Carlos Celis-Preciado, Simon Leclerc, Martine Duval, Dominic O Cliche, Lucie Brazeau, Félix-Antoine Vézina, Marylène Dussault, Pierre Larivée, Samuel Lemaire-Paquette, Simon Lévesque, Philippe Lachapelle, Simon Couillard
{"title":"Phenotyping the Responses to Systemic Corticosteroids in the Management of Asthma Attacks (PRISMA).","authors":"Carlos Celis-Preciado, Simon Leclerc, Martine Duval, Dominic O Cliche, Lucie Brazeau, Félix-Antoine Vézina, Marylène Dussault, Pierre Larivée, Samuel Lemaire-Paquette, Simon Lévesque, Philippe Lachapelle, Simon Couillard","doi":"10.1183/13993003.02391-2024","DOIUrl":"https://doi.org/10.1183/13993003.02391-2024","url":null,"abstract":"<p><strong>Background: </strong>Asthma attacks are heterogeneous. It is not known whether the response to oral corticosteroids (OCS) in acute asthma varies according to type-2 (T2) inflammatory biomarkers, blood eosinophil count (BEC) and exhaled nitric oxide (FeNO). We aim to explore the relationship between T2 biomarkers and response to OCS in acute asthma.</p><p><strong>Methods: </strong>We conducted a longitudinal observational study of people experiencing an asthma attack evaluated before and after a 7-day OCS course. The primary outcome was post-bronchodilator (BD) FEV<sub>1</sub> change according to ordinal BEC-FeNO 3-group categories (T2-Low/Low, BEC <0.15×10<sup>9</sup> cells·L<sup>-1</sup> and FeNO <25 ppb; T2-High/High, BEC ≥0.30×10<sup>9</sup> cells·L<sup>-1</sup> and FeNO ≥35 ppb and T2-Mid, not meeting Low/Low-High/High criteria). A key secondary outcome was the Asthma Control Questionnaire (ACQ-5) change. Exploratory outcomes included OCS-attributable adverse events.</p><p><strong>Results: </strong>Fifty-three people were enrolled with 16 (30%) T2-Low/Low, 27 (51%) T2-Mid and 10 (19%) T2-High/High asthma attacks. Post-BD FEV<sub>1</sub> changes increased with combined BEC-FeNO elevation (p-for-interaction=0.007), peaking in the T2-High/High phenotype (0.390±0.512L, p-for-trend<0.0001). Conversely, T2-Low/Low attacked achieved nonsignificant FEV<sub>1</sub> changes (0.017±0.153L). In univariable and multivariable analyses, only ordinal BEC-FeNO stratification - not symptoms nor FEV<sub>1</sub> - was a predictor of subsequent post-BD FEV<sub>1</sub> improvement. All patients improved ACQ-5, numerically peaking in the T2-High/High phenotype (-1.58±0.60, p-for-trend=0.08). All groups experienced similar OCS-attributable adverse events, with n=33 (62%) participants reporting ≥1 event.</p><p><strong>Conclusions: </strong>We found that objective improvement following OCS is confined to T2-High/High events. As in chronic asthma, greater T2 burden identifies a distinct clinical and therapeutic trajectory, whereas OCS-related adverse events are uniformly distributed.</p>","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":" ","pages":""},"PeriodicalIF":16.6,"publicationDate":"2025-02-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143413866","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}
Andrei M Darie, Leticia Grize, Kathleen Jahn, Anna Salina, Jonathan Röcken, Matthias J Herrmann, Maria Pascarella, Vivian Suarez, Werner Strobel, Michael Tamm, Daiana Stolz
