Respiratory Medicine and Research最新文献

{"title":"Burden of unexplained dyspnea: a single-center study","authors":"Anissa Reguig ,&nbsp;Clémence Remy ,&nbsp;Lidwine Wemeau ,&nbsp;David Montaigne ,&nbsp;Thierry Perez ,&nbsp;Cécile Chenivesse ,&nbsp;Nathalie Bautin","doi":"10.1016/j.resmer.2025.101234","DOIUrl":"10.1016/j.resmer.2025.101234","url":null,"abstract":"<div><div>Unexplained dyspnea refers to unpleasant breathing sensations without any disorder being diagnosed. We aimed to study the burden of unexplained dyspnea, by assessing health-related quality of life (SF-36 questionnaire), characteristics of dyspnea (mMRC, multidimensional dyspnea profile (MDP) and Borg score at exercise), hyperventilation symptoms (Nijmegen questionnaire), anxiety and depression (HAD scale), exercise capacity (peak V̇O2) and ventilatory response to exercise. Of the 62 patients referred to our Dyspnea center, we included 29 patients with confirmed unexplained dyspnea after a complete systematic evaluation (a cause of dyspnea was found in 29 patients and 4 cases were excluded for missing values)<em>.</em> They were aged 57 years [47;66], including 20 (69 %) women. All SF-36 scores were low: physical functioning 50 [33;75], limitation due to physical health 50 [0;63], limitation due to emotional problems 67 [0;100], fatigue 30 [15;43], emotional well-being 52 [44;66], social functioning 63 [38;75], bodily pain 45 [23;58], general health 50 [30;55]. Most patients (23 (79 %)) had activity-limiting dyspnea (mMRC≥2), 15 (55 %) had a low exercise capacity (peakV’O2&lt;85 %) and 20 (74 %) had either exertional hyperventilation (threshold V̇E/V̇CO2&gt;34) or low Vt expansion (peak Vt/FVC&lt;40 %). Hyperventilation symptoms (Nijmegen&gt;23) were present in 17 (59 %) patients, and associated with lower scores in some domains of the SF-36 questionnaire and higher HAD and MDP anxiety subscores. Our results suggest a heavy burden of unexplained dyspnea on physical and mental health. Expert centers may help in the diagnosis and thus enable earlier symptomatic management.</div></div>","PeriodicalId":48479,"journal":{"name":"Respiratory Medicine and Research","volume":"89 ","pages":"Article 101234"},"PeriodicalIF":1.8,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145829019","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Apical and basolateral alpha-1 antitrypsin delivery modulate CFTR and STAT3 in human airway epithelia grown at air-liquid interface","authors":"Kokilavani Sivaraman ,&nbsp;Stephanie Tamm ,&nbsp;Elena Korenbaum ,&nbsp;Beata Olejnicka ,&nbsp;Wenzhang Si ,&nbsp;Sabine Wrenger ,&nbsp;Frauke Stanke ,&nbsp;Sabina Janciauskiene","doi":"10.1016/j.resmer.2025.101236","DOIUrl":"10.1016/j.resmer.2025.101236","url":null,"abstract":"","PeriodicalId":48479,"journal":{"name":"Respiratory Medicine and Research","volume":"89 ","pages":"Article 101236"},"PeriodicalIF":1.8,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145921100","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"An original story of a coma","authors":"Malo Miquel ,&nbsp;Cécile Devloo ,&nbsp;Alexandre Vasseur ,&nbsp;Jéromine Alessandri ,&nbsp;Jean-Philippe Rigaud ,&nbsp;Pierre-Louis Declercq","doi":"10.1016/j.resmer.2025.101241","DOIUrl":"10.1016/j.resmer.2025.101241","url":null,"abstract":"","PeriodicalId":48479,"journal":{"name":"Respiratory Medicine and Research","volume":"89 ","pages":"Article 101241"},"PeriodicalIF":1.8,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145883320","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The preponderance of genetic variations in paediatric pulmonary hypertension","authors":"Julien Grynblat ,&nbsp;Mélanie Eyries ,&nbsp;Marine Ambar-Akkaoui ,&nbsp;Marilyne Levy ,&nbsp;Mathilde Meot ,&nbsp;Isabelle Szezepanski ,&nbsp;Julien Ranchoup ,&nbsp;Alessia Callegari ,&nbsp;Julie Karila-Cohen ,&nbsp;Caroline Bonnet ,&nbsp;Pierre Marijon ,&nbsp;Jerome Champ ,&nbsp;Florence Coulet ,&nbsp;Caroline Ovaert ,&nbsp;Frédéric Perros ,&nbsp;Fabrice Antigny ,&nbsp;Pascale Maragnes ,&nbsp;Guy Vaksmann ,&nbsp;Marc Humbert ,&nbsp;Sophie Guiti Malekzadeh Milani ,&nbsp;Damien Bonnet","doi":"10.1016/j.resmer.2026.101277","DOIUrl":"10.1016/j.resmer.2026.101277","url":null,"abstract":"<div><h3>Objectives</h3><div>To determine the prevalence and phenotypes of children with heritable pulmonary arterial hypertension (PAH) and those with pulmonary hypertension (PH) associated with genetic disorders.