Respiration最新文献

{"title":"Sarcoidosis and Systemic Comorbidities: Sex-Related Differences in a Multicenter Italian Cohort.","authors":"Tommaso Pianigiani, Giulio Natalello, Elisa Bianchi, Bianca Boncompagni, Gabriela Marlene Morales Blanco, Ferdinando Galizia, Akter Dilroba, Martina Meocci, Sofia Marrucci, Matteo Peruzzi, Laura Bergantini, Sara Lonni, Sara Solveig Fois, Giovanni Franco, Lorena Gallotti, Alessandro Giuseppe Fois, Fabrizio Luppi, Anna Agnese Stanziola, Fabiano Di Marco, Paolo Cameli, Elena Bargagli","doi":"10.1159/000553468","DOIUrl":"10.1159/000553468","url":null,"abstract":"<p><strong>Introduction: </strong>Sarcoidosis is a systemic granulomatous disease characterized by heterogeneous clinical presentation and variable organ involvement. Beyond pulmonary manifestations, extrapulmonary disease and associated comorbidities substantially contribute to disease burden and clinical complexity. Although sex-related differences in sarcoidosis phenotype have been reported, data on sex-specific patterns of systemic comorbidities remain limited.</p><p><strong>Methods: </strong>We retrospectively analyzed 1,313 patients with sarcoidosis followed at five Italian referral centers. Systemic comorbidities, disease phenotype, and treatment exposure were systematically recorded and analyzed according to biological sex. Organ involvement was assessed using the WASOG Sarcoidosis Organ Assessment Instrument.</p><p><strong>Results: </strong>Female patients showed a significantly higher prevalence of autoimmune, bone, and psychiatric comorbidities, as well as a greater burden of extrapulmonary sarcoidosis, particularly cutaneous and ocular involvement. Extrapulmonary disease was associated with increased systemic comorbidity burden. Malignancy occurred within the broader context of systemic comorbidities, was more frequent among female patients, and was not associated with treatment exposure.</p><p><strong>Conclusion: </strong>Sarcoidosis is characterized by sex-specific patterns of systemic comorbidities and disease expression that extend beyond pulmonary involvement. These findings support the relevance of sex-aware clinical assessment and highlight the need for prospective studies to better define comorbidity trajectories and inform personalized management strategies.</p>","PeriodicalId":21048,"journal":{"name":"Respiration","volume":" ","pages":"1-4"},"PeriodicalIF":2.6,"publicationDate":"2026-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148423655","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":"AWMF S2k Guideline: Diagnosis and Assessment of Lung Disease Caused by Occupational Silica Dust Exposure (Silicosis) - Disease No. 4101 as per Occupational Diseases Ordinance (BKV) Annex 1.","authors":"Alexandra Marita Preisser, Christian Eisenhawer, Luminița I Cerviș, Andreas Deimling, Melanie Duell, Inke Sabine Feder, Bernhard Heise, Karina Hofmann-Preiß, Danny David Jonigk, Thomas Kraus, Kathrin Ludwig, Dennis Nowak, Andrea Tannapfel, Fred Dieter Zagrodnik, Heinrich Worth","doi":"10.1159/000553350","DOIUrl":"10.1159/000553350","url":null,"abstract":"<p><p>This manuscript is a translation of the corresponding German Guideline \"S2k-Leitlinie Diagnostik und Begutachtung der Berufskrankheit Nr. 4101 Quarzstaublungenerkrankung (Silikose) der Berufskrankheitenverordnung (BKV).\" The guideline provides a structured framework for the expert assessment of quartz dust-related lung disease (Occupational Disease No. 4101, German Ordinance on Occupational Diseases, BKV). It targets medical assessment experts who are responsible for determining the presence of silicosis and related conditions, assessing their causal relationship to occupational quartz dust exposure, and evaluating functional consequences including loss or reduction of earning capacity (MdE). In addition, it offers guidance for clerks of statutory accident insurance institutions as well as social courts and aimed to improve the plausibility, internal consistency, and transparency of expert reports and resulting decisions for affected individuals. The guideline specifies required investigations and their evaluation, emphasizing that symptomatology, cardiopulmonary functional, and conventional chest radiography display rather low levels of correlation. It details radiological and pathological manifestations of silicosis on qualified low-dose volumetric HRCT, including typical findings in lung parenchyma, hilum, mediastinum, and lymph nodes, with notes on standards, limitations, and diagnostic pitfalls.</p>","PeriodicalId":21048,"journal":{"name":"Respiration","volume":" ","pages":"1-33"},"PeriodicalIF":2.6,"publicationDate":"2026-07-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13577655/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148405577","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}

