John A Mackintosh, Simon L F Walsh, Mario Silva, Lucio Calandriello, Stephen M Humphries, David A Lynch, Helen E Jo, Ian Glaspole, Christopher Grainge, Nicole Goh, Peter M A Hopkins, Yuben Moodley, Paul N Reynolds, Christopher Zappala, Gregory Keir, Wendy A Cooper, Annabelle M Mahar, Samantha Ellis, Athol U Wells, Tamera J Corte
Background and objectives: In fibrotic lung disease, radiological features of usual interstitial pneumonia (UIP) are associated with adverse outcomes. The aim of this study was to evaluate the likelihood of definite UIP on HRCT as a determinant of outcome in patients with pulmonary fibrosis.
Methods: All available HRCT from the Australian IPF Registry (AIPFR) were retrospectively reviewed by two radiologists. The radiologists were asked to assign a likelihood (%) within each of the four ATS/ERS/JRS/ALAT 2018 UIP categories, summating to 100%, enabling them to not be constrained by a single UIP category (e.g., 5% definite UIP, 95% alternative to UIP). Likelihood of UIP was defined as the percentage likelihood score for the definite UIP category. Semi-quantitative features were also estimated. Cox proportional hazards regression was performed to evaluate the prognostic utility of the likelihood scores.
Results: HRCT was available for analysis in 515 patients. Median follow-up of the cohort was 4.1 years (IQR 2.0-6.2 years). Median total ILD extent was 30% (IQR20-45%), with traction bronchiectasis and honeycombing present in 96.3% and 45.4% of cases respectively, and all three variables were associated with worse transplant-free survival. A pattern of definite or probable UIP was favoured in 26.4% and 32.4% of cases respectively. Likelihood of definite UIP was independently associated with both reduced transplant-free survival (HR1.12, 95% CI 1.04-1.20; p = 0.002) and 12-month progression (HR1.19, 95% CI 1.04-1.35; p = 0.009) and was superior to the ATS/ERS/JRS/ALAT 2018 guideline-based categories for these associations.
Conclusions: We demonstrate that estimation of the likelihood of definite UIP is strongly associated with transplant-free survival and 12-month disease progression.
背景和目的：在纤维化肺疾病中，通常间质性肺炎（UIP）的影像学特征与不良结局相关。本研究的目的是评估HRCT上确定UIP作为肺纤维化患者预后决定因素的可能性。方法：两位放射科医生回顾性分析了澳大利亚IPF登记处（AIPFR）所有可用的HRCT。要求放射科医生在四个ATS/ERS/JRS/ALAT 2018 UIP类别中每个类别中分配一个可能性（%），总和为100%，使他们不受单一UIP类别的限制（例如，5%的确定UIP， 95%的替代UIP）。UIP的可能性被定义为确定的UIP类别的百分比可能性得分。还估计了半定量特征。采用Cox比例风险回归来评估似然评分的预后效用。结果：515例患者可进行HRCT分析。中位随访期为4.1年（IQR为2.0-6.2年）。中位总ILD范围为30% (IQR20-45%)，牵引性支气管扩张和蜂窝状分别出现在96.3%和45.4%的病例中，所有这三个变量都与较差的无移植生存相关。明确或可能的upp模式分别在26.4%和32.4%的病例中得到青睐。明确的UIP的可能性与减少的无移植生存（HR1.12, 95% CI 1.04-1.20; p = 0.002）和12个月的进展（HR1.19, 95% CI 1.04-1.35; p = 0.009）独立相关，并且优于ATS/ERS/JRS/ALAT 2018基于指南的分类。结论：我们证明，估计确定的UIP的可能性与无移植生存和12个月的疾病进展密切相关。
{"title":"Likelihood of Usual Interstitial Pneumonia: A Novel Approach to Prognosis for Pulmonary Fibrosis.","authors":"John A Mackintosh, Simon L F Walsh, Mario Silva, Lucio Calandriello, Stephen M Humphries, David A Lynch, Helen E Jo, Ian Glaspole, Christopher Grainge, Nicole Goh, Peter M A Hopkins, Yuben Moodley, Paul N Reynolds, Christopher Zappala, Gregory Keir, Wendy A Cooper, Annabelle M Mahar, Samantha Ellis, Athol U Wells, Tamera J Corte","doi":"10.1002/resp.70196","DOIUrl":"https://doi.org/10.1002/resp.70196","url":null,"abstract":"<p><strong>Background and objectives: </strong>In fibrotic lung disease, radiological features of usual interstitial pneumonia (UIP) are associated with adverse outcomes. The aim of this study was to evaluate the likelihood of definite UIP on HRCT as a determinant of outcome in patients with pulmonary fibrosis.