Therapeutic Advances in Respiratory Disease最新文献

{"title":"A real-world study to evaluate effectiveness of mepolizumab in treating severe asthma in Taiwan (REMIT).","authors":"Shih-Lung Cheng, Shu-Min Lin, Chung-Kan Peng, Ming-Cheng Chan, Sheng-Yeh Shen, Ping-Hung Kuo, Chien-Hao Lai, Chou-Chin Lan, Chung-Yu Chen, Ching-Hsiung Lin, Kuang-Ming Liao, Po-Hao Feng, Jiin-Torng Wu, Yu-Feng Wei, Xiaomeng Xu, Rafael Alfonso-Christancho, Tina Lai, Aldo Navarro, Dominique Milea, Diahn-Warng Perng","doi":"10.1177/17534666241308406","DOIUrl":"10.1177/17534666241308406","url":null,"abstract":"<p><strong>Background: </strong>REMIT is the first real-world study of mepolizumab effectiveness in patients with severe asthma (SA) in Taiwan.</p><p><strong>Objectives: </strong>The primary objective evaluated changes in clinically significant exacerbations (CSEs; defined as use of oral corticosteroids (OCS) or emergency department (ED) visits and/or hospitalizations) in the 12 months pre- and post-mepolizumab treatment. Secondary objectives assessed changes in the number of CSEs requiring ED visits/hospitalizations and daily maintenance OCS (mOCS) dosage 12 months pre- and post-mepolizumab treatment. Three- and four-component clinical remissions were analyzed based on OCS-free, exacerbation-free, and asthma control (± stability in lung function).</p><p><strong>Design: </strong>REMIT was a retrospective, observational, self-controlled study analyzing patients in Taiwan with SA who were newly prescribed subcutaneous mepolizumab 100 mg Q4W.</p><p><strong>Methods: </strong>Data were extracted from records of 15 medical centers in Taiwan for patients indexed between November 1, 2018 and October 31, 2020.</p><p><strong>Results: </strong>A total of 170 patients were included: mean age at index date, 58.7 years; 53.5% female; 100% Chinese; 7.1% with chronic rhinosinusitis with nasal polyps, 1.8% with eosinophilic granulomatosis with polyangiitis, 1.2% with hypereosinophilic syndrome; and 55.7% with blood eosinophil count >300/µL. Pre-treatment, 71.2% had ⩾2 exacerbations, and 28.7% were on mOCS; 75.3% had no prior biologic treatment, and 24.7% had switched from other biologics. Most patients (80.0%) completed ⩾10 mepolizumab doses. Following the first mepolizumab administration (index date), CSEs reduced by 46.0% (rate ratio (RR): 0.545, 95% confidence interval (CI): 0.418-0.710; <i>p</i> < 0.0001) in the 12 months post-index. Exacerbations requiring ED visits/hospitalization reduced by 46.9% (RR: 0.531, 95% CI: 0.349-0.808; <i>p</i> = 0.0031). Median mOCS dose reduced by 100% by end of study and 81.8% of patients discontinued mOCS post-treatment. After 1 year of mepolizumab treatment, 28% and 23% patients achieved three- and four-component clinical remission, respectively.</p><p><strong>Conclusion: </strong>Mepolizumab use in a patient population in Taiwan with SA significantly reduced CSEs and mOCS use in routine clinical practice.</p>","PeriodicalId":22884,"journal":{"name":"Therapeutic Advances in Respiratory Disease","volume":"19 ","pages":"17534666241308406"},"PeriodicalIF":3.3,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11744627/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143011950","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cystic fibrosis: a model for research and management of respiratory diseases.","authors":"Almudena Felipe Montiel, Antonio Álvarez Fernández, Alvar Agustí, Eva Polverino","doi":"10.1177/17534666251329792","DOIUrl":"10.1177/17534666251329792","url":null,"abstract":"","PeriodicalId":22884,"journal":{"name":"Therapeutic Advances in Respiratory Disease","volume":"19 ","pages":"17534666251329792"},"PeriodicalIF":3.3,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11963717/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143764710","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Heterogeneity and individualized therapy for eosinophilic granulomatosis with polyangiitis.","authors":"Lijuan Hua, Min Xie","doi":"10.1177/17534666251318615","DOIUrl":"10.1177/17534666251318615","url":null,"abstract":"<p><p>Eosinophilic granulomatosis with polyangiitis (EGPA), as a heterogeneous component of antineutrophil cytoplasmic antibody-associated vasculitis, may be induced by a series of environmental and genetic factors, involved with a variety of immune cells and immune components, and presented with