Respiratory investigation最新文献

New diagnostic criteria and current issues for pulmonary hypertension 肺动脉高压的新诊断标准和当前问题

IF 2.4

Respiratory investigation Pub Date : 2024-09-07 DOI: 10.1016/j.resinv.2024.09.002

引用次数: 0

Transesophageal ultrasound-guided bronchoscopic Acquire TBNB versus Vizishot2 TBNA needles for neoplastic lesions: A retrospective study 经食道超声引导下支气管镜 Acquire TBNB 与 Vizishot2 TBNA 针治疗肿瘤病灶：回顾性研究

IF 2.4

Respiratory investigation Pub Date : 2024-09-04 DOI: 10.1016/j.resinv.2024.08.016

{"title":"Transesophageal ultrasound-guided bronchoscopic Acquire TBNB versus Vizishot2 TBNA needles for neoplastic lesions: A retrospective study","authors":"","doi":"10.1016/j.resinv.2024.08.016","DOIUrl":"10.1016/j.resinv.2024.08.016","url":null,"abstract":"<div><h3>Background</h3><p>Lung cancer is often diagnosed at an advanced stage; however, it has shown improved therapeutic efficacy with the introduction of molecularly targeted drugs and immune checkpoint inhibitors, necessitating accurate molecular diagnosis for effective treatment planning. Traditional sampling techniques, including endobronchial ultrasound-guided transbronchial needle aspiration, frequently require multiple biopsies to obtain sufficient tissues for multiplex testing, highlighting the need for more efficient methods. Therefore, we explored the diagnostic utility of endoscopic ultrasound with bronchoscope-guided fine-needle biopsy (EUS-B-FNB) versus fine-needle aspiration (EUS-B-FNA) in patients with lung cancer, focusing on tissue sample collection for molecular testing. The introduction of the Franseen needle in EUS-B-FNB, characterized by three beveled edges, allows for more tissue collection in cylinder form.</p></div><div><h3>Methods</h3><p>We retrospectively analyzed the data of 97 patients who underwent EUS-B-FNB or EUS-B-FNA at Hakodate Goryoukaku Hospital and evaluated diagnostic yields, safety, and nucleic acid concentrations using collected specimens.</p></div><div><h3>Results</h3><p>The diagnostic yields of EUS-B-FNB and EUS-B-FNA were comparable (92.2% vs. 92.3%), with no significant differences in complications. However, EUS-B-FNB provided significantly higher DNA and RNA concentrations (DNA; 41.05 vs. 10.20 ng/mL; P < 0.0001, RNA; 36.80 vs. 11.80 ng/mL; P = 0.0009), essential for comprehensive molecular testing.</p></div><div><h3>Conclusion</h3><p>This study highlights the potential of EUS-B-FNB for enhancing the molecular diagnosis of lung cancer by ensuring adequate tissue sample collection for multiplex testing, paving the way for personalized medicine. This technique is comparable in safety and efficacy to traditional methods while offering a substantial improvement in the quality of molecular diagnostics.</p></div>","PeriodicalId":20934,"journal":{"name":"Respiratory investigation","volume":null,"pages":null},"PeriodicalIF":2.4,"publicationDate":"2024-09-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142129977","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}

引用次数: 0

Reevaluating diagnosis in interstitial lung disease with a second multidisciplinary discussion 通过第二次多学科讨论重新评估间质性肺病的诊断

IF 2.4

Respiratory investigation Pub Date : 2024-09-04 DOI: 10.1016/j.resinv.2024.08.015

