal":{"name":"Annals of the American Thoracic Society","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-06-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144310953","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Ambient Air Pollution Exposure and Mortality in the Pulmonary Hypertension Association Registry.","authors":"Xinmei Huang, Aparna Balasubramanian, Jude Moutchia, Kayleen Williams, Nadine Al-Naamani, Melissa Batson, Amanda J Gassett, Jasleen Minhas, Joel D Kaufman, Peter J Leary, Meredith C McCormack, Stephen C Mathai, Steven M Kawut, Coralynn Sack","doi":"10.1513/AnnalsATS.202501-129OC","DOIUrl":"https://doi.org/10.1513/AnnalsATS.202501-129OC","url":null,"abstract":"<p><strong>Rationale: </strong>The effects of long-term ambient air pollution exposure on survival in pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) remain unclear.</p><p><strong>Objectives: </strong>Evaluate the association between exposure to PM2.5, NO2, and O3, and mortality or lung transplantation in the Pulmonary Hypertension Association Registry (PHAR).</p><p><strong>Methods: </strong>2,196 adult patients enrolled in the PHAR provided data between 2015 and 2024. Annual average concentrations of air pollutants, including PM2.5, NO2, and O3, estimated from validated spatiotemporal models in 2015 were linked to each participant's residential address. Cox proportional hazards models evaluated the associations between air pollutant exposures and risk of death or lung transplantation, adjusting for baseline demographics, individual and neighborhood socioeconomic factors (SES), disease severity, and spatial confounders. Additional analyses were adjusted for and stratified by nine U.S. census divisions.</p><p><strong>Results: </strong>Study participants were broadly distributed across U.S. regions, with 72.0% female and a mean age of 55.7. 35.6% had idiopathic PAH, 26.5% had connective tissue disease-associated PAH, and 14.5% had CTEPH. In models adjusted for demographics, individual and neighborhood SES, each IQR increase of PM2.5 was associated with a mortality or lung transplant hazard ratio (HR) of 1.16 (95% confidence interval [CI]: 1.01-1.33). This association was marginally attenuated and not statistically significant after adjusting for spatial covariates, with an HR of 1.12 (95% CI: 0.95-1.31) per IQR increase in PM2.5. We noted regional variation in the observed associations. No significant associations were found with NO2 or O3.</p><p><strong>Conclusions: </strong>Long-term ambient air pollution exposure was not significantly associated with survival in PHAR patients with PAH or CTEPH. Future research should investigate potential modifying effects of regional social determinants and healthcare-related factors on the relationship between air pollution exposure and mortality in these conditions.</p>","PeriodicalId":93876,"journal":{"name":"Annals of the American Thoracic Society","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-06-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144310951","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinical Practice Guidelines: Quality, Breadth, and Currency.","authors":"Christopher J Ryerson, Ann L Jennerich, Linda Nici, Ganesh Raghu, Marya Ghazipura, Shawn D Aaron, A Ioana Cristea, Charles Dela Cruz, M Bradley Drummond, Zarmina Ehsan, Scott E Evans, David Feller-Kopman, Don Hayes, Narayan P Iyer, Alex R Jenkins, Anne E Holland, Susan S Jacobs, Jerry A Krishnan, Madaline Macrea, Eyal Oren, Robert L Owens, Bram Rochwerg, Charlie Strange, Kevin C Wilson","doi":"10.1513/AnnalsATS.202501-097PS","DOIUrl":"https://doi.org/10.1513/AnnalsATS.202501-097PS","url":null,"abstract":"","PeriodicalId":93876,"journal":{"name":"Annals of the American Thoracic Society","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-06-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144310952","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Risk Factors of Posttraumatic-Stress-Disorder Symptom Trajectories for ICU Bereaved Family Members.","authors":"Fur-Hsing Wen, Li-Pang Chuang, Tsung-Hui Hu, Chung-Chi Huang, Wen-Chi Chou, Siew Tzuh Tang","doi":"10.1513/AnnalsATS.202504-458RL","DOIUrl":"https://doi.org/10.1513/AnnalsATS.202504-458RL","url":null,"abstract":"","PeriodicalId":93876,"journal":{"name":"Annals of the American Thoracic Society","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-06-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144310954","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The Relationship between Upper Airway Anatomy and Obesity in Patients with Obstructive Sleep Apnea.","authors":"Liyue Xu, Brendan T Keenan, Andrew S Wiemken, Bethany Staley, Bryndis Benediktsdottir, Sigurdur Juliusson, Allan I Pack, Thorarinn Gislason, Richard J Schwab","doi":"10.1513/AnnalsATS.202410-1027OC","DOIUrl":"https://doi.org/10.1513/AnnalsATS.202410-1027OC","url":null,"abstract":"<p><strong>Rationale: </strong>Obesity is the most important risk factor for obstructive sleep apnea (OSA). However, the complex relationship between obesity and upper airway anatomy (craniofacial structure, soft tissues, and airway caliber) has not been robustly examined in patients with OSA.