BreathePub Date : 2025-05-13eCollection Date: 2025-04-01DOI: 10.1183/20734735.0224-2024
Tiago Alfaro, Cormac McCarthy, Francesco Bonella, Elisabeth Bendstrup, Marissa O'Callaghan
{"title":"Summary for clinicians: ERS guidelines on pulmonary alveolar proteinosis.","authors":"Tiago Alfaro, Cormac McCarthy, Francesco Bonella, Elisabeth Bendstrup, Marissa O'Callaghan","doi":"10.1183/20734735.0224-2024","DOIUrl":"10.1183/20734735.0224-2024","url":null,"abstract":"<p><p>Pulmonary alveolar proteinosis (PAP) is a rare lung disease caused by accumulation of surfactant in the alveoli, leading to debilitating respiratory symptoms and impaired gas exchange. The recent European Respiratory Society guidelines provide evidence-based recommendations for its diagnosis and management. Autoimmune PAP (aPAP) is the most common form, driven by granulocyte-macrophage colony-stimulating factor (GM-CSF) autoantibodies. Recommended diagnostic tools include bronchoalveolar lavage and quantitative GM-CSF antibody testing. Whole lung lavage and inhaled GM-CSF are first-line treatments for symptomatic or progressive aPAP. Rituximab, plasmapheresis, and lung transplantation are options for refractory disease. Referral to expert centres is advised for diagnostic and therapeutic guidance. This case-based summary for clinicians highlights the best clinical approach to patients with suspicion or confirmation of PAP.</p>","PeriodicalId":9292,"journal":{"name":"Breathe","volume":"21 2","pages":"240224"},"PeriodicalIF":2.3,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12070199/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143970529","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}
BreathePub Date : 2025-05-13eCollection Date: 2025-04-01DOI: 10.1183/20734735.0169-2024
Nicola J Ronan, Feargal Helly, Michelle A Murray
{"title":"Lung transplantation for interstitial lung disease.","authors":"Nicola J Ronan, Feargal Helly, Michelle A Murray","doi":"10.1183/20734735.0169-2024","DOIUrl":"10.1183/20734735.0169-2024","url":null,"abstract":"<p><p>Interstitial lung diseases (ILDs) are now the most common indication for lung transplant internationally. Given that many lung transplant candidates with idiopathic pulmonary fibrosis are older, referral to a pulmonary rehabilitation programme is important to help mitigate the adverse outcomes associated with frailty. Despite this increase many patients with ILD who would potentially benefit from lung transplant are either not referred or referred too late. Particularly relevant in ILD which may have prominent extra-pulmonary manifestations is a multidisciplinary assessment of comorbidities which may impact on post lung transplant outcomes. Particular challenges in lung transplant for ILD are increasing age, comorbidities, donor lung sizing and the risk-benefit balance of single <i>versus</i> bilateral lung transplant. Evidence is continuing to evolve for lung transplant in rarer ILDs, including surfactant protein associated ILD and TERT mutations. Unfortunately, the number of potential lung transplant recipients exceeds available donor organs and some patients will die without transplant. Palliative care is an important aspect of managing patients on an active lung transplant list to help optimise physical and psychological symptoms associated with uncertainty on an active lung transplant list.</p>","PeriodicalId":9292,"journal":{"name":"Breathe","volume":"21 2","pages":"240169"},"PeriodicalIF":2.3,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12070198/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143963877","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}
BreathePub Date : 2025-05-13eCollection Date: 2025-04-01DOI: 10.1183/20734735.0008-2025
Clive Green, Sue Green, Lucy Robinson
{"title":"Our journey with pulmonary fibrosis: from challenges to change.","authors":"Clive Green, Sue Green, Lucy Robinson","doi":"10.1183/20734735.0008-2025","DOIUrl":"https://doi.org/10.1183/20734735.0008-2025","url":null,"abstract":"<p><p><b>Clive and Sue's story highlights the importance of support, education and research in managing RA-ILD. Their journey shows how community and awareness can improve quality of life and help create better treatment options for future patients.</b> https://bit.ly/4jqdsJN.</p>","PeriodicalId":9292,"journal":{"name":"Breathe","volume":"21 2","pages":"250008"},"PeriodicalIF":2.3,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12070200/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143975860","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}
BreathePub Date : 2025-05-13eCollection Date: 2025-04-01DOI: 10.1183/20734735.0229-2024
Christer Janson, Alex Wilkinson, Hanna Hisinger-Mölkänen, Bernardino Alcázar Navarrete, Kai-Michael Beeh, Federico Lavorini, Hannu Kankaanranta, John Pritchard, Charlotte Suppli Ulrik, Aarti Bansal, Satu Lähelmä, Lauri Lehtimäki
