Pub Date : 2024-08-15DOI: 10.1164/rccm.202407-1352ED
Anna J Podolanczuk, Toby M Maher, Wonder P Drake, Patricia J Sime, Oliver Eickelberg, Dawn L DeMeo, Luca Richeldi
{"title":"Pioneering Research Driving Progress toward a Cure for Interstitial Lung Disease (ILD): Introduction to the <i>AJRCCM</i> Special Issue on ILD (Part 2).","authors":"Anna J Podolanczuk, Toby M Maher, Wonder P Drake, Patricia J Sime, Oliver Eickelberg, Dawn L DeMeo, Luca Richeldi","doi":"10.1164/rccm.202407-1352ED","DOIUrl":"10.1164/rccm.202407-1352ED","url":null,"abstract":"","PeriodicalId":7664,"journal":{"name":"American journal of respiratory and critical care medicine","volume":null,"pages":null},"PeriodicalIF":19.3,"publicationDate":"2024-08-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11351802/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141981502","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-08-15DOI: 10.1164/rccm.202405-1008LE
Chris Ward, Amal Alamer, Rhys Jones, Michelle Lawton, Mike Griffin, Michael Drinnan, Ian Forrest, Joanne Patterson
{"title":"Integrating Aerodigestive Investigations in Progressive Pulmonary Fibrosis.","authors":"Chris Ward, Amal Alamer, Rhys Jones, Michelle Lawton, Mike Griffin, Michael Drinnan, Ian Forrest, Joanne Patterson","doi":"10.1164/rccm.202405-1008LE","DOIUrl":"10.1164/rccm.202405-1008LE","url":null,"abstract":"","PeriodicalId":7664,"journal":{"name":"American journal of respiratory and critical care medicine","volume":null,"pages":null},"PeriodicalIF":19.3,"publicationDate":"2024-08-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11351811/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141465644","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-08-15DOI: 10.1164/rccm.202406-1123LE
David N O'Dwyer, John S Kim, Imre Noth
{"title":"Reply to Ward <i>et al.</i>: Integrating Aerodigestive Investigations in Progressive Pulmonary Fibrosis.","authors":"David N O'Dwyer, John S Kim, Imre Noth","doi":"10.1164/rccm.202406-1123LE","DOIUrl":"10.1164/rccm.202406-1123LE","url":null,"abstract":"","PeriodicalId":7664,"journal":{"name":"American journal of respiratory and critical care medicine","volume":null,"pages":null},"PeriodicalIF":19.3,"publicationDate":"2024-08-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11351800/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141465648","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-08-15DOI: 10.1164/rccm.202311-2111LE
Jonathan S Kurche, Carlyne D Cool, Rachel Z Blumhagen, Evgenia Dobrinskikh, David Heinz, Jeremy A Herrera, Ivana V Yang, David A Schwartz
{"title":"<i>MUC5B</i> Idiopathic Pulmonary Fibrosis Risk Variant Promotes a Mucosecretory Phenotype and Loss of Small Airway Secretory Cells.","authors":"Jonathan S Kurche, Carlyne D Cool, Rachel Z Blumhagen, Evgenia Dobrinskikh, David Heinz, Jeremy A Herrera, Ivana V Yang, David A Schwartz","doi":"10.1164/rccm.202311-2111LE","DOIUrl":"10.1164/rccm.202311-2111LE","url":null,"abstract":"","PeriodicalId":7664,"journal":{"name":"American journal of respiratory and critical care medicine","volume":null,"pages":null},"PeriodicalIF":19.3,"publicationDate":"2024-08-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11351810/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141454662","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-08-14DOI: 10.1164/rccm.202407-1417LE
Bo-Guen Kim, Kyungdo Han, Hyun Lee
{"title":"Reply to Yu <i>et al.</i>: Elevated Suicide Risk in Idiopathic Pulmonary Fibrosis Patients.","authors":"Bo-Guen Kim, Kyungdo Han, Hyun Lee","doi":"10.1164/rccm.202407-1417LE","DOIUrl":"https://doi.org/10.1164/rccm.202407-1417LE","url":null,"abstract":"","PeriodicalId":7664,"journal":{"name":"American journal of respiratory and critical care medicine","volume":null,"pages":null},"PeriodicalIF":19.3,"publicationDate":"2024-08-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141981500","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-08-14DOI: 10.1164/rccm.202402-0374SO
Sara C Auld, Amy K Barczak, William Bishai, Anna K Coussens, Intan M W Dewi, Steven C Mitini-Nkhoma, Caleb Muefong, Threnesan Naidoo, Anil Pooran, Cari Stek, Adrie J C Steyn, Liku Tezera, Naomi F Walker
Post-tuberculosis (TB) lung disease (PTLD) is increasingly recognized as a major contributor to the global burden of chronic lung disease, with recent estimates indicating that over half of TB survivors have impaired lung function after successful completion of TB treatment. However, the pathologic mechanisms that contribute to PTLD are not well understood, thus limiting the development of therapeutic interventions to improve long-term outcomes after TB. This report summarizes the work of the "Pathogenesis and Risk Factors Committee" for the Second International Post-Tuberculosis Symposium, which took place in Stellenbosch, South Africa in April 2023. The committee first identified six areas with high translational potential: (1) tissue matrix destruction, including the role of matrix metalloproteinase dysregulation and neutrophil activity, (2) fibroblasts and profibrotic activity, (3) granuloma fate and cell death pathways, (4) mycobacterial factors including pathogen burden, (5) animal models, and (6) the impact of key clinical risk factors including HIV, diabetes, smoking, malnutrition, and alcohol. We share here the key findings from a literature review of those areas, highlighting knowledge gaps and areas where further research is needed.