various clinical manifestations, with multiple organs and systems (respiratory, skin, heart, kidney, nerve, etc.) involved. The choice of glucocorticoid (GC) dosage and immunosuppressant in traditional treatment strategies varies greatly from individual to individual and is not universally applicable in all the EGPA phenotype spectrum, especially in relapsing or refractory diseases. With the understanding of the heterogeneity of EGPA, a variety of therapeutic approaches are emerging and improving the traditional treatment model. In this review, we summarized the heterogeneity of EGPA etiology and pathogenesis. Clinical and pathological manifestations of the same organ involved also show significant differences and there are even gender differences. Biological treatments that mainly target type 2 inflammatory pathways are widely used in clinical practice for remission induction and maintenance of EGPA. Targeted biological therapy has shown excellent performance in reducing GC dosage and controlling symptoms and recurrence. However, a large number of high-quality randomized controlled studies are still under research for relapsing or refractory EGPA with special organ involvement. We believe that EGPA has a highly heterogeneous phenotype spectrum, and the treatment patterns targeting key molecules in the pathogenesis are of great value for individual treatment of EGPA.</p>","PeriodicalId":22884,"journal":{"name":"Therapeutic Advances in Respiratory Disease","volume":"19 ","pages":"17534666251318615"},"PeriodicalIF":3.3,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11843704/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143469210","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Optimizing diagnostic yield in pulmonary lesions: impact of combined sampling tools and EBUS-TBNA during radial EBUS.","authors":"Eduardo Tuta-Quintero, Luis F Giraldo-Cadavid, Catalina Sanmiguel-Reyes, Maria E Navia, Ricardo Cardenas, Alirio Bastidas, Angelica Mora, Nelson Páez-Espinel, Lucía Viola, Miguel Suárez, Libardo Jiménez-Maldonado, Mauricio Durán, Jacqueline Mugnier, Javier Flandes","doi":"10.1177/17534666251357699","DOIUrl":"10.1177/17534666251357699","url":null,"abstract":"<p><strong>Background: </strong>Radial endobronchial ultrasound (r-EBUS) is a minimally invasive procedure used to evaluate pulmonary lesions suspicious of cancer. Current information on the effect of combining different sampling tools used during r-EBUS or the addition of linear EBUS (EBUS-TBNA) on its diagnostic performance is limited.</p><p><strong>Objectives: </strong>To evaluate the effect on diagnostic performance of the systematic addition of different sampling tools and EBUS-TBNA during r-EBUS, as well as the rate of peri-procedural complications.</p><p><strong>Design: </strong>This was an observational, analytical cohort study designed to evaluate diagnostic accuracy.</p><p><strong>Methods: </strong>We calculated diagnostic accuracy statistics and used the Cochran-Armitage statistical test to assess the effect of combining techniques on diagnostic performance. Diagnostic success (DS) was defined as true positives and true negatives, while diagnostic failure was defined as false positives and false negatives.</p><p><strong>Results: </strong>A total of 309 patients were included, with a mean age of 67.9 years (standard deviation: 10.97); 50.8% (157/309) were male. The bronchial washing had a DS rate of 49%, while bronchial brushing showed a DS rate of 61%. The combination of bronchial washing and bronchial brushing improved the r-EBUS performance to 63%. Combining bronchial washing, bronchial brushing, and transbronchial biopsy increased the performance to 70%, and the addition of EBUS-TBNA raised the diagnostic performance to 80% (<i>p</i> < 0.001; Cochran-Armitage test). The overall complication rate was 6.4% (20/309), with pneumothorax occurring in 1.2% (4/309), bronchospasm in 3.8% (12/309), and bleeding in 1.2% (4/309).</p><p><strong>Conclusion: </strong>The use of multiple sampling tools significantly contributes to the DS of r-EBUS, particularly with the addition of EBUS-TBNA. This approach maintains a low complication rate.</p>","PeriodicalId":22884,"journal":{"name":"Therapeutic Advances in Respiratory Disease","volume":"19 ","pages":"17534666251357699"},"PeriodicalIF":3.