{"title":"Real-World Long-Term Efficacy of Chemoimmunotherapy in Advanced Stage Small-Cell Lung Cancer.","authors":"Arne Peeters, Els Wauters, Isabelle Wauters, Karolien Van Hove, Christophe Dooms","doi":"10.1159/000553352","DOIUrl":"10.1159/000553352","url":null,"abstract":"<p><strong>Introduction: </strong>Since IMpower133 and CASPIAN phase 3 trials, chemoimmunotherapy has become the first-line systemic treatment for advanced-stage small-cell lung carcinoma (SCLC). We conducted a study on the long-term efficacy.</p><p><strong>Methods: </strong>A retrospective cohort study was performed on advanced-stage SCLC patients who initiated treatment with first-line carboplatin-etoposide-atezolizumab between January 2018 and December 2022 at the University Hospitals Leuven. Follow-up data were collected until February 2025. The Kaplan-Meier method was used for estimating survival outcomes, and Cox regression models were used to assess the association between baseline clinical characteristics (age, ECOG Performance Status, metastatic sites, s-albumin, s-lactate dehydrogenase, s-C-reactive protein, s-NSE, neutrophil-lymphocyte ratio [NLR], modified Glasgow Prognostic Score [mGPS], and Lung Immune Prognostic Index [LIPI] score) and survival outcomes.</p><p><strong>Results: </strong>A total of 74 patients were included. The median progression-free survival (PFS) was 4.8 months (95% CI: 4.5-5.0), and median overall survival (OS) was 11.5 months (95% CI: 8.9-14.6). The 1-, 2-, and 5-year PFS and OS rates were 16.2% (95% CI: 8.9-25.5), 9.5% (95% CI: 4.2-17.4), and 8.1% (95% CI: 3.3-15.7) and 47.3% (95% CI: 35.6-58.1), 18.9% (95% CI: 11.0-28.5), and 8.9% (95% CI: 3.2-18.2%), respectively. In multivariable analysis, bone metastasis (HR 2.21; 95% CI: 1.26-3.87), age (+1 year, HR 1.03; 95% CI: 1.00-1.07), s-NSE >250 μg/L (HR 4.55; 95% CI: 2.13-9.72), and s-albumin ≥35 g/dL (HR 0.49; 95% CI: 0.25-0.99) were significantly associated with OS. During induction phase, chemotherapy dose reduction and discontinuation occurred in 31% and 8%, respectively. Overall, immune-related adverse events occurred in 21 patients (28%), leading to discontinuation of atezolizumab in 7 patients (10%).</p><p><strong>Conclusion: </strong>Our study provides long-term safety and efficacy outcomes of first-line chemoimmunotherapy in a real-world cohort of advanced-stage SCLC. In multivariable analysis, we found that baseline conventional biomarkers (age, bone metastasis, s-albumin, and s-NSE) did significantly predict OS, while systemic inflammation scores (NLR, mGPS, and LIPI) did not.</p>","PeriodicalId":21048,"journal":{"name":"Respiration","volume":" ","pages":"1-13"},"PeriodicalIF":2.6,"publicationDate":"2026-07-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148376777","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":"Residual Apnoea-Hypopnoea Index Measurement from Positive Airway Pressure Devices versus Home Polygraphy: A Method Comparison Study Using Real-World Data.","authors":"Stephanie K Mansell, Sharon Augustt, Francesca Gowing, Deborah Ridout, Stephen Hilton, Cherry Kilbride, Eleanor Main, Silvia Schievano, Swapna Mandal","doi":"10.1159/000553192","DOIUrl":"10.1159/000553192","url":null,"abstract":"<p><strong>Introduction: </strong>Residual apnoea-hypopnoea index (AHI) derived from positive airway pressure (PAP) device downloads is frequently used to assess treatment effectiveness in obstructive sleep apnoea (OSA). However, the accuracy of device reported AHI in real-world settings remains uncertain. This study evaluated the agreement between PAP device-derived AHI and AHI measured using home-based cardiorespiratory polygraphy.