{"title":"Preventing oxygen desaturation during bronchoscopy in COPD patients using high flow oxygen <i>versus</i> standard management: the randomised controlled PROSA 2 Trial.","authors":"Andrei M Darie, Leticia Grize, Kathleen Jahn, Anna Salina, Jonathan Röcken, Matthias J Herrmann, Maria Pascarella, Vivian Suarez, Werner Strobel, Michael Tamm, Daiana Stolz","doi":"10.1183/13993003.01586-2024","DOIUrl":"https://doi.org/10.1183/13993003.01586-2024","url":null,"abstract":"<p><strong>Background: </strong>Patients with chronic obstructive pulmonary disease (COPD) are at increased risk for developing additional respiratory comorbidities associated with smoking, and are thus prone to undergo flexible bronchoscopy. However, COPD patients have increased periprocedural complications risk and lower oxygen saturation during bronchoscopy.</p><p><strong>Methods: </strong>This was an investigator-initiated, single centre, open-label randomised controlled trial designed to assess the benefits of high flow nasal oxygen compared to conventional low flow oxygen by nasal cannula during conscious sedation for bronchoscopy in patients with COPD. Low flow was supplied at a starting rate of 4 L / min and gradually increased up to 12 L / min to maintain the oxygen saturation (SpO2) above 90%. High flow delivered using LM Flow 100 (Löwenstein Medical GmbH, Bad Ems, Germany) starting at a rate of 60 L / min and an inspired fraction of oxygen (FiO2) of 0.6 was increased up to 80 L / min to preserve the SpO2 above 90%. The primary endpoint was cumulative hypoxaemia time.</p><p><strong>Results: </strong>We randomised 600 COPD cases with a median age of 69.0 (62.0-76.0) years to either high flow (295) or low flow (305). The cumulative hypoxaemia time was 53% lower in the high flow group (1.8% [95%CI 1.5-2.2] <i>versus</i> 3.8% [95%CI 3.2-4.5] of monitoring time, p<0.001). Additionally, the high flow group experienced 3.0 (1.0-6.0) hypoxaemia events (SpO2<90%) as compared to 6.0 (3.0-10.0) in the low flow group (p<0.001). The low flow group had five-fold higher odds of experiencing hypoxaemia during bronchoscopy (OR 5.1 [95%CI 3.2-8.2], p<0.001).</p><p><strong>Conclusion: </strong>High flow is feasible, decreases cumulative hypoxaemia time and reduces hypoxaemia events during bronchoscopy in patients with COPD but does not impact patient comfort.</p><p><strong>Clinical trial registration: </strong>This trial was registered on the International Clinical Trials Registry Platform, ISRCTN18159882.</p>","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":" ","pages":""},"PeriodicalIF":16.6,"publicationDate":"2025-02-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143413870","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}
Andrey V Zinchuk, Clete A Kushida, Alexander Walker, Andrew Wellman, Ali Azarbarzin, Raichel M Alex, Andrew W Varga, Scott A Sands, H Klar Yaggi
{"title":"Arousal threshold modifies the effect of CPAP on executive function among individuals with obstructive sleep apnoea.","authors":"Andrey V Zinchuk, Clete A Kushida, Alexander Walker, Andrew Wellman, Ali Azarbarzin, Raichel M Alex, Andrew W Varga, Scott A Sands, H Klar Yaggi","doi":"10.1183/13993003.01183-2024","DOIUrl":"10.1183/13993003.01183-2024","url":null,"abstract":"<p><strong>Background: </strong>Obstructive sleep apnoea (OSA) is associated with neurocognitive dysfunction. However, randomised trials evaluating the effects of continuous positive airway pressure (CPAP) on neurocognition in those without dementia do not show a benefit. We thus aimed to assess whether arousal threshold (ArTH) modifies the effect of CPAP on neurocognitive function.</p><p><strong>Methods: </strong>We performed a secondary analysis of a randomised, sham-controlled trial (Apnea Positive Pressure Long-term Efficacy Study (APPLES); ClinicalTrials.gov: NCT00051363). ArTH was estimated from polysomnography using a translatable method. Neurocognitive outcomes included the Sustained Working Memory Test-Overall Mid-Day (SWMT-OMD) score (executive function, primary outcome), with the Pathfinder Number Test total time (attention) and Buschke Selective Reminding Test sum recall (learning and memory) as secondary outcomes. Generalised linear modelling assessed whether the effect of CPAP was modified by baseline ArTH (treatment×ArTH interaction). 