</p><p><strong>Objectives: </strong>To evaluate the relationship between obesity, based on body mass index (BMI), and upper airway anatomic structures in adult patients with moderate or severe OSA.</p><p><strong>Methods: </strong>In this cross-section study, five hundred and eighty-three patients with apnea hypopnea index (AHI) ≥15 events/hour (mean age 53.7±10.4 years, 81.0% male) were included from Iceland Sleep Apnea Cohort. Airway sizes, soft tissue volumes, and craniofacial dimensions were quantified using three-dimensional magnetic resonance imaging (MRI). We examined how upper airway anatomy associated with BMI using linear regression (continuous BMI) and analysis of covariance (ANCOVA; BMI categories), adjusting for age, sex and AHI.</p><p><strong>Results: </strong>Most upper airway anatomy was significantly associated with BMI among patients with OSA. Higher BMI was associated with a different airway shape, including larger minimum anteroposterior distance at both the retropalatal and retroglossal regions and smaller minimum lateral distance in the retropalatal region. All pharyngeal soft tissues were larger with greater BMI, including the volumes of the tongue (and tongue fat), soft palate (and soft palate fat), lateral walls, fat pads, epiglottis and pterygoids. Patients with lower BMI had smaller craniofacial measures (e.g., distances between hyoid, retropogonion, and 3rd cervical vertebrae, intramandibular volume and naso-oropharyngeal areas) and more retrognathia. BMI was only weakly associated with the proportion of mandibular space occupied by soft tissues (with no difference among BMI groups), suggesting comparable intraoral \"crowdedness\" among patients with OSA at different levels of obesity, albeit for different reasons.</p><p><strong>Conclusions: </strong>Results support associations between obesity and airway shape, soft tissue volumes, and craniofacial measures among patients with moderate-to-severe OSA. These relationships provide insights into anatomical traits leading to OSA in lean and obese patients, and can inform more personalized treatment options.</p>","PeriodicalId":93876,"journal":{"name":"Annals of the American Thoracic Society","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-06-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144310955","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinical Utility of Pulmonary Function Testing in Assessing Longitudinal Outcomes of Deployed Veterans with Preserved Spirometry.","authors":"Siyang Zeng, Nisha C Jani, Anays M Sotolongo, Gang Luo, Mehrdad Arjomandi, Michael J Falvo","doi":"10.1513/AnnalsATS.202411-1205OC","DOIUrl":"https://doi.org/10.1513/AnnalsATS.202411-1205OC","url":null,"abstract":"<p><p><b>Rationale-</b>Deployment to the Southwest Asia Theater of Military Operations is associated with new-onset respiratory symptoms, yet commonly-used parameters on pulmonary function test (PFT) are typically reported to be within the normal range for most deployers- referred to here as deployment-exposed veterans with preserved spirometry (DEPS). The relationship between these normal-range PFT parameters and long-term outcomes has not been thoroughly investigated. <b>Objective-</b>Evaluate the clinical utility of PFT parameters among DEPS and examine associations with baseline respiratory symptoms, functional limitations, healthcare utilization, and longitudinal trajectories. <b>Methods-</b>We identified veterans in the U.S. Department of Veterans Affairs Airborne Hazards and Open Burn Pit Registry who enrolled in the Veterans Health Administration and had at least one PFT after the end of their initial deployment (1990-2019) and before completion of the Registry self-assessment questionnaire (2014-2024). We defined DEPS as having no airflow obstruction (forced expiratory volume in 1 second-to-forced vital capacity ratio≥lower-limit-of-normal or LLN) or restriction (total lung capacity≥LLN). We applied adjusted mixed-effects regression and machine learning to assess the predictive value of PFT parameters for key outcomes. We also examined the longitudinal