IF 2.3

Breathe Pub Date : 2025-06-17 eCollection Date: 2025-04-01 DOI: 10.1183/20734735.0230-2024

Diana Marangu-Boore, Jane S Lucas, Nicole Beydon

{"title":"Nasal nitric oxide measurement for the diagnosis of primary ciliary dyskinesia: summary of the European Respiratory Society technical standard.","authors":"Diana Marangu-Boore, Jane S Lucas, Nicole Beydon","doi":"10.1183/20734735.0230-2024","DOIUrl":"10.1183/20734735.0230-2024","url":null,"abstract":"Nasal nitric oxide (nNO) measurement is important in the primary ciliary dyskinesia (PCD) diagnostic pathway because levels are consistently very low in most patients. Machine type, environmental factors, respiratory manoeuvres and report interpretation are fundamental considerations when performing nNO testing. A European Respiratory Society Task Force recently published standards for testing which we summarise and discuss in this article. There are two main types of nNO machines: chemiluminescence and electrochemical analysers. Chemiluminescence analysers are highly accurate, reliable, real-time and have been validated in multicentre studies but are less portable and more expensive to purchase and maintain in comparison to electrochemical devices. Several factors may influence nNO levels and need to be addressed during patient preparation for testing. Factors including acute viral infections and nose bleeds may contribute to falsely low nNO levels, whereas high ambient NO levels may falsely increase nNO. Tidal breathing, breath-hold and exhalation against resistance are the three main respiratory manoeuvres used in nNO sampling and require a minimal, modest and high level of patient cooperation respectively. Finally, standardised reporting of nNO testing and the correct interpretation helps clinicians to formulate an appropriate clinical plan towards an accurate PCD diagnosis.","PeriodicalId":9292,"journal":{"name":"Breathe","volume":"21 2","pages":"240230"},"PeriodicalIF":2.3,"publicationDate":"2025-06-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12171850/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144315954","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}

引用次数: 0

A new era in the treatment of progressive fibrosing interstitial lung diseases. 进行性纤维化间质性肺疾病治疗的新时代。

IF 2.3

Breathe Pub Date : 2025-06-17 eCollection Date: 2025-04-01 DOI: 10.1183/20734735.0259-2024

Anna Denis, Panagiota Tsiri, Julien Guiot, Argyris Tzouvelekis

{"title":"A new era in the treatment of progressive fibrosing interstitial lung diseases.","authors":"Anna Denis, Panagiota Tsiri, Julien Guiot, Argyris Tzouvelekis","doi":"10.1183/20734735.0259-2024","DOIUrl":"10.1183/20734735.0259-2024","url":null,"abstract":"Idiopathic pulmonary fibrosis (IPF) and progressive pulmonary fibrosis (PPF) are characterised by an irreversible progression of pulmonary fibrosis and functional lung decline. Current antifibrotic therapies (nintedanib and pirfenidone for IPF and nintedanib for PPF) can reduce disease progression but not halt or reverse it. PPF and IPF share common pathophysiological pathways that need to be further elucidated for the development of novel therapeutic strategies. The educational aim of this review is to explain the pathogenic pathways that have led to the discovery of new therapeutic agents and their favourable implementation in phase 2 and 3 studies. This includes phosphodiesterase 4 inhibitors, αvβ6 and αvβ1 integrin inhibitors, lymphosphatidic acid antagonists, inhaled treprostinil, hedgehog inhibitors, tyrosine kinase inhibitors and angiotensin type 2 receptor agonists. The aim is also to better understand current therapeutic challenges and future perspectives, including cellular therapies, exosomes and their cargoes, as well as the integration of transcriptomics and proteomics, plus gene therapy.","PeriodicalId":9292,"journal":{"name":"Breathe","volume":"21 2","pages":"240259"},"PeriodicalIF":2.3,"publicationDate":"2025-06-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12171857/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144315948","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}

引用次数: 0

An unprecedented pathology in a 20-year-old with progressive dyspnoea and parenchymal infiltrates. 一个20岁的患者出现了前所未有的病理进展性呼吸困难和实质浸润。

IF 2.3

Breathe Pub Date : 2025-06-17 eCollection Date: 2025-04-01 DOI: 10.1183/20734735.0040-2025

Ali Al-Mukhaizeem, Katherine O'Reilly, Paul Reddy, Sean Gaine, Brian McCullagh, Sarah Cullivan