</p><p><strong>Methods: </strong>This secondary analysis of the 3DPiPPIn randomised controlled trial included adults with OSA (AHI ≥15 events/h) naïve to domiciliary PAP therapy. Participants underwent two-night home cardiorespiratory polygraphy at 3 and 6 months. Residual AHI was obtained from Löwenstein PAP devices via data download. Agreement between methods was assessed using Bland-Altman analysis, intraclass correlation coefficients (ICCs), and weighted Kappa. Sensitivity and specificity for detecting inadequate control (AHI ≥7 events/h) were calculated. The association between average leak and AHI discrepancy was examined using linear regression.</p><p><strong>Results: </strong>Ninety-eight participants were included. PAP-derived AHI values consistently underestimated polygraphy-derived AHI across all time points. Agreement was poor, with ICCs of 0.02 at 3 months and 0.16 at 6 months and low diagnostic agreement across severity categories at both 3 months (_K0.10, 95% CI: 0 to 0.26) and 6 months (_K0.09, 95% CI: 0 to 0.28). Bland-Altman plots demonstrated systematic underestimation with wide limits of agreement. Sensitivity for detecting inadequate control was very low at both time points (3 months 12% and 6 months 21%), despite high specificity (97% at 3 months and 95% at 6 months). Higher interface leak was significantly associated with greater underestimation of AHI (3 months: slope = 0.45, R2 = 0.18, p < 0.0001; 6 months: slope = 0.38, R2 = 0.14, p < 0.0001).</p><p><strong>Conclusion: </strong>PAP device downloads substantially underestimated residual AHI and demonstrated poor agreement with home cardiorespiratory polygraphy. These findings highlight important limitations of device-derived AHI in real-world practice, particularly in the presence of interface leak. Where treatment adequacy is uncertain, confirmatory assessment with polygraphy remains necessary.</p>","PeriodicalId":21048,"journal":{"name":"Respiration","volume":" ","pages":"1-11"},"PeriodicalIF":2.6,"publicationDate":"2026-06-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13472600/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148332262","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}

{"title":"Unveiling Sex Differences in Severe Acute Chronic Obstructive Pulmonary Disease Exacerbations Requiring Intensive Care Unit Admission: Evidence from a French Cohort.","authors":"Alexis Ferré, Nassim Sahki, Sylvain Diop, Pauline Puechoultres, Georges Abi-Abdallah, Stéphane Legriel","doi":"10.1159/000552665","DOIUrl":"10.1159/000552665","url":null,"abstract":"<p><strong>Introduction: </strong>Data on sex differences during acute exacerbations of chronic obstructive pulmonary disease (COPD) management and mortality in the intensive care unit (ICU) are scarce. This study aimed to describe and compare sex differences in management and their impact on mortality.</p><p><strong>Methods: </strong>We conducted a monocentric retrospective cohort study on all patients admitted to our ICU between 2015 and 2022 for a severe acute exacerbation of COPD. Logistic multivariate regression analysis was performed.</p><p><strong>Results: </strong>A total of 508 patients were included, of whom 331 (65.2%) were males and 177 (34.8%) were females. Female patients had a higher proportion of severe COPD (GOLD stage ≥ 3) than males (59.3% vs. 54.4%, p = 0.032), whereas males exhibited significantly more cardiovascular comorbidities. Three-month mortality was 19.3% in males and 18.6% in females (p = 0.84). In multivariate analysis, factors independently associated with 3-month mortality were older age (hazard ratio [HR] per year = 1.04, p = 0.001), immunodeficiency (HR = 1.66, p = 0.022), higher performance status (HR per point = 1.68, p < 0.001), long-term oxygen therapy (HR = 1.80, p = 0.006), and invasive mechanical ventilation (HR = 2.59, 95% CI: 1.34-5.01, p = 0.05). Sex was not associated with 3-month mortality (p = 0.5).</p><p><strong>Conclusion: </strong>Despite distinct phenotypes between males and females, management and outcomes of severe COPD exacerbations in ICU were similar, underscoring the need to better understand sex-related determinants in critical COPD care.