trajectories of DEPS's PFT patterns using interval-censored Cox proportional hazards regression. <b>Results-</b>Among eligible veterans with complete data (n=3,814), 68% reported respiratory symptoms (modified Medical Research Council or mMRC≥1) but most had preserved spirometry (DEPS, 49%), followed by obstructive (28%), restrictive (21%), and mixed (2%) PFT patterns. Among DEPS (n=1,879, 37±10 years-old, 86% male, 15% current and 19% former smoker, 64% mMRC≥1), several PFT parameters were linked to outcomes, but diffusing capacity (DL_CO) was the strongest predictor, showing significant relationships with many outcomes (Odds ratios ranging from 0.46 to 0.89, all P<0.05). Among the 289 DEPS with follow-up PFT 6±4 years later, those with baseline isolated reduction in DL_CO (<LLN) were more likely to develop restrictive impairments compared to those with normal DL_CO (33% vs. 19%, P=0.048). <b>Conclusion-</b>In a national sample of deployed veterans with military environmental exposures, approximately half had preserved spirometry (DEPS), but many endorsed substantial respiratory symptoms and functional limitation. DEPS with reduced DL_CO appear to be at increased risk for developing lung parenchymal disease over time.</p>","PeriodicalId":93876,"journal":{"name":"Annals of the American Thoracic Society","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-06-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144287449","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A Normal Forced Vital Capacity Does Not Reliably or Equitably Exclude Restriction.","authors":"Alexander T Moffett, Aparna Balasubramanian, Meredith C McCormack, Jaya Aysola, Scott D Halpern, Gary E Weissman","doi":"10.1513/AnnalsATS.202501-009RL","DOIUrl":"10.1513/AnnalsATS.202501-009RL","url":null,"abstract":"","PeriodicalId":93876,"journal":{"name":"Annals of the American Thoracic Society","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-06-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144287448","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Moderate-to-Severe Impairment of DL_CO and iso↓DL_CO Are Associated with All-Cause Mortality in People with HIV.","authors":"Michelle H Zhang, Jessica Fitzpatrick, Jake Branchini, Katerina L Byanova, Rebecca Abelman, Alyssa Feinberg, Peter W Hunt, Jue Lin, Laurence Huang","doi":"10.1513/AnnalsATS.202407-774OC","DOIUrl":"https://doi.org/10.1513/AnnalsATS.202407-774OC","url":null,"abstract":"<p><p>Rationale The most common lung function abnormality in people with HIV (PWH) is a low diffusing capacity for carbon monoxide (DL_CO), which is associated with distinct plasma biomarker patterns, increased symptom burden, and increased mortality. In PWH undergoing spirometry and DL_CO measurement, the most common abnormality is an abnormal DL_CO with normal spirometry (iso↓DL_CO), which is associated with distinct biomarkers and increased symptom burden but whose association with mortality is unknown. Objective: To investigate the relationship between abnormal DL_CO, including iso↓DL_CO, and mortality in PWH. Methods Adult PWH underwent pre- and post-bronchodilator spirometry and single-breath DL_CO measurements from April 2013-January 2023. DL_CO was categorized as normal (DL_CO≥lower limit of normal, LLN), mildly abnormal (60%predicted≤DL_CO<LLN), or moderate-to-severely abnormal (DL_CO≤60%predicted). Iso↓DL_CO was defined as abnormal DL_CO with normal spirometry (FEV1/FVC≥LLN, FEV1≥LLN, FVC≥LLN). Participants' present vital status was assessed through electronic health records review and the National Death Index. Multivariable Cox proportional hazards models adjusted for age, smoking status, history of bacterial pneumonia, and CD4 count were used to estimate associations between DL_CO and all-cause mortality. Results Among 241 participants, 205 (85%) were male and 26 (11%) died during follow-up of 1055 person-years. Among 178 participants with normal spirometry, 16 (9%) died over 770 person-years. Both moderate-to-severe DL_CO impairment (aHR 4.67; 95%CI 1.69-12.9; p=0.003) and moderate-to-severe iso↓DL_CO (aHR 11.0; 95% CI 2.20-55.1; p=0.004) were significantly associated with increased mortality. Conclusions Moderate-to-severe impairment of DL_CO and iso↓DL_CO are associated with all-cause mortality in PWH. Further studies evaluating mechanistic pathways and cause-specific mortality are warranted.</p>","PeriodicalId":93876,"journal":{"name":"Annals of the American Thoracic Society","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-06-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144287451","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}