{"title":"Reevaluating diagnosis in interstitial lung disease with a second multidisciplinary discussion","authors":"","doi":"10.1016/j.resinv.2024.08.015","DOIUrl":"10.1016/j.resinv.2024.08.015","url":null,"abstract":"<div><h3>Background</h3><p>The importance of multidisciplinary discussion (MDD) for diagnosing interstitial lung disease (ILD) is emphasized by several international guidelines. While initial diagnoses are often provisional and require periodic re-evaluation, there is a lack of literature regarding the role of follow-up MDD in clinical practice.</p></div><div><h3>Methods</h3><p>From September 2020 to January 2022, patients underwent an initial MDD (MDD1) based on clinical, radiological, and pathological evaluations. Each diagnosis was assigned a confidence level. One year later, a second MDD (MDD2) was conducted for re-evaluation, based on subsequent clinical and radiological information. Changes in diagnosis and confidence levels between MDD1 and MDD2 were assessed.</p></div><div><h3>Results</h3><p>Among 52 patients enrolled in both MDDs, the diagnosis for 13 (25%) was revised at MDD2. Of these, 10 patients were initially diagnosed with unclassifiable ILD, and 3 received a low confidence diagnosis of either idiopathic pulmonary fibrosis or idiopathic nonspecific interstitial pneumonia. The most common diagnostic revision was due to the deterioration after antigen exposure or improvement after antigen avoidance, which resulted in a revised diagnosis of HP at MDD2.</p></div><div><h3>Conclusions</h3><p>Our findings underscore the importance of periodic reassessment of MDD to improve the accuracy of ILD diagnosis. This study highlights the significance of longitudinal clinical and radiological evaluation for diagnostic revision, even in situations when rebiopsy is not feasible<strong>.</strong></p></div>","PeriodicalId":20934,"journal":{"name":"Respiratory investigation","volume":null,"pages":null},"PeriodicalIF":2.4,"publicationDate":"2024-09-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142137010","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}

引用次数: 0

Prevalence and characteristics of dysfunctional breathing in patients with asthma in the Japanese population 日本人口中哮喘患者呼吸功能紊乱的发生率和特征

IF 2.4

Respiratory investigation Pub Date : 2024-08-31 DOI: 10.1016/j.resinv.2024.08.004

{"title":"Prevalence and characteristics of dysfunctional breathing in patients with asthma in the Japanese population","authors":"","doi":"10.1016/j.resinv.2024.08.004","DOIUrl":"10.1016/j.resinv.2024.08.004","url":null,"abstract":"<div><h3>Background</h3><p>Dysfunctional breathing (DB) is a major asthma comorbidity; however, it is not well recognized in Japan. Moreover, it has rarely been reported in the asthma population, and its clinical characteristics are unclear. We aimed to clarify the clinical characteristics of DB as a comorbidity in patients with asthma in Japan. Questionnaire surveys were conducted among patients with asthma at medical facilities in three regions of Japan (Niigata, Kumamoto, and Tokyo).</p></div><div><h3>Methods</h3><p>This cross-sectional questionnaire survey targeting patients with asthma who had regularly visited medical institutions and their doctors was conducted from September to November 2021. The questionnaire addressed the control status and method of treatment. The diagnosis of DB was evaluated using the Nijmegen questionnaire (NQ).</p></div><div><h3>Results</h3><p>There were 2087 eligible participants. Based on their NQ scores, 217 patients were classified into the DB group (NQ ≥ 19). There were significant differences with respect to sex, disease duration, Asthma Control Test (ACT) scores, Patient Health Questionnaire-9 (PHQ-9) scores, type-2 biomarkers, pulmonary function indices, treatment methods, severity, and asthma exacerbations in the previous year between the DB and non-DB groups. In the multivariate analysis, there were significant differences in sex, disease duration (≥15 y), ACT scores (<20), and PHQ-9 scores (≥10). The cluster analysis of cases with DB classified the population into four clusters.</p></div><div><h3>Conclusions</h3><p>The asthma population with DB exhibited several characteristics, including depression and poorly controlled asthma. Further large-scale interventional investigations with longer follow-up periods are necessary to verify these findings.</p></div>","PeriodicalId":20934,"journal":{"name":"Respiratory investigation","volume":null,"pages":null},"PeriodicalIF":2.4,"publicationDate":"2024-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2212534524001254/pdfft?md5=4d5d350c1ded9c0122b7ac147e624dc7&pid=1-s2.0-S2212534524001254-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142095146","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Assessment of health-related quality of life in patients with nontuberculous mycobacterial pulmonary disease: A comprehensive review 评估非结核分枝杆菌肺病患者的健康相关生活质量：综述

IF 2.4

Respiratory investigation Pub Date : 2024-08-31 DOI: 10.1016/j.resinv.2024.08.009