833 participants with OSA (apnoea-hypopnoea index ≥10 events·h<sup>-1</sup>), available ArTH and outcomes were analysed (active CPAP n=437 and sham CPAP n=396).</p><p><strong>Results: </strong>For executive function, the effect of CPAP treatment was modified by ArTH (p<sub>interaction</sub>=0.042). Specifically, for every 1sd increase in ArTH, the SWMT-OMD score improved by 0.091 (95% CI 0.003-0.178) in active compared to sham CPAP at 6 months; at ArTH 1sd above the mean, SWMT-OMD improvements were nearly three times that in those with average ArTH (0.139 (95% CI 0.018-0.261) <i>versus</i> 0.053 (95% CI -0.034-0.140), respectively). No effect modification was observed for attention (p=0.311) or learning and memory (p=0.744).</p><p><strong>Conclusion: </strong>In OSA, a higher ArTH is associated with greater improvements in executive function following CPAP therapy.</p>","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":" ","pages":""},"PeriodicalIF":16.6,"publicationDate":"2025-02-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142686627","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}
Julien Guiot, Béatrice André, Judith Potjewijd, Pierre Jacquerie, Sébastien Cremers, Monique Henket, Latifa Idoufkir, Claire Remacle, Rachid Tobal, Laurie Giltay, Catherine Moermans, Fanny Gester, Barbara Polese, Malik Hamaïdia, Ingrid Struman, Edouard Louis, Michel Malaise, Dominique de Seny, Pieter van Paassen, Renaud Louis, Clio Ribbens, Makon-Sébastien Njock
{"title":"Association of fibrotic-related extracellular vesicle microRNAs with lung involvement in systemic sclerosis.","authors":"Julien Guiot, Béatrice André, Judith Potjewijd, Pierre Jacquerie, Sébastien Cremers, Monique Henket, Latifa Idoufkir, Claire Remacle, Rachid Tobal, Laurie Giltay, Catherine Moermans, Fanny Gester, Barbara Polese, Malik Hamaïdia, Ingrid Struman, Edouard Louis, Michel Malaise, Dominique de Seny, Pieter van Paassen, Renaud Louis, Clio Ribbens, Makon-Sébastien Njock","doi":"10.1183/13993003.00276-2024","DOIUrl":"https://doi.org/10.1183/13993003.00276-2024","url":null,"abstract":"<p><strong>Background: </strong>There is a pressing need to identify early biomarkers of lung involvement in systemic sclerosis (SSc) to start as soon as possible antifibrotic therapy. We aimed to identify extracellular vesicle-derived microRNAs (EV-miRNAs) that are differentially expressed between SSc patients with and without interstitial lung disease (ILD), explore their diagnostic value and investigate their functional properties.</p><p><strong>Methods: </strong>Small EVs (sEVs) derived from plasma were isolated from 91 well-characterised SSc patients with ILD (SSc-ILD, n=45), without ILD (SSc-no ILD, n=46) and 43 matched healthy subjects (HS). Small RNA sequencing followed by quantitative RT-PCR were used to identify and validate sEV-miRNAs associated to SSc-ILD. Correlations between SSc-ILD-associated miRNAs and clinical parameters were assessed, as well as the impact of related miRNAs/sEVs on fibrosis.</p><p><strong>Results: </strong>We identified a 4-miRNA signature associated with ILD in SSc context (miR-584-5p, miR-744-5p, miR-1307-3p and miR-10b-5p) (ROC AUC=0.85, 95% CI 0.76-0.94, p<0.0001). Deeper analysis revealed a correlation of these candidates with pulmonary function tests (DLCO and FVC), highlighting their capacity to monitor lung fibrosis progression in SSc patients. Furthermore, SSc-ILD-associated sEV miRNAs are positively correlated and enriched in circulating lymphocytes, suggesting that these immune cells are their cellular source. Finally, functional studies highlighted an alteration of functional properties of sEVs in SSc-ILD context mainly due to the transfer of profibrotic miR-584-5p in lung fibroblasts.