{"title":"How to move towards more sustainable asthma care in Europe: an expert opinion paper.","authors":"Christer Janson, Alex Wilkinson, Hanna Hisinger-Mölkänen, Bernardino Alcázar Navarrete, Kai-Michael Beeh, Federico Lavorini, Hannu Kankaanranta, John Pritchard, Charlotte Suppli Ulrik, Aarti Bansal, Satu Lähelmä, Lauri Lehtimäki","doi":"10.1183/20734735.0229-2024","DOIUrl":"10.1183/20734735.0229-2024","url":null,"abstract":"<p><p><b>The healthcare sector is one of the primary emitters of greenhouse gases in the public sector; this viewpoint's distillation of expert opinions emphasises what more sustainable asthma care could look like and presents possible solutions to achieving it</b> https://bit.ly/41fQ364.</p>","PeriodicalId":9292,"journal":{"name":"Breathe","volume":"21 2","pages":"240229"},"PeriodicalIF":2.3,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12070203/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143953901","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}
BreathePub Date : 2025-05-13eCollection Date: 2025-04-01DOI: 10.1183/20734735.0004-2025
Honorata Marczak, Katarzyna Krenke, Matthias Griese, Julia Carlens, Elias Seidl, Carlee Gilbert, Nagehan Emiralioglu, Alba Torrent-Vernetta, Brigitte Willemse, Ralph Epaud, Celine Delestrain, Camille Louvrier, Václav Koucký, Nadia Nathan
{"title":"An update on diagnosis and treatments of childhood interstitial lung diseases.","authors":"Honorata Marczak, Katarzyna Krenke, Matthias Griese, Julia Carlens, Elias Seidl, Carlee Gilbert, Nagehan Emiralioglu, Alba Torrent-Vernetta, Brigitte Willemse, Ralph Epaud, Celine Delestrain, Camille Louvrier, Václav Koucký, Nadia Nathan","doi":"10.1183/20734735.0004-2025","DOIUrl":"10.1183/20734735.0004-2025","url":null,"abstract":"<p><p>Childhood interstitial lung diseases (chILDs) are rare and heterogeneous disorders associated with significant morbidity and mortality. The clinical presentation of chILD typically includes chronic or recurrent respiratory signs and symptoms with diffuse radiographic abnormalities on chest imaging. Diagnosis requires a structured, multi-step approach. Treatment options are limited, with disease-specific therapies available only in selected cases and management relying primarily on supportive care. Awareness of chILDs has been steadily increasing. New diagnoses, advanced diagnostic tests, and novel treatments are emerging each year, highlighting the importance of collaborative, multidisciplinary teams in providing comprehensive care for children and families affected by these complex conditions. On behalf of the European Respiratory Society Clinical Research Collaboration for chILD (ERS CRC chILD-EU), this review provides an updated overview of the diagnostic approach and management strategies for chILDs.</p>","PeriodicalId":9292,"journal":{"name":"Breathe","volume":"21 2","pages":"250004"},"PeriodicalIF":2.3,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12070201/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143980945","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}
BreathePub Date : 2025-05-13eCollection Date: 2025-04-01DOI: 10.1183/20734735.0261-2024
Eirini Vasarmidi, Julie C Worrell, Irma Mahmutovic Persson, Naheem Yaqub, Ewa Miądlikowska, Cindy Barnig, Agnes Boots, Niki L Reynaert, Sara Cuevas Ocaña
{"title":"Insights into interstitial lung disease pathogenesis.","authors":"Eirini Vasarmidi, Julie C Worrell, Irma Mahmutovic Persson, Naheem Yaqub, Ewa Miądlikowska, Cindy Barnig, Agnes Boots, Niki L Reynaert, Sara Cuevas Ocaña","doi":"10.1183/20734735.0261-2024","DOIUrl":"10.1183/20734735.0261-2024","url":null,"abstract":"<p><p>This review summarises some of the key features of interstitial lung diseases (ILDs) from a translational science point of view and brings insights into potential therapeutic options. Genetic predisposition and environmental factors like smoking, pollution and infections significantly impact the onset, progression and treatment response in ILDs, highlighting the need for personalised management. Fibroblasts are central to ILD pathology, influencing the tissue microenvironment, immune cell interactions and extracellular matrix (ECM) production, making them critical therapeutic targets. Monocyte-derived M2 macrophages drive fibrosis in idiopathic pulmonary fibrosis by secreting cytokines and remodelling the ECM. Understanding macrophage subtypes and their dynamics offers new therapeutic possibilities. Chronic type 2 immunity contributes to fibrosis, emphasising the need to enhance protective markers in order to even out the balance shift of pathological immune responses in ILD treatments. Serum biomarkers like Krebs von den Lungen-6 (KL-6), surfactant protein (SFTP) D, matrix metalloproteinase-7 (MMP-7), and C-C motif chemokine ligand (CCL)-18 are valuable for diagnosing and predicting ILD progression, although more research is needed for clinical application. Animal models, especially bleomycin-based models, offer insights into ILD pathology, but challenges like lung hyperinflation highlight the need for careful model selection and translational research to bridge preclinical and clinical findings.</p>","PeriodicalId":9292,"journal":{"name":"Breathe","volume":"21 2","pages":"240261"},"PeriodicalIF":2.3,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12070197/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143959910","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}