</div></div><div><h3>Methods</h3><div>We retrospectively analysed 133 paediatric cases with PAH, with a median age of 6 years.</div></div><div><h3>Results</h3><div>83 patients had isolated PAH, 41 had associated congenital heart disease, and 9 had suspected pulmonary veno occlusive disease. The female/male sex ratio was 1.8/1. Affected genes were <em>BMPR2</em> [13]<em>, SOX17</em> [10], <em>TBX4</em> [8], <em>ACVRL1</em> [9], <em>EIF2AK4</em> [4], <em>GDF2</em> [2], <em>BMP10</em> [1], and <em>KCNK3</em> [1]. <em>SOX17</em> variants were more common in children with APAH-CHD (p = 0.01). Additionally, 18% of patients had genetic disorders associated with PH, including Chuvash syndrome (n = 2), incontinentia pigmenti (n = 1), 5 patients with RASopathy (3 Noonan syndromes and 2 neurofibromatosis type 1), Cantu syndrome (n = 1), Alagille syndrome (n = 1), Bourneville tuberous sclerosis (n = 1), mitochondrial diseases (n = 5), Myhre syndrome (n = 1), and chromosomal rearrangements (n = 6). Overall, a significant proportion of patients had either pathogenic variants in known PAH genes or genetic disorders complicated by pulmonary vascular disease.</div></div><div><h3>Conclusion</h3><div>The prevalence of genetic disorders and heritable PAH in children appears to be higher than previously reported, affecting over half of the paediatric population. This study emphasizes the importance of systematic genetic testing, including next-generation sequencing panels, in apparently idiopathic PAH and newly diagnosed PH patients. Larger genomic sequencing may be necessary to identify novel genes associated with these conditions when initial testing is negative.</div></div>","PeriodicalId":48479,"journal":{"name":"Respiratory Medicine and Research","volume":"89 ","pages":"Article 101277"},"PeriodicalIF":1.8,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147876068","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Diffuse infiltrative lymphocytic syndrome in recently diagnosed HIV patient: a case-report DILS-ILD responsive to steroids","authors":"Nicolas Duhamel ,&nbsp;Pierre-Yves Jeandel ,&nbsp;Marie Ottavi ,&nbsp;Giorgio Toni ,&nbsp;Julie Merindol ,&nbsp;Michael Levraut","doi":"10.1016/j.resmer.2025.101223","DOIUrl":"10.1016/j.resmer.2025.101223","url":null,"abstract":"","PeriodicalId":48479,"journal":{"name":"Respiratory Medicine and Research","volume":"89 ","pages":"Article 101223"},"PeriodicalIF":1.8,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145521165","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Real-world outcomes of patients treated with ≥17 cycles of pembrolizumab monotherapy as first-line therapy and for previously treated advanced NSCLC: multicenter observational study in France","authors":"Maurice Pérol ,&nbsp;Clarisse Audigier-Valette ,&nbsp;Hervé Léna ,&nbsp;Marie-Ange Massiani ,&nbsp;Xavier Quantin ,&nbsp;Melissa Santorelli ,&nbsp;Marion Apert ,&nbsp;Axelle Spampinato ,&nbsp;Thomas Burke ,&nbsp;Christos Chouaid","doi":"10.1016/j.resmer.2025.101238","DOIUrl":"10.1016/j.resmer.2025.101238","url":null,"abstract":"","PeriodicalId":48479,"journal":{"name":"Respiratory Medicine and Research","volume":"89 ","pages":"Article 101238"},"PeriodicalIF":1.8,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145866034","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Vibrating mesh nebulization increases tidal volume relative to jet nebulization during NIV in COPD exacerbation","authors":"César Cinesi-Gomez,&nbsp;María Garcia-Palacios,&nbsp;Lorena Bernabe-Vera,&nbsp;Mabel Coromoto Suarez-Pineda,&nbsp;Elisa Serrano-Carrasco,&nbsp;Jose Andres Sanchez-Nicolas","doi":"10.1016/j.resmer.2026.101248","DOIUrl":"10.1016/j.resmer.2026.101248","url":null,"abstract":"<div><h3>Background and importance</h3><div>Noninvasive ventilation (NIV) is the first-line treatment for acute exacerbations of chronic obstructive pulmonary disease (COPD) with hypercapnic respiratory failure (HRF). Bronchodilator therapy is essential during NIV, but real-world clinical comparisons of nebulization devices in this acute setting remain limited.