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12319195/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144761423","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Corrigendum to \"A real-world study to evaluate effectiveness of mepolizumab in treating severe asthma in Taiwan (REMIT)\".","authors":"","doi":"10.1177/17534666251322341","DOIUrl":"10.1177/17534666251322341","url":null,"abstract":"","PeriodicalId":22884,"journal":{"name":"Therapeutic Advances in Respiratory Disease","volume":"19 ","pages":"17534666251322341"},"PeriodicalIF":3.3,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11847316/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143477036","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Impact of rheumatoid arthritis, seropositivity and disease-modifying anti-rheumatic drugs on mortality risk in bronchiectasis.","authors":"Hayoung Choi, Kyungdo Han, Jin Hyung Jung, Anthony De Soyza, Hyungjin Kim, Dong Wook Shin, Hyun Lee","doi":"10.1177/17534666251360071","DOIUrl":"10.1177/17534666251360071","url":null,"abstract":"<p><strong>Background: </strong>Comorbid rheumatoid arthritis (RA) is known to be associated with excess mortality in patients with bronchiectasis. However, whether excess mortality is affected by RA seropositivity and is altered by using disease-modifying anti-rheumatic drugs (DMARDs) remains unknown.</p><p><strong>Objectives: </strong>To assess the association between comorbid RA and mortality in participants with bronchiectasis, plus the impacts of seropositivity and DMARDs on this association.</p><p><strong>Design: </strong>A retrospective cohort study.</p><p><strong>Methods: </strong>Mortality rates were compared between participants with bronchiectasis-RA overlap syndrome (BROS) (<i>n</i> = 3355; 2632 seropositive RA (SPRA) and 723 seronegative RA (SNRA)) and 1:5 age- and sex-matched participants with bronchiectasis only (<i>n</i> = 16,240) who were enrolled between 2010 and 2017 in the Korean National Health Insurance Service database. The participants were followed up from 1 year after RA diagnosis or the corresponding index date to the date of death, censored date, or 31 December 2020.</p><p><strong>Results: </strong>During a median follow-up of 5.8 years (interquartile range, 4.2-7.8 years), participants with BROS revealed a 2.09-fold higher mortality risk compared with participants with bronchiectasis only, even after adjusting for potential confounders (95% confidence interval (CI), 1.88-2.33). In an analysis of RA serologic status using a fully adjusted model, participants with SPRA and those with SNRA showed 2.34-fold (95% CI, 2.09-2.62) and 1.29-fold (95% CI, 1.01-1.65) increased risks, respectively, than participants with bronchiectasis only. DMARDs use was related to increased mortality.</p><p><strong>Conclusion: </strong>The presence of RA doubles the mortality risk in patients with bronchiectasis. Increased mortality risk was more evident in patients with SPRA and those who use DMARDs. Causality cannot be ascertained, but these data suggest that rheumatic inflammation may affect disease progression and excess mortality in patients with BROS.</p>","PeriodicalId":22884,"journal":{"name":"Therapeutic Advances in Respiratory Disease","volume":"19 ","pages":"17534666251360071"},"PeriodicalIF":3.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12329186/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144790096","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Thanks to Reviewers.","authors":"","doi":"10.1177/17534666251335195","DOIUrl":"https://doi.org/10.1177/17534666251335195","url":null,"abstract":"","PeriodicalId":22884,"journal":{"name":"Therapeutic Advances in Respiratory Disease","volume":"19 ","pages":"17534666251335195"},"PeriodicalIF":3.3,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144052513","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Palliative care for chronic respiratory diseases in low- and middle-income countries: a narrative review.","authors":"Zeina Al Achkar, Toufic Chaaban","doi":"10.1177/17534666251318616","DOIUrl":"10.1177/17534666251318616","url":null,"abstract":"<p><p>Palliative care is essential for patients with chronic pulmonary diseases, especially in low- and middle-income countries (LMICs). Chronic respiratory diseases (CRDs), such as chronic obstructive pulmonary disease and interstitial lung diseases, cause significant morbidity and mortality globally, with