</p>","PeriodicalId":21048,"journal":{"name":"Respiration","volume":" ","pages":"1-13"},"PeriodicalIF":2.6,"publicationDate":"2026-06-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148332357","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":"Intrapleural Fibrinolytics Use in Children with Pleural Empyema: Safe and Effective.","authors":"Carmen C M de Jong, Ilia E Heijting, Peter J F M Merkus, Jolt Roukema, Lara S van den Wijngaart, Milanthy S Pourier","doi":"10.1159/000553095","DOIUrl":"10.1159/000553095","url":null,"abstract":"<p><strong>Introduction: </strong>We assessed the number of children with pleural empyema in which video-assisted thoracoscopy (VATS) could be avoided in a center where standard therapy is drainage using a chest drain with/without intrapleural fibrinolytics.</p><p><strong>Methods: </strong>We included all children referred for chest drainage of a pleural effusion to Radboud University Medical Center between January 2022 and April 2023. Children were referred in case of pneumonia with respiratory distress and suspected pleural effusion on the chest X-ray or an effusion size of >20 mm in the chest ultrasound, indicating the need for chest drainage or VATS. We analyzed symptoms, test results, and treatment.</p><p><strong>Results: </strong>Thirty-two children aged 0-16 years were referred for treatment of pleural effusion. Most children presented with fever, cough, and dyspnea (72%). Four children presented with abdominal pain (13%), which led to 2 appendectomies prior to referral. In 11 (33%) children, an invasive group A streptococcus was cultured from the pleural fluid. All patients were treated with antibiotics. One patient received VATS primarily. 27 (84%) patients received a chest drain, and 4 patients continued conservative treatment. 14 (52%) of the 27 children with a chest drain were treated with intrapleural fibrinolytics (alteplase or urokinase) because of persistent pleural effusion on ultrasound despite chest drainage. No side effects were observed. Two patients received VATS secondary to chest drainage without receiving prior intrapleural fibrinolytics.</p><p><strong>Conclusion: </strong>Intrapleural fibrinolytic treatment appeared safe and could avoid VATS in children with poor drainage, and is therefore a useful minimal invasive treatment in children with pleural empyema.</p>","PeriodicalId":21048,"journal":{"name":"Respiration","volume":" ","pages":"1-4"},"PeriodicalIF":2.6,"publicationDate":"2026-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148284282","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":"Follow-Up Care after Adult Lung Transplantation: Summary of an Evidence-Based Clinical Practice Guideline for German-Speaking Countries.","authors":"Jens Gottlieb, Alexandra Wald, Brigitte Bucher, Burkhard Tapp, Caroline Hirsch, Christian Schneider, Hauke Winter, Heinrike Wilkens, Henning Geldmacher, Ina Hettich, Peter Jaksch, Macé M Schuurmans, Mario Schiffer, Matthias Hecker, Monika Nothacker, Nicole Skoetz, Nina Kreuzberger, Oliver Witzke, Roland Schmitt, Susana Jörger-Tuti, Vasiliki Bessa, Wolfgang Veit, Christian Taube, Nikolaus Kneidinger","doi":"10.1159/000553086","DOIUrl":"10.1159/000553086","url":null,"abstract":"<p><p>Lung transplantation (LTx) is a life-saving intervention for end-stage lung disease, yet long-term outcomes remain inferior compared to other solid organ transplants. No evidence-based national or international guidelines for post-transplant follow-up care existed prior to this work. This guideline aims to provide treating physicians with structured, evidence-based recommendations for follow-up care after LTx in adult patients. The guideline was developed with a multidisciplinary guideline group according to the guidance of the Association of the Scientific Medical Societies in Germany manual for evidence- and consensus-based guidelines. A systematic literature search was conducted across MEDLINE, Cochrane, Epistemonikos, and CINAHL databases through September 2024, focusing on randomized controlled trials and systematic reviews. Evidence quality was assessed using the GRADE framework and the Oxford Centre for Evidence-Based Medicine (CEBM) 2011 criteria. Consensus was established through