{"title":"Assessment of health-related quality of life in patients with nontuberculous mycobacterial pulmonary disease: A comprehensive review","authors":"","doi":"10.1016/j.resinv.2024.08.009","DOIUrl":"10.1016/j.resinv.2024.08.009","url":null,"abstract":"<div><p>The global incidence of nontuberculous mycobacterial pulmonary disease (NTM-PD) is increasing. The primary aim of pharmacotherapeutic treatment for NTM-PD is to achieve negative bacterial conversion, but this goal is challenging, especially in cases with poor prognosis factors. Moreover, recurrence frequently occurs following successful treatment. Consequently, patient-reported outcomes (PROs) have attracted an increasing amount of attention due to their potential to elucidate the pathophysiology of NTM-PD. The current review article aimed to describe the current understanding of PROs related to health-related quality of life (HRQoL). HRQoL is influenced by a variety of factors; notably, those factors associated with the prognosis of NTM-PD significantly impair HRQoL. In patients with refractory NTM-PD, HRQoL tends to worsen over time. Assessing HRQoL through PROs involves short-term or long-term evaluation tools, which are selected based on their relevance to the patient's condition and the clinician's goals. Understanding the nuances of PROs can be helpful for delivering empathetic care tailored to patients in even the most complex treatment scenarios.</p></div>","PeriodicalId":20934,"journal":{"name":"Respiratory investigation","volume":null,"pages":null},"PeriodicalIF":2.4,"publicationDate":"2024-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2212534524001308/pdfft?md5=82646c04cfbdabd17b2ce74b1c8f6a45&pid=1-s2.0-S2212534524001308-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142095055","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Lung imaging in COPD and asthma 慢性阻塞性肺病和哮喘的肺部成像

IF 2.4

Respiratory investigation Pub Date : 2024-08-29 DOI: 10.1016/j.resinv.2024.08.014

{"title":"Lung imaging in COPD and asthma","authors":"","doi":"10.1016/j.resinv.2024.08.014","DOIUrl":"10.1016/j.resinv.2024.08.014","url":null,"abstract":"<div><p>Chronic obstructive pulmonary disease (COPD) and asthma are common lung diseases with heterogeneous clinical presentations. Lung imaging allows evaluations of underlying pathophysiological changes and provides additional personalized approaches for disease management. This narrative review provides an overview of recent advances in chest imaging analysis using various modalities, such as computed tomography (CT), dynamic chest radiography, and magnetic resonance imaging (MRI). Visual CT assessment localizes emphysema subtypes and mucus plugging in the airways. Dedicated software quantifies the severity and spatial distribution of emphysema and the airway tree structure, including the central airway wall thickness, branch count and fractal dimension of the tree, and airway-to-lung size ratio. Nonrigid registration of inspiratory and expiratory CT scans quantifies small airway dysfunction, local volume changes and shape deformations in specific regions. Lung ventilation and diaphragm movement are also evaluated on dynamic chest radiography. Functional MRI detects regional oxygen transfer across the alveolus using inhaled oxygen and ventilation defects and gas diffusion into the alveolar–capillary barrier tissue and red blood cells using inhaled hyperpolarized <sup>129</sup>Xe gas. These methods have the potential to determine local functional properties in the lungs that cannot be detected by lung function tests in patients with COPD and asthma. Further studies are needed to apply these technologies in clinical practice, particularly for early disease detection and tailor-made interventions, such as the efficient selection of patients likely to respond to biologics. Moreover, research should focus on the extension of healthy life expectancy in patients at higher risk and with established diseases.</p></div>","PeriodicalId":20934,"journal":{"name":"Respiratory investigation","volume":null,"pages":null},"PeriodicalIF":2.4,"publicationDate":"2024-08-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2212534524001357/pdfft?md5=32f1763a43da6250614dd7ccc07d1abe&pid=1-s2.0-S2212534524001357-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142095054","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Cough severity visual analog scale scores and quality of life in patients with refractory or unexplained chronic cough 难治性或不明原因慢性咳嗽患者的咳嗽严重程度视觉模拟量表评分和生活质量

IF 2.4

Respiratory investigation Pub Date : 2024-08-27 DOI: 10.1016/j.resinv.2024.08.005