</div></div><div><h3>Objective</h3><div>To compare the clinical efficacy of vibrating mesh nebulizers (VMN) versus jet nebulizers (JN) in COPD patients undergoing NIV, using changes in tidal volume (VT) as the primary outcome.</div></div><div><h3>Design, settings and participants</h3><div>A longitudinal observational study was performed in the ventilatory unit of the emergency department of a tertiary hospital. Patients requiring NIV for HRF were allocated to two cohorts: VMN (October 2023–March 2024) and JN (January 2015–May 2017). Ventilatory parameters, including VT, were recorded at baseline, 15 minutes, 1 hour, and 3 hours. The primary outcome was the percentage change in VT (ΔVT) from baseline.</div></div><div><h3>Main results</h3><div>Eighty-four patients were analyzed (VMN: n=41; JN: n=43). Baseline characteristics were comparable between groups. The VMN cohort exhibited a significantly greater increase in VT at 1 hour and 3 hours (p&lt;0.05) compared with the JN cohort. ΔVT mirrored these findings. No significant correlation was observed between VT and pressure support levels.</div></div><div><h3>Conclusion</h3><div>In this real-world ED study of COPD patients treated with continuous NIV, VMN was associated with a greater early increase in VT than JN, consistent with more effective bronchodilator delivery. Given the historical, non-randomized design across different time periods and ventilators, and the use of VT as an indirect endpoint, these findings should be interpreted as hypothesis-generating but provide a clinically relevant starting point supporting preferential VMN use in acute practice.</div></div>","PeriodicalId":48479,"journal":{"name":"Respiratory Medicine and Research","volume":"89 ","pages":"Article 101248"},"PeriodicalIF":1.8,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146077230","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Impact of a pulmonary rehabilitation maintenance program on long-term healthcare resource utilization and costs in COPD patients: ancillary analysis from a real-life post-rehabilitation cohort","authors":"Fares Gouzi ,&nbsp;Thomas Espie ,&nbsp;Steven Lopes ,&nbsp;Hélène Forthin ,&nbsp;Elise Galmes ,&nbsp;Léo Blervaque ,&nbsp;Christian Préfaut ,&nbsp;François Carbonnel","doi":"10.1016/j.resmer.2025.101243","DOIUrl":"10.1016/j.resmer.2025.101243","url":null,"abstract":"<div><h3>Rational</h3><div>Chronic Obstructive Pulmonary Disease (COPD) is a major cause of healthcare system use and related cost. While pulmonary rehabilitation (PR) is efficient, maintenance programs (PR-MA) can maintain the benefits over time. Yet, the long-term impact (&gt;36 months) of PR-MA on healthcare resource and cost has never been assessed. Recently, a PR-MA program based on self-help associations has shown a clinical efficacy beyond 36 months, Thus, we aimed to assess the effect of this PR-MA program on long-term hospitalizations and costs versus usual care (PR-UC).</div></div><div><h3>Methods</h3><div>We performed an ancillary analysis of the post-rehabilitation LTAir+R cohort study, which compared a PR-MA group (n=144) to a matched PR-UC group (n=137) of COPD patients. Data were collected in 82 PR-MA and 93 PR-UC patients, from the Montpellier University Hospital database and patient records over 60 months, including hospitalizations, consultations, emergency visits, and associated costs.</div></div><div><h3>Results</h3><div>In PR-AM vs. PR-UC group, the hospitalization probability reduction almost reached significance (hazard ratio: 1.68; p=0.05). The costs of each hospitalization day (867±116 vs. 1213±138 euros/day; p&lt;0.05), emergency visits (8±5 euros/year vs. 12±3 euros/year) and medical consultations (110±3 vs. 174±3 euros/year; p&lt;0.01) were significantly lower in the PR-AM vs. PR-UC group. Last, PR-MA mitigated the increase in total hospitalizations (p&lt;0.001) associated with the follow-up duration.