structured consensus conferences, with recommendation strength classified as strong (\"We recommend\"), conditional (\"We suggest\"), or open (\"may or may not\"). Twelve evidence-based recommendations were developed, spanning five clinical domains: immunosuppression, infections, chronic lung allograft dysfunction (CLAD), and bone health. Tacrolimus was recommended over ciclosporin as the preferred calcineurin inhibitor, based on moderate-quality evidence demonstrating a significantly lower incidence of CLAD across four randomized controlled trials. Trimethoprim-sulfamethoxazole prophylaxis against Pneumocystis jirovecii and extended valganciclovir prophylaxis for cytomegalovirus-seropositive recipients were recommended. For CLAD, azithromycin was strongly recommended as a therapeutic trial to assess reversibility of graft dysfunction. Regarding bone health, daily calcium and vitamin D supplementation were strongly recommended for all recipients, with bisphosphonates, denosumab, or teriparatide indicated in those with osteopenia or manifest osteoporosis. This guideline provides the first structured, evidence-based framework for follow-up care after LTx in German-speaking countries. Tacrolimus-based triple immunosuppression, targeted infection prophylaxis, CLAD prevention, and systematic bone protection represent the cornerstones of post-transplant care. The guideline will remain valid until July 2030.</p>","PeriodicalId":21048,"journal":{"name":"Respiration","volume":" ","pages":"1-17"},"PeriodicalIF":2.6,"publicationDate":"2026-06-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13446881/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148265897","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}

{"title":"Learning Curve for Transbronchial Lung Cryobiopsy in Interstitial Lung Disease Diagnosis: A Multicenter Experience.","authors":"Run Tong, Shuliang Guo, Yang Xia, Yishi Li, Guowu Zhou, Weidong Xu, Yunchao Su, Mingming Deng, José M Porcel, Felix J F Herth, Gang Hou","doi":"10.1159/000553097","DOIUrl":"10.1159/000553097","url":null,"abstract":"<p><strong>Introduction: </strong>Transbronchial lung cryobiopsy (TBLC) is increasingly used to diagnose interstitial lung disease (ILD). However, the learning curve associated with this technique remains unclear.</p><p><strong>Methods: </strong>This retrospective analysis included the first 100 patients suspected of having ILD for whom TBLC was performed by six physicians (three chief physicians and three attending physicians) across three centers in China. The diagnostic yield, procedural information, and complications were compared between consecutive groups of patients. Logistic regression was employed to analyze the median diagnostic yields across ten subgroups of operative experience, categorized in intervals of 10 patients, to delineate the learning curve.</p><p><strong>Results: </strong>The overall multidisciplinary discussion (MDD)-based diagnostic rate for ILD after TBLC was 75% (450 of 600 procedures). The diagnostic yield increased with time. Learning curves revealed that chief physicians achieved proficiency after approximately 60 procedures and attending physicians after approximately 90. The sample biopsy was larger for chief physicians than for attending physicians (median, 19 mm2 vs. 15.25 mm2, p < 0.001). The complication rates were acceptable, with no significant difference between the chief and attending physicians over their first 100 procedures.</p><p><strong>Conclusion: </strong>TBLC is a safe and effective diagnostic approach for ILD, but operators require extensive training to achieve proficiency. Senior physicians reached competency earlier than junior colleagues (after several dozen procedures), underscoring the importance of structured training programs and standardized protocols for facilitating safe implementation.