{"title":"Cough severity visual analog scale scores and quality of life in patients with refractory or unexplained chronic cough","authors":"","doi":"10.1016/j.resinv.2024.08.005","DOIUrl":"10.1016/j.resinv.2024.08.005","url":null,"abstract":"<div><h3>Background</h3><p>Refractory chronic cough (RCC) and unexplained chronic cough (UCC) adversely affect patients’ quality of life (QoL). This multicenter, non-interventional study evaluates the relationship between cough severity and QoL and other patient-reported outcomes (PROs) in Spanish outpatients.</p></div><div><h3>Methods</h3><p>RCC/UCC patients self-administered a printed survey comprising the cough-severity visual analog scale (VAS), adapted Cough Severity Diary (CSD), and Leicester Cough Questionnaire (LCQ), plus purpose-designed items regarding the physical and everyday-life impact of cough. Patients were stratified into VAS score tertiles. The impact of cough on QoL and other PROs in each tertile, and relationships between LCQ scores and the tertiles, were assessed.</p></div><div><h3>Results</h3><p>The VAS was completed by 189 patients, and VAS score tertiles were identified as 0–50, 60–70, and 80–100 mm. The only between-tertile difference in demographic or cough characteristics was cough duration. VAS score tertiles were linearly associated with mean LCQ domain and total scores, as well as the proportion of patients with the highest scores on all adapted CSD items, and almost all physical and everyday-life impact items. In multiple linear-regression models, an increase of one tertile in the VAS score was associated with a decrease of 2.23 points in the LCQ total score, indicating poorer cough-related QoL.</p></div><div><h3>Conclusion</h3><p>As self-assessed in patients with RCC/UCC, cough-severity VAS scores were strongly associated with the impact of cough on QoL and everyday life. Patients with VAS scores of 60–100 mm reported the greatest impact and thus may benefit the most from targeted cough therapies.</p></div>","PeriodicalId":20934,"journal":{"name":"Respiratory investigation","volume":null,"pages":null},"PeriodicalIF":2.4,"publicationDate":"2024-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2212534524001266/pdfft?md5=e1e5f03ef75732fce9e170221ecab706&pid=1-s2.0-S2212534524001266-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142087619","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Efficacy, safety, and pharmacokinetics of inhaled treprostinil in Japanese patients with pulmonary hypertension associated with interstitial lung disease 日本间质性肺病肺动脉高压患者吸入曲普瑞替尼的疗效、安全性和药代动力学

IF 2.4

Respiratory investigation Pub Date : 2024-08-27 DOI: 10.1016/j.resinv.2024.07.020

{"title":"Efficacy, safety, and pharmacokinetics of inhaled treprostinil in Japanese patients with pulmonary hypertension associated with interstitial lung disease","authors":"","doi":"10.1016/j.resinv.2024.07.020","DOIUrl":"10.1016/j.resinv.2024.07.020","url":null,"abstract":"<div><h3>Background</h3><p>The INCREASE trial, conducted in the United States, showed that inhaled treprostinil improved exercise capacity in pulmonary hypertension associated with interstitial lung disease (PH-ILD). However, hemodynamic and pharmacokinetic measurements were not performed in the trial. The objective of this trial was to evaluate the efficacy on hemodynamics and exercise capacity, safety, and pharmacokinetics (PK) of inhaled treprostinil in Japanese patients with PH-ILD.</p></div><div><h3>Methods</h3><p>This trial was a multicenter, non-randomized, open-label, single-arm trial of patients with PH-ILD. Inhaled treprostinil was administered at 3 breaths (18 μg)/session four times daily, and the dose was gradually increased to a maximum of 12 breaths (72 μg)/session. The primary endpoints were the change of pulmonary vascular resistance index (PVRI) and peak 6-min walking distance (6MWD) from baseline to week 16. Endpoints also included other efficacy parameters, safety, and PK.</p></div><div><h3>Results</h3><p>Twenty patients received inhaled treprostinil. At week 16, PVRI decreased from baseline by −40.1% (95% CI, −53.1 to −27.2) and peak 6MWD increased by 13.0 m (95% CI, −15.0 to 49.0). The most frequently reported adverse events related with treprostinil were cough, malaise and blood pressure decreased. PK was similar to those in pulmonary arterial hypertension (PAH) patients.</p></div><div><h3>Conclusions</h3><p>Treatment with inhaled treprostinil using the same dosing regimen as in the INCREASE trial resulted in improvements in hemodynamics and exercise capacity with a favorable tolerability and safety profile in Japanese patients with PH-ILD.</p></div>","PeriodicalId":20934,"journal":{"name":"Respiratory investigation","volume":null,"pages":null},"PeriodicalIF":2.4,"publicationDate":"2024-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2212534524001217/pdfft?md5=d6cd2f0673b6f84e80830ccd173f2fdc&pid=1-s2.0-S2212534524001217-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142087620","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Factors associated with presbyphagia in patients with community-acquired pneumonia: A cross-sectional study 社区获得性肺炎患者老花眼的相关因素：横断面研究

IF 2.4

Respiratory investigation Pub Date : 2024-08-27 DOI: 10.1016/j.resinv.2024.08.008