</div></div><div><h3>Conclusion</h3><div>In addition to its long-term clinical efficacy, this PR-MA program showed a positive impact on hospitalization and healthcare use costs. The effect on hospitalization number and costs could be larger in patients with the longest PR-MA adherence.</div></div>","PeriodicalId":48479,"journal":{"name":"Respiratory Medicine and Research","volume":"89 ","pages":"Article 101243"},"PeriodicalIF":1.8,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145834963","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Second-line treatment after first-line osimertinib without a targetable resistance mechanism: what remains standard?","authors":"Julien Ancel,&nbsp;Simon Baldacci","doi":"10.1016/j.resmer.2026.101273","DOIUrl":"10.1016/j.resmer.2026.101273","url":null,"abstract":"","PeriodicalId":48479,"journal":{"name":"Respiratory Medicine and Research","volume":"89 ","pages":"Article 101273"},"PeriodicalIF":1.8,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147802691","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Peridiaphragmatic inflammation and fibrosis in myositis associated interstitial lung disease; a case series","authors":"Joseph B. Pryor ,&nbsp;Joshua J. Solomon ,&nbsp;Jeffrey J. Swigris ,&nbsp;Tami J. Bang ,&nbsp;Andrea Fuentealba ,&nbsp;Rebecca Keith ,&nbsp;Michael P. Mohning ,&nbsp;Matthew Koslow ,&nbsp;Evans R. Fernández Pérez ,&nbsp;Tristan J. Huie ,&nbsp;Joseph Cooley ,&nbsp;Cori Fratelli ,&nbsp;David A. Lynch ,&nbsp;Zulma X. Yunt ,&nbsp;Liudmila Kastsianok","doi":"10.1016/j.resmer.2025.101219","DOIUrl":"10.1016/j.resmer.2025.101219","url":null,"abstract":"<div><h3>Introduction</h3><div>Peridiaphragmatic inflammation and fibrosis (PDIF) is a radiologic feature in myositis-associated interstitial lung disease (M-ILD). This study aims to formally define PDIF as seen on high-resolution computed tomography (HRCT) and evaluate its association with clinical and serologic features of M-ILD.</div></div><div><h3>Methods</h3><div>We conducted a retrospective review of 46 patients diagnosed with M-ILD at National Jewish Health, who were evaluated for the presence of PDIF on HRCT scans. Three thoracic radiologists developed the criteria, and two independently assessed 169 longitudinal CT scans from the 46 patients for predefined imaging criteria, including PDIF. Demographic, clinical, serologic, and pulmonary function test data were collected and analyzed.</div></div><div><h3>Results</h3><div>PDIF was identified in 28.2 % (13/46) of patients and in 18.9 % (32/169) of the reviewed scans. Among those with PDIF, 69.2 % had this finding on their initial HRCT scan prior to starting therapy, and it persisted in subsequent scans. PDIF was associated with stable radiologic pattern over time. Patients with PDIF had a high prevalence of anti-Jo antibodies (69 %) and anti-Ro52 antibodies (61.5 %). Despite ongoing treatment, all patients with PDIF experienced dyspnea, and 8/13 had concurrent myositis. There was no significant difference in forced vital capacity (FVC) or diffusing capacity for carbon monoxide (DLCO) trends over time between patients with and without PDIF.</div></div><div><h3>Conclusion</h3><div>PDIF is a relatively common finding in M-ILD, and appears to be particularly associated with anti-synthetase syndrome. Further research is needed to determine the sensitivity, specificity, and clinical implications of PDIF across all inflammatory ILDs. Early recognition of PDIF may aid in the diagnosis and management of M-ILD, but does not appear to be associated with disease progression.</div></div>","PeriodicalId":48479,"journal":{"name":"Respiratory Medicine and Research","volume":"89 ","pages":"Article 101219"},"PeriodicalIF":1.8,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145597947","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}