José María Hernández-Pérez, Silvia Castillo-Corullón, Ilaria Ferrarotti, Beatriz Martínez-Delgado, Francisco Casas-Maldonado, Francisco José Dasí-Fernández
{"title":"Pushing the Boundaries of Knowledge in Alpha-1 Antitrypsin Deficiency.","authors":"José María Hernández-Pérez, Silvia Castillo-Corullón, Ilaria Ferrarotti, Beatriz Martínez-Delgado, Francisco Casas-Maldonado, Francisco José Dasí-Fernández","doi":"10.1016/j.arbres.2026.09.002","DOIUrl":"https://doi.org/10.1016/j.arbres.2026.09.002","url":null,"abstract":"<p><p>Alpha-1 antitrypsin deficiency (AATD) is a rare inherited disorder caused by mutations in the SERPINA1 gene. AATD is one of the leading causes of emphysema in young adults and of liver cirrhosis in children and adults. Despite significant underdiagnosis and high clinical variability, recent research is rapidly transforming the field. Advances in molecular biology and translational medicine have identified new biomarkers and therapeutic targets, although reliable predictors of disease progression remain limited. While augmentation therapy is the only approved pharmacological option for pulmonary involvement and no specific treatment exists for liver disease, innovative strategies are remodeling the therapeutic landscape. Gene therapy, RNA interference, gene editing, and epigenetic modulation are showing promising preclinical and early clinical results, aiming to correct the underlying defect rather than manage symptoms. This review focuses on these emerging advances, highlighting cutting-edge preclinical and translational developments with potential to redefine AATD management and move toward curative approaches.</p>","PeriodicalId":8339,"journal":{"name":"Archivos De Bronconeumologia","volume":" ","pages":""},"PeriodicalIF":8.7,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149103889","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}
Adrià Moncusí, Andrea Burón, Gabriela Lugon, Francesc Macià, Maria Olivia Cabrera-Godoy, Josep Alfons Espinàs, Roberto Chalela, Mercè Marzo-Castillejo, José Maria Maiques, Maria Sala, Carlijn M. van der Aalst, Xavier Castells
{"title":"Risk Perception and Intention to Quit Smoking Among High-Risk Participants Enrolled in a European Lung Cancer Screening Trial in Spain","authors":"Adrià Moncusí, Andrea Burón, Gabriela Lugon, Francesc Macià, Maria Olivia Cabrera-Godoy, Josep Alfons Espinàs, Roberto Chalela, Mercè Marzo-Castillejo, José Maria Maiques, Maria Sala, Carlijn M. van der Aalst, Xavier Castells","doi":"10.1016/j.arbres.2026.04.013","DOIUrl":"10.1016/j.arbres.2026.04.013","url":null,"abstract":"","PeriodicalId":8339,"journal":{"name":"Archivos De Bronconeumologia","volume":"62 10","pages":"Pages 740-743"},"PeriodicalIF":8.7,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147863453","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}
Paola Faverio, Davide Gandola, Marco Mura, Sara Conti, Umberto Zanini, Sergio Harari, Antonella Caminati, Francesco Bini, Michele Mondoni, Jacopo Cefalo, Giovanni Franco, Alessia Fumagalli, Lorenzo Giovanni Mantovani, Fabrizio Luppi, Davide Ippolito, Cesare Maino
{"title":"Identification of a Cut-Off Value to Identify Sarcopenia Through Pectoralis Muscle Area in Fibrotic ILDs: An International Study","authors":"Paola Faverio, Davide Gandola, Marco Mura, Sara Conti, Umberto Zanini, Sergio Harari, Antonella Caminati, Francesco Bini, Michele Mondoni, Jacopo Cefalo, Giovanni Franco, Alessia Fumagalli, Lorenzo Giovanni Mantovani, Fabrizio Luppi, Davide Ippolito, Cesare Maino","doi":"10.1016/j.arbres.2026.03.011","DOIUrl":"10.1016/j.arbres.2026.03.011","url":null,"abstract":"","PeriodicalId":8339,"journal":{"name":"Archivos De Bronconeumologia","volume":"62 10","pages":"Pages 727-730"},"PeriodicalIF":8.7,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147833081","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}
Oriol Sibila, Lidia Perea, Alvar Agustí, Rosa Faner