</p><p><strong>Conclusions: </strong>Our sEV-based biomarker approach enabled to identify a promising 4-miRNA signature characteristic of ILD in SSc patients. Furthermore, the profibrotic properties of SSc-ILD-associated sEVs suggest a prominent role of these vesicles on SSc severity.</p>","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":" ","pages":""},"PeriodicalIF":16.6,"publicationDate":"2025-02-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143413864","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}
Neil Martin, Michael E Wechsler, Christopher E Brightling
{"title":"Reply to: Response and remission in asthma with tezepelumab: overlapping concepts informing on type-2 inflammatory-dependent treatment effects.","authors":"Neil Martin, Michael E Wechsler, Christopher E Brightling","doi":"10.1183/13993003.02434-2024","DOIUrl":"10.1183/13993003.02434-2024","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"65 2","pages":""},"PeriodicalIF":16.6,"publicationDate":"2025-02-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11822240/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143413880","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}
Nicola Andrea Marchi, Sébastien Bailly, Indu Ayappa, Raphaël Heinzer
{"title":"Arousal threshold and the effect of CPAP on neurocognitive function: a new step towards a precision medicine approach for treating obstructive sleep apnoea.","authors":"Nicola Andrea Marchi, Sébastien Bailly, Indu Ayappa, Raphaël Heinzer","doi":"10.1183/13993003.02383-2024","DOIUrl":"https://doi.org/10.1183/13993003.02383-2024","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"65 2","pages":""},"PeriodicalIF":16.6,"publicationDate":"2025-02-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143413874","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}
Bryce N Balmain, Andrew R Tomlinson, Josh T Goh, James P MacNamara, Denis J Wakeham, Tiffany L Brazile, Michael G Leahy, Kevin C Lutz, Linda S Hynan, Benjamin D Levine, Satyam Sarma, Tony G Babb
{"title":"Pulmonary gas exchange in relation to exercise pulmonary hypertension in patients with heart failure with preserved ejection fraction.","authors":"Bryce N Balmain, Andrew R Tomlinson, Josh T Goh, James P MacNamara, Denis J Wakeham, Tiffany L Brazile, Michael G Leahy, Kevin C Lutz, Linda S Hynan, Benjamin D Levine, Satyam Sarma, Tony G Babb","doi":"10.1183/13993003.00722-2024","DOIUrl":"10.1183/13993003.00722-2024","url":null,"abstract":"<p><strong>Background: </strong>Exercise pulmonary hypertension, defined as a mean pulmonary arterial pressure (mPAP)/cardiac output (<i>Q̇c</i>) slope >3 WU during exercise, is common in patients with heart failure with preserved ejection fraction (HFpEF). However, the pulmonary gas exchange-related effects of an exaggerated exercise pulmonary hypertension (EePH) response are not well defined, especially in relation to dyspnoea on exertion and exercise intolerance.</p><p><strong>Methods: </strong>48 HFpEF patients underwent invasive (pulmonary and radial artery catheters) constant-load (20 W) and maximal incremental cycle testing. Haemodynamic measurements (mPAP and <i>Q̇c</i>), arterial blood and expired gases, and ratings of perceived breathlessness (Borg 0-10 scale) were obtained. The mPAP/<i>Q̇c</i> slope was calculated from rest to 20 W. Those with a mPAP/<i>Q̇c</i> slope ≥4.2 (median) were classified as HFpEF+EePH (n=24) and those with a mPAP/<i>Q̇c</i> slope <4.2 were classified as HFpEF (without EePH) (n=24). The alveolar-arterial oxygen tension difference, dead space to tidal volume ratio (Bohr equation) and the minute ventilation to carbon dioxide production slope (from rest to 20 W) were calculated.