{"title":"Pathogenesis of Post-Tuberculosis Lung Disease: Defining Knowledge Gaps and Research Priorities at the 2<sup>nd</sup> International Post-Tuberculosis Symposium.","authors":"Sara C Auld, Amy K Barczak, William Bishai, Anna K Coussens, Intan M W Dewi, Steven C Mitini-Nkhoma, Caleb Muefong, Threnesan Naidoo, Anil Pooran, Cari Stek, Adrie J C Steyn, Liku Tezera, Naomi F Walker","doi":"10.1164/rccm.202402-0374SO","DOIUrl":"https://doi.org/10.1164/rccm.202402-0374SO","url":null,"abstract":"<p><p>Post-tuberculosis (TB) lung disease (PTLD) is increasingly recognized as a major contributor to the global burden of chronic lung disease, with recent estimates indicating that over half of TB survivors have impaired lung function after successful completion of TB treatment. However, the pathologic mechanisms that contribute to PTLD are not well understood, thus limiting the development of therapeutic interventions to improve long-term outcomes after TB. This report summarizes the work of the \"Pathogenesis and Risk Factors Committee\" for the Second International Post-Tuberculosis Symposium, which took place in Stellenbosch, South Africa in April 2023. The committee first identified six areas with high translational potential: (1) tissue matrix destruction, including the role of matrix metalloproteinase dysregulation and neutrophil activity, (2) fibroblasts and profibrotic activity, (3) granuloma fate and cell death pathways, (4) mycobacterial factors including pathogen burden, (5) animal models, and (6) the impact of key clinical risk factors including HIV, diabetes, smoking, malnutrition, and alcohol. We share here the key findings from a literature review of those areas, highlighting knowledge gaps and areas where further research is needed.</p>","PeriodicalId":7664,"journal":{"name":"American journal of respiratory and critical care medicine","volume":null,"pages":null},"PeriodicalIF":19.3,"publicationDate":"2024-08-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141981499","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-08-13DOI: 10.1164/rccm.202403-0524OC
Margaret L Salisbury, Cheryl Markin, Tisra Fadely, Adam R Guttentag, Stephen M Humphries, David A Lynch, Jonathan A Kropski, Timothy S Blackwell
Rationale: Relatives of patients with familial pulmonary fibrosis (FPF) are at increased risk to develop FPF. Interstitial lung abnormalities (ILAs) are a radiologic biomarker of subclinical disease, but the implications of very mild abnormalities remain unclear.
Objectives: To quantify the progression risk among FPF relatives with abnormalities below the threshold for ILAs as described by the Fleischner Society and to describe the characteristics of participants with new or progressive ILAs during observation.
Methods: Asymptomatic FPF relatives undergo serial screening high-resolution chest CT (HRCT). For this analysis, Early ILAs (no minimum threshold of lung involvement) were sub-classified as Mild (all interstitial abnormalities involve <5% of a lung zone) or Moderate (any abnormality involves >5%). Identification of new or progressive ILAs on HRCT, or development of Pulmonologist-diagnosed clinical FPF were defined as progression. Covariate-adjusted logistic regression identified progression-associated characteristics.