</p><p><strong>Methods: </strong>All available HRCT from the Australian IPF Registry (AIPFR) were retrospectively reviewed by two radiologists. The radiologists were asked to assign a likelihood (%) within each of the four ATS/ERS/JRS/ALAT 2018 UIP categories, summating to 100%, enabling them to not be constrained by a single UIP category (e.g., 5% definite UIP, 95% alternative to UIP). Likelihood of UIP was defined as the percentage likelihood score for the definite UIP category. Semi-quantitative features were also estimated. Cox proportional hazards regression was performed to evaluate the prognostic utility of the likelihood scores.</p><p><strong>Results: </strong>HRCT was available for analysis in 515 patients. Median follow-up of the cohort was 4.1 years (IQR 2.0-6.2 years). Median total ILD extent was 30% (IQR20-45%), with traction bronchiectasis and honeycombing present in 96.3% and 45.4% of cases respectively, and all three variables were associated with worse transplant-free survival. A pattern of definite or probable UIP was favoured in 26.4% and 32.4% of cases respectively. Likelihood of definite UIP was independently associated with both reduced transplant-free survival (HR1.12, 95% CI 1.04-1.20; p = 0.002) and 12-month progression (HR1.19, 95% CI 1.04-1.35; p = 0.009) and was superior to the ATS/ERS/JRS/ALAT 2018 guideline-based categories for these associations.</p><p><strong>Conclusions: </strong>We demonstrate that estimation of the likelihood of definite UIP is strongly associated with transplant-free survival and 12-month disease progression.</p>","PeriodicalId":21129,"journal":{"name":"Respirology","volume":" ","pages":""},"PeriodicalIF":6.3,"publicationDate":"2026-01-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145934767","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}
Lucile Sesé, Isabella Annesi-Maesano, Pierre Antoine Juge, Sophie Mayer, Johanna Sigaux, Bruno Crestani, Luca Semerano, Boris Dessimond, Raphael Borie, Marie-Pierre Debray, Bruno Combe, Arnaud Constantin, Morgane Didier, Pierre Yves Brillet, Anna Alari, Yurdagul Uzunhan, Philippe Dieudé, Hilario Nunes
Background and objective: Rheumatoid arthritis-associated interstitial lung disease (RA-ILD) and idiopathic pulmonary fibrosis share common risk factors, including the MUC5B promoter variant (rs35705950). We examined the contribution of air pollutants to ILD risk in an RA population and assessed the modifying effect of genetic susceptibility.
Methods: This study included patients with RA who had undergone chest HRCT. MUC5B status was collected when available. Exposure levels to NO2, PM10, and PM2.5 were estimated based on residential postal code using a dispersion model. Logistic regression analysed the influence of air pollution on ILD occurrence, and the interaction between air pollution and MUC5B status was evaluated.
Results: We included 594 patients with RA (mean age: 58 ± 12 years, 71% women, 44% current or ex-smokers), of whom 227 (38%) had ILD and 292 patients were genotyped for MUC5B rs35705950. Increased long-term exposure to NO2 (OR: 1.04 [1.01-1.06]), PM2.5 (OR: 1.11 [1.10-1.24]), and PM10 (OR: 1.15 [1.05-1.26]) was associated with ILD, after adjusting for age, sex, smoking status and HRCT year. A negative interaction between PM10/PM2.5 exposure and the T allele suggested pollution's effect on ILD risk was attenuated in the presence of the minor T allele.
Conclusion: Long-term exposure to air pollution is an independent risk factor for RA-ILD and its effect may be modified according to MUC5B rs35705950 genotype.