</p><p><strong>Results: </strong>Arterial oxygen tension was lower (p=0.03) and dead space to tidal volume ratio was higher (p=0.03) at peak exercise in HFpEF+EePH than in HFpEF. The alveolar-arterial oxygen tension difference was similar at peak exercise between groups (p=0.14); however, patients with HFpEF+EePH achieved the peak alveolar-arterial oxygen tension difference at a lower peak work rate (p<0.01). The minute ventilation to carbon dioxide production slope was higher in HFpEF+EePH than in HFpEF (p=0.01). Perceived breathlessness was ≥1 unit higher at 20 W and peak oxygen uptake was lower (p<0.01) in HFpEF+EePH than in HFpEF.</p><p><strong>Conclusions: </strong>These data suggest that EePH contributes to pulmonary gas exchange impairments during exercise by causing a ventilation/perfusion mismatch that provokes both ventilatory inefficiency and hypoxaemia, both of which seem to contribute to dyspnoea on exertion and exercise intolerance in patients with HFpEF.</p>","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":" ","pages":""},"PeriodicalIF":16.6,"publicationDate":"2025-02-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142603762","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}
Effrosyni D Manali, Matthias Griese, Nadia Nathan, Yurdagül Uzunhan, Raphael Borie, Katarzyna Michel, Nicolaus Schwerk, Justyna Fijolek, Elżbieta Radzikowska, Felix Chua, Rishi Pabary, Nesrin Mogulkoc, Cormac McCarthy, Maria Kallieri, Andriana I Papaioannou, Nural Kiper, Martina Koziar Vasakova, Ladislav Lacina, Maria Molina-Molina, Alba Torrent-Vernetta, Theofanis Tsiligiannis, Bulent Karadag, Maria Kokosi, Elisabetta A Renzoni, Coline H M van Moorsel, Ilaria Campo, Elisabeth Bendstrup, Thomas Skovhus Prior, Antje Prasse, Francesco Bonella, Vincent Cottin, Rémi Diesler, Antoine Froidure, Lykourgos Kolilekas, Lampros Fotis, Konstantinos Douros, Athanasios G Kaditis, Florence Jeny, Simon Chauveau, Hilario Nunes, Azrine Dahbia, Francesca Mariani, Joanne J van der Vis, Karlijn Groen, Ela Erdem Eralp, Yasemin Gokdemir, Derya Kocakaya, Sehnaz Olgun Yildizeli, Ebru Yalçın, Nagehan Emiralioğlu, Halime Nayir Buyuksahin, Helen O'Brien, Oguz Karcıoglu, Demet Can, Alper Ezircan, Gokcen Kartal Ozturk, Nesrin Ocal, Hasan Yuksel, Sedef Narin Tongal, Martina Safrankova, Katerina Kourtesi, Camille Louvrier, Caroline Kannengiesser, Aurelie Fabre, Marie Legendre, Bruno Crestani, Petr Pohunek, Andrew Bush, Spyros A Papiris
{"title":"Childhood interstitial lung disease survivors in adulthood: a European collaborative study.","authors":"Effrosyni D Manali, Matthias Griese, Nadia Nathan, Yurdagül Uzunhan, Raphael Borie, Katarzyna Michel, Nicolaus Schwerk, Justyna Fijolek, Elżbieta Radzikowska, Felix Chua, Rishi Pabary, Nesrin Mogulkoc, Cormac McCarthy, Maria Kallieri, Andriana I Papaioannou, Nural Kiper, Martina Koziar Vasakova, Ladislav Lacina, Maria Molina-Molina, Alba Torrent-Vernetta, Theofanis Tsiligiannis, Bulent Karadag, Maria Kokosi, Elisabetta A Renzoni, Coline H M van Moorsel, Ilaria Campo, Elisabeth Bendstrup, Thomas Skovhus Prior, Antje Prasse, Francesco Bonella, Vincent Cottin, Rémi Diesler, Antoine Froidure, Lykourgos Kolilekas, Lampros Fotis, Konstantinos Douros, Athanasios G Kaditis, Florence Jeny, Simon Chauveau, Hilario Nunes, Azrine Dahbia, Francesca Mariani, Joanne J van der Vis, Karlijn Groen, Ela Erdem Eralp, Yasemin Gokdemir, Derya Kocakaya, Sehnaz Olgun Yildizeli, Ebru Yalçın, Nagehan Emiralioğlu, Halime Nayir Buyuksahin, Helen O'Brien, Oguz Karcıoglu, Demet Can, Alper Ezircan, Gokcen Kartal Ozturk, Nesrin Ocal, Hasan Yuksel, Sedef Narin Tongal, Martina Safrankova, Katerina Kourtesi, Camille Louvrier, Caroline Kannengiesser, Aurelie Fabre, Marie Legendre, Bruno Crestani, Petr Pohunek, Andrew Bush, Spyros A Papiris","doi":"10.1183/13993003.00680-2024","DOIUrl":"10.1183/13993003.00680-2024","url":null,"abstract":"<p><strong>Background: </strong>Interstitial lung disease is rarer in children than adults, but, with increasing diagnostic awareness, more cases are being discovered. The prognosis of childhood interstitial lung disease is often poor, but increasing numbers are now surviving into adulthood.