Measurements and main results: From 2008-2023, 273 participants in follow-up procedures were 53.2 9.4 years old at enrollment, 95 (35%) were male, and 73/268 (27%) were ever-smokers. During a mean follow-up of 6.2 3.0 years, progression occurred among 31/211 (15%) of those with absence of ILAs at enrollment, 32/49 (65%) of Mild ILAs, and 10/13 (77%) of Moderate ILAs. Mild ILAs had 9.15 (95% CI 4.40-19.00, p<0.0001) times and Moderate ILAs had 17.14 (95% CI 4.42-66.49, p<0.0001) times the odds of progression as subjects without ILAs.
Conclusions: In persons at-risk for FPF, minor interstitial abnormalities, including reticulation that is unilateral or involves <5% of a lung zone, frequently represent subclinical disease.
{"title":"Progressive Early Interstitial Lung Abnormalities in Persons At-Risk for Familial Pulmonary Fibrosis: A Prospective Cohort Study.","authors":"Margaret L Salisbury, Cheryl Markin, Tisra Fadely, Adam R Guttentag, Stephen M Humphries, David A Lynch, Jonathan A Kropski, Timothy S Blackwell","doi":"10.1164/rccm.202403-0524OC","DOIUrl":"https://doi.org/10.1164/rccm.202403-0524OC","url":null,"abstract":"<p><strong>Rationale: </strong>Relatives of patients with familial pulmonary fibrosis (FPF) are at increased risk to develop FPF. Interstitial lung abnormalities (ILAs) are a radiologic biomarker of subclinical disease, but the implications of very mild abnormalities remain unclear.</p><p><strong>Objectives: </strong>To quantify the progression risk among FPF relatives with abnormalities below the threshold for ILAs as described by the Fleischner Society and to describe the characteristics of participants with new or progressive ILAs during observation.</p><p><strong>Methods: </strong>Asymptomatic FPF relatives undergo serial screening high-resolution chest CT (HRCT). For this analysis, Early ILAs (no minimum threshold of lung involvement) were sub-classified as Mild (all interstitial abnormalities involve <5% of a lung zone) or Moderate (any abnormality involves >5%). Identification of new or progressive ILAs on HRCT, or development of Pulmonologist-diagnosed clinical FPF were defined as progression. Covariate-adjusted logistic regression identified progression-associated characteristics.</p><p><strong>Measurements and main results: </strong>From 2008-2023, 273 participants in follow-up procedures were 53.2 9.4 years old at enrollment, 95 (35%) were male, and 73/268 (27%) were ever-smokers. During a mean follow-up of 6.2 3.0 years, progression occurred among 31/211 (15%) of those with absence of ILAs at enrollment, 32/49 (65%) of Mild ILAs, and 10/13 (77%) of Moderate ILAs. Mild ILAs had 9.15 (95% CI 4.40-19.00, p<0.0001) times and Moderate ILAs had 17.14 (95% CI 4.42-66.49, p<0.0001) times the odds of progression as subjects without ILAs.</p><p><strong>Conclusions: </strong>In persons at-risk for FPF, minor interstitial abnormalities, including reticulation that is unilateral or involves <5% of a lung zone, frequently represent subclinical disease.</p>","PeriodicalId":7664,"journal":{"name":"American journal of respiratory and critical care medicine","volume":null,"pages":null},"PeriodicalIF":19.3,"publicationDate":"2024-08-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141974867","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-08-12DOI: 10.1164/rccm.202407-1380LE
Mayson L A Sousa, Bhushan H Katira, Sheena Bouch, Vanessa Hsing, Doreen Engelberts, Marcelo B P Amato, Martin Post, Laurent Brochard
{"title":"Reply to Bihari <i>et al.</i>: Alveolar Collapse Is a Threat in Injured Lungs but What About the Airway Opening Pressure.","authors":"Mayson L A Sousa, Bhushan H Katira, Sheena Bouch, Vanessa Hsing, Doreen Engelberts, Marcelo B P Amato, Martin Post, Laurent Brochard","doi":"10.1164/rccm.202407-1380LE","DOIUrl":"https://doi.org/10.1164/rccm.202407-1380LE","url":null,"abstract":"","PeriodicalId":7664,"journal":{"name":"American journal of respiratory and critical care medicine","volume":null,"pages":null},"PeriodicalIF":19.3,"publicationDate":"2024-08-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141970435","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"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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