a heavy burden in LMICs. Despite the need, access to palliative care in LMICs is limited, leading to inadequate symptom management and support. Palliative care benefits include improved quality of life, reduced healthcare costs, and increased patient and family satisfaction. However, barriers in LMICs, including limited resources, infrastructure, and trained providers, as well as cultural and regulatory challenges, hinder care delivery. Early integration of palliative care for patients with CRDs can enhance outcomes and reduce healthcare utilization, yet it remains underutilized in these regions. This review highlights the challenges and impact of palliative care for CRDs in these regions. Addressing these issues requires regulatory reforms, provider education, and investments in healthcare infrastructure. Solutions include national policies, training healthcare professionals, telemedicine, and research collaborations. Understanding and addressing barriers to palliative care in LMICs is crucial for improving care quality and outcomes for patients with CRDs.</p>","PeriodicalId":22884,"journal":{"name":"Therapeutic Advances in Respiratory Disease","volume":"19 ","pages":"17534666251318616"},"PeriodicalIF":3.3,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11831687/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143433824","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A mixed-methods pilot study of domiciliary nasal high-flow therapy for breathlessness in people with chronic obstructive pulmonary disease who do not qualify for domiciliary long-term oxygen therapy.","authors":"Natasha Smallwood, Amy Pascoe, Catherine Buchan, Aaron K Wong, David Currow, Brian Le","doi":"10.1177/17534666251314722","DOIUrl":"10.1177/17534666251314722","url":null,"abstract":"<p><strong>Background: </strong>High-flow nasal oxygen (HFNO) therapy delivers humidified, heated air with flow rates of up to 60 L/min with oxygen entrained. HFNO has advantages over conventional oxygen therapy, including precise and reliable fraction of inspired oxygen delivery, therefore is recommended as first-line treatment for people with acute hypoxaemic respiratory failure.</p><p><strong>Objectives: </strong>This pilot study aimed to determine the feasibility and acceptability of domiciliary nasal high flow (NHF) without entrained oxygen for people with chronic obstructive pulmonary disease (COPD) and severe breathlessness.</p><p><strong>Design: </strong>Single-arm, mixed-methods, pilot study of an 8-day, air-only NHF intervention in adults with COPD and severe breathlessness not requiring domiciliary oxygen therapy.</p><p><strong>Methods: </strong>Participants were educated and advised to use NHF for ⩾7 h per night for 7 nights with day use as desired. Patient-reported outcome measures were assessed on Days 3, 5 and 8.</p><p><strong>Primary outcome: </strong>feasibility.</p><p><strong>Secondary outcomes: </strong>breathlessness (dyspnoea), fatigue, quality of life, physical function, sleep, tolerability and safety. Acceptability was also assessed through semi-structured interviews.</p><p><strong>Results: </strong>Fifteen participants were enrolled (mean age 73.6; 40% women; mean FEV₁ 41% predicted, mean DLCO 43.0% predicted; mean modified Medical Research Council score 3.7). Thirteen (87%) completed the trial, with 8 (54%) keeping the device at the end of the trial and 3 (20%) continuing use long-term. Adherence varied, with average daily usage higher amongst participants who kept the device compared to those who returned it (6.8 h ± 2.3 h vs 3.4 h ± 3.7 h). No changes in worst breathlessness (mean = 0.7, SD = 1.2, <i>p</i> = 0.109), dyspnoea mastery (mean = 0.3, SD = 0.6, <i>p</i> = 0.176) or fatigue (mean = 0.0, SD = 2.4, <i>p</i> = 1.00) were observed at Day 8 compared to baseline. No significant adverse events were reported. Qualitative interviews demonstrated subjective improvements in breathlessness, dry mouth and sputum production for some participants, whilst others found NHF uncomfortable. Fear of NHF dependence and concerns regarding long-term running costs were reported.</p><p><strong>Conclusion: </strong>Domiciliary NHF was a feasible intervention, albeit with varied adoption and acceptability. These trial implementation outcomes may have affected preliminary effectiveness outcomes. Further research is required to determine what role domiciliary NHF may have for people with COPD and severe breathlessness.