{"title":"Factors associated with presbyphagia in patients with community-acquired pneumonia: A cross-sectional study","authors":"","doi":"10.1016/j.resinv.2024.08.008","DOIUrl":"10.1016/j.resinv.2024.08.008","url":null,"abstract":"<div><h3>Background</h3><p>Presbyphagia, an age-related decline in swallowing function, is considered a precursor stage of dysphagia and a risk state that can lead to aspiration pneumonia and malnutrition. We examined factors associated with presbyphagia in patients with community-acquired pneumonia (CAP).</p></div><div><h3>Methods</h3><p>A cross-sectional study was conducted with 80 patients with CAP aged ≥65 years admitted to an acute care hospital between June 2021 and April 2024. Presbyphagia was assessed using the 10-item Eating Assessment Tool. The survey items included grip strength, body mass index, the Mini-Cog©, repetitive saliva swallowing test, tongue pressure, and evaluations for sarcopenia and frailty. Logistic regression analysis was performed to examine the factors associated with presbyphagia after adjusting for age and sex.</p></div><div><h3>Results</h3><p>Of 80 patients, 44 (55%) had presbyphagia. The presbyphagia group was older, had lower Barthel Index scores, and had a higher proportion of history of cerebrovascular accident, sarcopenia and frailty than the non-presbyphagia group. Logistic regression analysis revealed frailty (adjusted odds ratio: 3.106, 95% confidence interval: 1.161–8.313, <em>p</em> = 0.024) was significantly associated with presbyphagia.</p></div><div><h3>Conclusions</h3><p>Our results revealed a significant association between presbyphagia and frailty in patients with CAP. The relationship between presbyphagia and frailty suggests that these conditions are not caused by a single functional decline or structural change but by a combination of factors. Therefore, it is crucial to comprehensively evaluate presbyphagia in patients with CAP to provide appropriate interventions.</p></div>","PeriodicalId":20934,"journal":{"name":"Respiratory investigation","volume":null,"pages":null},"PeriodicalIF":2.4,"publicationDate":"2024-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2212534524001291/pdfft?md5=0500f55545a511c9d5c16e2ba9967372&pid=1-s2.0-S2212534524001291-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142087626","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

“Bubbly lung”, a honeycombing variant with more favorable outcome "气泡肺"，结果更理想的蜂窝状变体

IF 2.4

Respiratory investigation Pub Date : 2024-08-26 DOI: 10.1016/j.resinv.2024.08.012

{"title":"“Bubbly lung”, a honeycombing variant with more favorable outcome","authors":"","doi":"10.1016/j.resinv.2024.08.012","DOIUrl":"10.1016/j.resinv.2024.08.012","url":null,"abstract":"<div><h3>Background</h3><p>Although radiologic honeycombing is generally associated with progressive fibrosis and a dismal prognosis, some patients display an unexpectedly indolent clinical course. We aimed to assess for variants of honeycombing associated with a more favorable prognosis.</p></div><div><h3>Methods</h3><p>A computer-assisted search was conducted to identify patients encountered at Mayo Clinic from 1998 to 2022 who had undergone chest CT that manifested exuberant honeycombing. Medical records and chest imaging studies were reviewed to identify clinical, pulmonary function and radiologic features.</p></div><div><h3>Results</h3><p>Among 136 patients with exuberant honeycombing, 23 patients were identified with CT features of what we termed “bubbly lung” characterized by extensive macrocystic fibrosis; 17 (74%) were female with a median age of 71 years (range, 32–88) at baseline. Underlying diagnoses were ANCA associated vasculitis (22%), overlap CTD (22%), rheumatoid arthritis (17%), IPF (17%), IPAF (9%), systemic sclerosis (4%), undifferentiated CTD (4%), and dermatomyositis (4%). Median FVC was 78% predicted (range 35–112), median DLco was 41% predicted (range 10–92), and median TLC was 73% predicted (range 57–116). Serial FVC measurements were available for 19 (83%) patients with a median interval of 4.7 years (range: 0.4–20); median FVC change per year was 23 ml (range −279 to +232) and median FVC % predicted change per year was 0.00% (range −3.20 to +6.79%). The median survival was 7.1 years, 5-year survival was 76% (95% CI: 58%–100%) and 10-year survival was 48%.</p></div><div><h3>Conclusions</h3><p>“Bubbly lung” is a variant of exuberant honeycombing that is associated with better-than-expected outcome and FVC decline per year.</p></div>","PeriodicalId":20934,"journal":{"name":"Respiratory investigation","volume":null,"pages":null},"PeriodicalIF":2.4,"publicationDate":"2024-08-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142076026","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}

引用次数: 0