</p><p><strong>Aim: </strong>To characterise childhood interstitial lung disease survivors and identify their impact on adult interstitial lung disease centres.</p><p><strong>Methods: </strong>This was a European study (34 adult and childhood interstitial lung disease centres) reporting incident/prevalent cases of childhood interstitial lung disease survivors from January to July 2023. Epidemiological, clinical, physiological and genetic data were collected.</p><p><strong>Results: </strong>244 patients were identified with a median (interquartile range) age at diagnosis of 12.5 years (6-16 years) and age at study inclusion of 25 years (22-33 years), with 51% male, 86% nonsmokers and a median (interquartile range) % predicted forced vital capacity of 70% (47-89%) and diffusing capacity of the lungs for carbon monoxide of 48% (32-75%). 32% were prescribed long-term oxygen and 227 (93%) were followed up in adult centres whereas 17 (7%) never transitioned. The commonest diagnoses (82%) were childhood interstitial lung disease category B1 (sarcoidosis, hemosiderosis, connective tissue disorders, vasculitis) at 35%, A4 (surfactant-related) at 21%, B2 (bronchiolitis obliterans, hypersensitivity pneumonitis) at 14% and Bz (unclassified interstitial lung disease) at 13%. Bz patients had the worst functional status. 60% of all patients were still being prescribed corticosteroids. Re-specification of diagnosis and treatment were made after transition for 9.8% and 16% of patients, respectively. Not all childhood interstitial lung disease diagnoses were recognised in adult interstitial lung disease classifications.</p><p><strong>Conclusion: </strong>Childhood interstitial lung disease survivors are seen in most adult interstitial lung disease centres and only a minority continue follow-up in paediatric centres. Survivors have a significant loss of lung function. The heterogeneity of their aetiologies and therapeutic requirements has a real impact on adult interstitial lung disease centres. Re-specification of diagnosis and treatment may contribute to precision and personalisation of management.</p>","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":" ","pages":""},"PeriodicalIF":16.6,"publicationDate":"2025-02-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142785027","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}
{"title":"Heart or lungs? Why not both!","authors":"Vishal N Rao, Brian A Houston, Peter J Leary","doi":"10.1183/13993003.02308-2024","DOIUrl":"https://doi.org/10.1183/13993003.02308-2024","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"65 2","pages":""},"PeriodicalIF":16.6,"publicationDate":"2025-02-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143413877","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}
{"title":"How bad is your cough? The McMaster Cough Severity Questionnaire as a new tool to measure chronic cough.","authors":"Richard D Turner, Surinder S Birring","doi":"10.1183/13993003.02289-2024","DOIUrl":"https://doi.org/10.1183/13993003.02289-2024","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"65 2","pages":""},"PeriodicalIF":16.6,"publicationDate":"2025-02-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143364063","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}