</p><p><strong>Trial registration: </strong>ACTRN12621000044820.</p>","PeriodicalId":22884,"journal":{"name":"Therapeutic Advances in Respiratory Disease","volume":"19 ","pages":"17534666251314722"},"PeriodicalIF":3.3,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11898029/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143606358","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A clinical predictive model for the long-term survival of progressive fibrosis interstitial lung disease patients.","authors":"Jin-Min Gu, Si-Yao Xiao, Bo-Tao Chen, Shao-Ting Kang, Wei-Chao Li, Mei-Yi Zhang, Yang Yu, Gui-Ling Han","doi":"10.1177/17534666251379586","DOIUrl":"10.1177/17534666251379586","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Background: &lt;/strong&gt;In patients with chronic fibrosing interstitial lung disease (ILD), some may develop a progressive fibrosing (PF) phenotype, which presents as rapid progression and often results in poor clinical outcomes.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Objective: &lt;/strong&gt;The objective of this study was to construct and test a model to identify independent predictors of mortality in PF-ILD and to trace the lung function trajectory of patients with PF-ILD.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Design: &lt;/strong&gt;This multicenter retrospective cohort study enrolled patients with PF-ILD from two distinct centers with 8-year follow-up to develop and validate a prognostic nomogram based on clinical factors and assess longitudinal lung function trajectories.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Methods: &lt;/strong&gt;We enrolled patients diagnosed with PF-ILD from China-Japan Friendship Hospital (training cohort) and Jiulongpo Hospital of Traditional Chinese Medicine (validation cohort). Survival status was recorded during the 8-year follow-up period. Clinical demographics, laboratory data, pulmonary function test (PFT) results, and high-resolution computed tomography results were collected for analysis. A training cohort of patients with PF-ILD was used to identify predictors of mortality, which were then validated in an external cohort. A nomogram was established based on multivariate factors. The predictive performance of the model was evaluated using receiver operating characteristic curves and calibration curves. Survival estimates were performed using the Kaplan-Meier method and compared using the log-rank test. The PFT trajectory was estimated using a linear mixed model.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results: &lt;/strong&gt;A total of 1419 patients with PF-ILD from China-Japan Friendship Hospital (training cohort) and 282 patients with PF-ILD from Jiulongpo Hospital of Traditional Chinese Medicine (validation cohort) were enrolled. During the 8-year follow-up, 150 (10.57%) patients received lung transplantation, while 43.55% (&lt;i&gt;n&lt;/i&gt; = 618) of cases reached mortality, with a median survival time of 53 months in the BJ cohort. A predictive model was built based on ILD subgroups, baseline Forced Vital Capacity%&lt;sub&gt;pred&lt;/sub&gt; (FVC%pred), baseline Diffusing Capacity of the Lung for Carbon Monoxide%&lt;sub&gt;pred&lt;/sub&gt; (DLCO%pred), age at diagnosis, antifibrosis treatment, gastroesophageal reflux complication, C-Reactive Protein levels, and BMI. We also found AEs and progressive declines in FVC and DLCO, particularly after the third year post-diagnosis, were strongly associated with poor prognosis and may serve as important longitudinal biomarkers for risk stratification in PF-ILD.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusion: &lt;/strong&gt;A predictive model incorporating multiple factors effectively predicted 8-year survival in patients with PF-ILD. In addition to these baseline predictors, AEs and progressive declines in FVC and DLCO were strongly associated with poor prognosis and may serve as valuable longitudinal biomarkers for ongoing risk strati","PeriodicalId":22884,"journal":{"name":"Therapeutic Advances in Respiratory Disease","volume":"19 ","pages":"17534666251379586"},"PeriodicalIF":3.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12477370/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145186737","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}