引用次数: 0

Systemic autoimmune rheumatic diseases-associated interstitial lung disease: a pulmonologist's perspective. 系统性自身免疫性风湿病相关间质性肺疾病：肺科医生的观点

IF 2.3

Breathe Pub Date : 2025-06-17 eCollection Date: 2025-04-01 DOI: 10.1183/20734735.0171-2024

Niamh Boyle, Jonathan Miller, Sean Quinn, Jess Maguire, Aurelie Fabre, Kathleen Morrisroe, David J Murphy, Cormac McCarthy

{"title":"Systemic autoimmune rheumatic diseases-associated interstitial lung disease: a pulmonologist's perspective.","authors":"Niamh Boyle, Jonathan Miller, Sean Quinn, Jess Maguire, Aurelie Fabre, Kathleen Morrisroe, David J Murphy, Cormac McCarthy","doi":"10.1183/20734735.0171-2024","DOIUrl":"10.1183/20734735.0171-2024","url":null,"abstract":"Connective tissue disease (CTD)-associated interstitial lung disease (ILD) is a complex condition arising in various autoimmune disorders, such as systemic sclerosis, Sjögren disease, systemic lupus erythematosus and idiopathic inflammatory myopathies. The broader term of systemic autoimmune rheumatic diseases (SARDs) and SARD-ILD are increasingly adopted in various guidelines to allow inclusion of other rheumatic diseases such as rheumatoid arthritis. SARD-ILD significantly impacts morbidity and mortality, with disease manifestations ranging from mild to severe and life-threatening. Epidemiological data show varying ILD prevalence rates amongst SARDs, with fibrosis being a key pathological component secondary to immune-mediated inflammation and tissue remodelling. SARD-ILD presents diverse histological patterns, primarily nonspecific interstitial pneumonia and usual interstitial pneumonia, each informing prognosis and guiding therapeutic strategies. Diagnosis relies on a comprehensive evaluation of clinical, serological, radiological and histological data, involving a multidisciplinary team. Immunosuppressive therapy is the cornerstone of treatment, with concurrent use of anti-fibrotic agents in specific progressive cases. Disease management is stratified by severity, with distinct guidelines for stable, progressive and rapidly progressive ILD. The prognosis varies across SARD-ILD types, influenced by specific markers, imaging features, and response to therapy. In severe cases, lung transplantation may be considered. Early recognition remains critical in optimising outcomes for SARD-ILD patients.","PeriodicalId":9292,"journal":{"name":"Breathe","volume":"21 2","pages":"240171"},"PeriodicalIF":2.3,"publicationDate":"2025-06-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12171859/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144315955","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}

引用次数: 0

Summary for clinicians: ERS guidelines on pulmonary alveolar proteinosis. 临床总结：肺泡蛋白沉积症的ERS指南。

IF 2.3

Breathe Pub Date : 2025-05-13 eCollection Date: 2025-04-01 DOI: 10.1183/20734735.0224-2024

Tiago Alfaro, Cormac McCarthy, Francesco Bonella, Elisabeth Bendstrup, Marissa O'Callaghan

引用次数: 0

Lung transplantation for interstitial lung disease. 肺移植治疗间质性肺疾病。

IF 2.3

Breathe Pub Date : 2025-05-13 eCollection Date: 2025-04-01 DOI: 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":"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 versus 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.","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}

引用次数: 0

Our journey with pulmonary fibrosis: from challenges to change. 我们的肺纤维化之旅：从挑战到改变。

IF 2.3

Breathe Pub Date : 2025-05-13 eCollection Date: 2025-04-01 DOI: 10.1183/20734735.0008-2025

Clive Green, Sue Green, Lucy Robinson

引用次数: 0

How to move towards more sustainable asthma care in Europe: an expert opinion paper. 如何在欧洲实现更可持续的哮喘护理：一份专家意见文件。

IF 2.3

Breathe Pub Date : 2025-05-13 eCollection Date: 2025-04-01 DOI: 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

引用次数: 0

An update on diagnosis and treatments of childhood interstitial lung diseases. 儿童间质性肺疾病的诊断和治疗进展。

IF 2.3

Breathe Pub Date : 2025-05-13 eCollection Date: 2025-04-01 DOI: 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":"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.","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}

引用次数: 0

Insights into interstitial lung disease pathogenesis. 对间质性肺疾病发病机制的认识。

IF 2.3

Breathe Pub Date : 2025-05-13 eCollection Date: 2025-04-01 DOI: 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":"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.","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}

引用次数: 0

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