BreathePub Date : 2025-05-13eCollection Date: 2025-04-01DOI: 10.1183/20734735.0236-2024
Lucía Ferreiro, María E Toubes, Nuria Rodríguez-Núñez, Luis Valdés
{"title":"Asbestos and benign pleural diseases: a narrative review.","authors":"Lucía Ferreiro, María E Toubes, Nuria Rodríguez-Núñez, Luis Valdés","doi":"10.1183/20734735.0236-2024","DOIUrl":"10.1183/20734735.0236-2024","url":null,"abstract":"<p><p>The term \"asbestos\" is used to refer to a group of silicate minerals that often break down into fibres and whose inhalation over time can cause a number of diseases, especially pleuropulmonary diseases. While the most serious complications are malignant diseases, inhalation of these fibres can also cause benign pleural diseases such as round atelectasis, pleural plaques, diffuse pleural thickening and non-malignant asbestos pleural effusion. Although asbestos is banned in most developed countries (in the European Union, since 2002), it is still used in developing countries. Despite these restrictions, the prevalence of diseases due to inhalation remains high due to the long latency period between the onset of exposure and the onset of disease. In this paper we review benign pleural diseases induced by asbestos exposure, update the diagnostic criteria for these disorders, and describe the approaches suggested so far to differentiate them from malignant pleural diseases.</p>","PeriodicalId":9292,"journal":{"name":"Breathe","volume":"21 2","pages":"240236"},"PeriodicalIF":2.3,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12070196/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143974558","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}
BreathePub Date : 2025-05-13eCollection Date: 2025-04-01DOI: 10.1183/20734735.0106-2024
Andre Gie, Ruan Swanepoel, Marieke M van der Zalm, Pierre Goussard
{"title":"The use of forced oscillation technique in children with restrictive physiology.","authors":"Andre Gie, Ruan Swanepoel, Marieke M van der Zalm, Pierre Goussard","doi":"10.1183/20734735.0106-2024","DOIUrl":"10.1183/20734735.0106-2024","url":null,"abstract":"<p><p><b>The forced oscillation technique (FOT) may not identify lung function abnormalities in children with restrictive physiology. FOT findings correlate poorly with spirometry and plethysmography testing in children with diffuse parenchymal lung disease.</b> https://bit.ly/4h4liIh.</p>","PeriodicalId":9292,"journal":{"name":"Breathe","volume":"21 2","pages":"240106"},"PeriodicalIF":2.3,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12070202/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143966267","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}
BreathePub Date : 2025-04-17eCollection Date: 2025-04-01DOI: 10.1183/20734735.0167-2024
Sheridan G Mikhail, David N O'Dwyer
{"title":"The lung microbiome in interstitial lung disease.","authors":"Sheridan G Mikhail, David N O'Dwyer","doi":"10.1183/20734735.0167-2024","DOIUrl":"https://doi.org/10.1183/20734735.0167-2024","url":null,"abstract":"<p><p>Interstitial lung disease (ILD) is a heterogeneous chronic form of lung disease. The pathogenesis of ILD is poorly understood and a common form of ILD, idiopathic pulmonary fibrosis (IPF) is associated with poor prognosis. There is evidence for substantial dysregulated immune responses in ILD. The microbiome is a key regulator of the immune response, and the lung microbiome correlates with alveolar immunity and clinical outcomes in ILD. Most observational lung microbiome studies have been conducted in patients with IPF. A consistent observation in these studies is that the bacterial burden of the lung is elevated in patients with IPF and predicts mortality. However, our understanding of the mechanism is incomplete and our understanding of the role of the lung microbiome in other forms of ILD is limited. The microbiomes of the oropharynx and gut may have implications for the lung microbiome and pulmonary immunity in ILD but require substantial further research. Here, we discuss the studies supporting a role for the lung microbiome in the pathogenesis of IPF, and briefly describe the putative role of the oral-lung axis and the gut-lung axis in ILD.</p>","PeriodicalId":9292,"journal":{"name":"Breathe","volume":"21 2","pages":"240167"},"PeriodicalIF":2.3,"publicationDate":"2025-04-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12004254/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143980368","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}