{"title":"The Potential Role of DPP-1 Inhibitors in Chronic Obstructive Pulmonary Disease","authors":"Oriol Sibila, Lidia Perea, Alvar Agustí, Rosa Faner","doi":"10.1016/j.arbres.2026.06.008","DOIUrl":"10.1016/j.arbres.2026.06.008","url":null,"abstract":"","PeriodicalId":8339,"journal":{"name":"Archivos De Bronconeumologia","volume":"62 10","pages":"Pages 689-691"},"PeriodicalIF":8.7,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148403505","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":"New Perspectives in the Treatment of Bronchiectasis","authors":"Angela Tramontano, Mariagiovanna Caporaso, Giulia Macciocchi, Edoardo Simonetta, Mattia Nigro, Stefano Aliberti","doi":"10.1016/j.arbres.2025.12.008","DOIUrl":"10.1016/j.arbres.2025.12.008","url":null,"abstract":"<div><div>Bronchiectasis is a chronic, heterogeneous respiratory disease. Its pathogenesis involves airway inflammation, chronic infection, impaired mucociliary clearance, and progressive lung damage. Despite recent approval of Brensocatib, current management relies mostly on off-label therapies, highlighting the need for evidence-based strategies.</div><div>Given the heterogeneity of bronchiectasis, precision medicine approaches that integrate patient phenotypes, endotypes, and disease severity are crucial. Mapping the current landscape of randomized clinical trials (RCTs) highlights the efforts to improve patient outcomes and translate mechanistic insights into clinical practice. This review underscores the shift toward individualized, mechanism-based therapy in bronchiectasis management providing a comprehensive overview of current and emerging therapeutic approaches in bronchiectasis, focusing on ongoing and recent RCTs.</div></div>","PeriodicalId":8339,"journal":{"name":"Archivos De Bronconeumologia","volume":"62 10","pages":"Pages 717-726"},"PeriodicalIF":8.7,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146091842","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}
Alba Mulet, Jaime Signes-Costa, Estrella Fernández-Fabrellas, Jose A. Ros, Juan A. Rodríguez-Portal, Ada L. Andreu, Federico Pallardó, Pilar González-Cabo
{"title":"Mitochondrial Dysfunction and Telomeric Shortening as Long-term Complications After COVID-19","authors":"Alba Mulet, Jaime Signes-Costa, Estrella Fernández-Fabrellas, Jose A. Ros, Juan A. Rodríguez-Portal, Ada L. Andreu, Federico Pallardó, Pilar González-Cabo","doi":"10.1016/j.arbres.2026.03.018","DOIUrl":"10.1016/j.arbres.2026.03.018","url":null,"abstract":"<div><h3>Objectives</h3><div>To evaluate the potential role of telomere shortening and mitochondrial dysfunction in the development of long-term complications of COVID-19, including pulmonary fibrosis.</div></div><div><h3>Methods</h3><div>We analyzed 132 patients from the prospective COVID-FIBROTIC cohort 1 year after hospitalization for bilateral pneumonia. Leukocyte telomere length was measured and compared with that of 78 age- and sex-matched controls. Fibrosis biomarkers and radiological findings were assessed at 2 and 12 months. In a subgroup of 44 patients, mitochondrial protein expression was evaluated 2 years after infection using reverse-phase protein arrays. Associations among telomere length, mitochondrial proteins, inflammatory markers, and fibrotic sequelae were analyzed.</div></div><div><h3>Results</h3><div>Leukocyte telomere length was significantly shorter in patients than in controls (AUC, 0.84; <em>P</em> <!--><<!--> <!-->.0001), independent of age or acute disease severity. At 12 months, 29% of patients showed fibrotic lung changes on HRCT. Elevated periostin and IL-6 levels correlated with persistent mitochondrial protein alterations at 2 years. Mitochondrial proteins such as ETFβ and PKM2 differentiated patients with fibrotic sequelae and those with marked telomere attrition, suggesting their role as biomarkers. Moreover, in patients with fibrosis, telomere shortening correlated inversely with ACO1 levels, a protein involved in oxidative stress and iron metabolism.</div></div><div><h3>Conclusions</h3><div>SARS-CoV-2 infection induces sustained telomere shortening and long-term mitochondrial dysfunction, both of which are associated with fibrotic sequelae. These findings support a pathogenic link among mitochondrial dysregulation, telomere attrition, and pulmonary fibrosis, resembling mechanisms described in idiopathic pulmonary fibrosis. Monitoring these biomarkers may help identify patients at risk of chronic post-COVID complications.</div></div>","PeriodicalId":8339,"journal":{"name":"Archivos De Bronconeumologia","volume":"62 10","pages":"Pages 701-708"},"PeriodicalIF":8.7,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148315334","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}