{"title":"Effects of Air Pollution Exposure and the MUC5B rs35705950 Variant on Interstitial Lung Disease Risk in Patients With Rheumatoid Arthritis.","authors":"Lucile Sesé, Isabella Annesi-Maesano, Pierre Antoine Juge, Sophie Mayer, Johanna Sigaux, Bruno Crestani, Luca Semerano, Boris Dessimond, Raphael Borie, Marie-Pierre Debray, Bruno Combe, Arnaud Constantin, Morgane Didier, Pierre Yves Brillet, Anna Alari, Yurdagul Uzunhan, Philippe Dieudé, Hilario Nunes","doi":"10.1002/resp.70181","DOIUrl":"https://doi.org/10.1002/resp.70181","url":null,"abstract":"<p><strong>Background and objective: </strong>Rheumatoid arthritis-associated interstitial lung disease (RA-ILD) and idiopathic pulmonary fibrosis share common risk factors, including the MUC5B promoter variant (rs35705950). We examined the contribution of air pollutants to ILD risk in an RA population and assessed the modifying effect of genetic susceptibility.</p><p><strong>Methods: </strong>This study included patients with RA who had undergone chest HRCT. MUC5B status was collected when available. Exposure levels to NO<sub>2</sub>, PM<sub>10</sub>, and PM<sub>2.5</sub> were estimated based on residential postal code using a dispersion model. Logistic regression analysed the influence of air pollution on ILD occurrence, and the interaction between air pollution and MUC5B status was evaluated.</p><p><strong>Results: </strong>We included 594 patients with RA (mean age: 58 ± 12 years, 71% women, 44% current or ex-smokers), of whom 227 (38%) had ILD and 292 patients were genotyped for MUC5B rs35705950. Increased long-term exposure to NO<sub>2</sub> (OR: 1.04 [1.01-1.06]), PM<sub>2.5</sub> (OR: 1.11 [1.10-1.24]), and PM<sub>10</sub> (OR: 1.15 [1.05-1.26]) was associated with ILD, after adjusting for age, sex, smoking status and HRCT year. A negative interaction between PM<sub>10</sub>/PM<sub>2.5</sub> exposure and the T allele suggested pollution's effect on ILD risk was attenuated in the presence of the minor T allele.</p><p><strong>Conclusion: </strong>Long-term exposure to air pollution is an independent risk factor for RA-ILD and its effect may be modified according to MUC5B rs35705950 genotype.</p>","PeriodicalId":21129,"journal":{"name":"Respirology","volume":" ","pages":""},"PeriodicalIF":6.3,"publicationDate":"2026-01-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145901112","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}
Pub Date : 2026-01-01Epub Date: 2025-10-19DOI: 10.1002/resp.70148
Rosemary S C Horne
{"title":"Progress Towards Personalised Treatment of Paediatric Obstructive Sleep Apnoea.","authors":"Rosemary S C Horne","doi":"10.1002/resp.70148","DOIUrl":"10.1002/resp.70148","url":null,"abstract":"","PeriodicalId":21129,"journal":{"name":"Respirology","volume":" ","pages":"19-20"},"PeriodicalIF":6.3,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145329846","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}
Pub Date : 2026-01-01Epub Date: 2025-11-09DOI: 10.1002/resp.70150
Coline H M van Moorsel, Thijs W Hoffman
{"title":"Increasing the Yield of Genetic Diagnoses in ILD.","authors":"Coline H M van Moorsel, Thijs W Hoffman","doi":"10.1002/resp.70150","DOIUrl":"10.1002/resp.70150","url":null,"abstract":"","PeriodicalId":21129,"journal":{"name":"Respirology","volume":" ","pages":"13-14"},"PeriodicalIF":6.3,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145482875","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}
Pub Date : 2026-01-01Epub Date: 2025-11-05DOI: 10.1002/resp.70155
Shawn D Aaron
{"title":"Early Diagnosis of COPD-How Can we Do Better?","authors":"Shawn D Aaron","doi":"10.1002/resp.70155","DOIUrl":"10.1002/resp.70155","url":null,"abstract":"","PeriodicalId":21129,"journal":{"name":"Respirology","volume":" ","pages":"27-28"},"PeriodicalIF":6.3,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12783941/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145445503","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}
Pub Date : 2026-01-01Epub Date: 2025-10-07DOI: 10.1111/resp.70134
Chalerm Liwsrisakun, Warawut Chaiwong
{"title":"Re-Evaluating the Role of Inhaled Corticosteroids in COVID-19 Management.","authors":"Chalerm Liwsrisakun, Warawut Chaiwong","doi":"10.1111/resp.70134","DOIUrl":"10.1111/resp.70134","url":null,"abstract":"","PeriodicalId":21129,"journal":{"name":"Respirology","volume":" ","pages":"17-18"},"PeriodicalIF":6.3,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145239547","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}
Pub Date : 2026-01-01Epub Date: 2025-12-16DOI: 10.1002/resp.70185
Udit Chaddha, Abhinav Agrawal, Antoni Rosell, Pyng Lee, Septimiu Dan Murgu
{"title":"Response to 'A Mechanistic and Morphologic Approach to Managing Benign Central Airway Obstruction'.","authors":"Udit Chaddha, Abhinav Agrawal, Antoni Rosell, Pyng Lee, Septimiu Dan Murgu","doi":"10.1002/resp.70185","DOIUrl":"10.1002/resp.70185","url":null,"abstract":"","PeriodicalId":21129,"journal":{"name":"Respirology","volume":" ","pages":"107-108"},"PeriodicalIF":6.3,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145768990","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}
Pub Date : 2026-01-01Epub Date: 2025-10-28DOI: 10.1002/resp.70138
Joanne L Dickinson
{"title":"Broadening Our Understanding of Genetic Risk to Interstitial Lung Disease in Understudied Populations.","authors":"Joanne L Dickinson","doi":"10.1002/resp.70138","DOIUrl":"10.1002/resp.70138","url":null,"abstract":"","PeriodicalId":21129,"journal":{"name":"Respirology","volume":" ","pages":"10-12"},"PeriodicalIF":6.3,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145392621","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}
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