</p>","PeriodicalId":21048,"journal":{"name":"Respiration","volume":" ","pages":"1-10"},"PeriodicalIF":2.6,"publicationDate":"2026-06-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148265852","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":"Real-World Sotatercept Eligibility: Analysis from the FOCUS-PAH Registry.","authors":"Giulio Savonitto, Giovanni Santi, Matteo Pagnesi, Pier Paolo Bocchino, Piero Gentile, Mauro Acquaro, Mauro Driussi, Luciana D Apos Angelo, Francesca Macera, Luke S Howard, Matteo Toma, Gaia Miceli, Davide Barbisan, Valentino Collini, Carlo Maria Lombardi, Carolin Bauleo, Matteo Rugolotto, Elena Bertarelli, Gianluca Pagnoni, Emanuele Bobbio, Sven-Erik Bartfay, Johanna Wermström, Laura Scelsi, Alberto Giannoni, Suela Vani, Massimo Imazio, Edoardo Airo, Francesca Coppi, Claudia Raineri, Marco Metra, Andrea Garascia, Clara Hjalmarsson, Gianfranco Sinagra, Francesco Lo Giudice, Pietro Ameri, Davide Stolfo","doi":"10.1159/000552663","DOIUrl":"10.1159/000552663","url":null,"abstract":"<p><strong>Introduction: </strong>Pulmonary arterial hypertension (PAH) remains burdened by suboptimal outcomes despite contemporary therapy. Sotatercept showed clinical benefit in STELLAR and ZENITH, yet trial criteria may limit real-world implementation. We quantified real-world eligibility at baseline and during follow-up and identified baseline predictors of non-eligibility.</p><p><strong>Methods: </strong>We retrospectively analyzed 657 patients with incident PAH (2001-2024), excluding 116 with incomplete follow-up data. Inclusion criteria mirroring STELLAR were WHO functional class II/III, PVR ≥5 WU, and stable background therapy. Exclusion criteria included severe comorbidities or recent cardiovascular events. Multivariable models identified predictors of non-eligibility. Extended eligibility per ZENITH criteria was also assessed.</p><p><strong>Results: </strong>Among 541 analyzed patients, 104 (19%) were ineligible at baseline, mainly due to non-permitted PAH subtypes (71%) or vasoreactivity (29%). During a median 45-month follow-up, cumulative follow-up eligibility (i.e., patients who met criteria at least once after baseline) was 50%, with most exclusions due to PVR <5 WU (53%) or recent therapy changes (53%). Older age (HR 1.011, p = 0.003) and triple therapy (HR 2.841, p < 0.001) predicted non-eligibility, while idiopathic PAH was protective (HR 0.742, p = 0.025). Integrating ZENITH criteria increased eligibility to 56% (+6% vs. STELLAR).</p><p><strong>Conclusion: </strong>STELLAR criteria exclude a substantial portion of real-world PAH patients, particularly during follow-up, when therapy intensification is often required per the 7th World Symposium algorithm. ZENITH criteria offer only a modest improvement. Tailored selection strategies and optimal timing for initiation are needed to enhance sotatercept's applicability. Future trials should consider enrichment strategies to broaden treatment eligibility.</p>","PeriodicalId":21048,"journal":{"name":"Respiration","volume":" ","pages":"1-11"},"PeriodicalIF":2.6,"publicationDate":"2026-06-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148239771","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":"Novel Endobronchial Valve: A Case Series on the Technical Feasibility of the PulmValve in Severe Heterogeneous Emphysema.","authors":"Difei Chen, Jingwei Liu, Weizhan Luo, Lianyue Yang, Jinsheng Lin, Jing Zhang, Shiyue Li","doi":"10.1159/000552824","DOIUrl":"10.1159/000552824","url":null,"abstract":"<p><strong>Introduction: </strong>Bronchoscopic lung volume reduction with endobronchial valves is an established therapy for selected patients with severe heterogeneous emphysema. The PulmValve is a novel lobar endobronchial valve designed to achieve occlusion with larger-diameter valves. We present here its first-in-human exploratory experience.</p><p><strong>Case presentation: </strong>Six male patients underwent successful lobar valve implantation, with a median of 1 valve per treated lobe. No intraprocedural complications, device malfunctions, or valve migration were observed during follow-up. Four serious adverse events occurred, including 1 hydropneumothorax, 1 acute exacerbation of chronic obstructive pulmonary disease, 1 pulmonary infection in a non-treated lobe, and 1 sudden cardiac death. The fatal event was adjudicated as possibly unrelated. Early improvement in dyspnea was noted in most patients. Compared to baseline, at 3 months, forced expiratory volume in 1 s improved by 80 mL (30-310) and residual volume decreased by 200 mL (-890 to 430), accompanied by 6-min walk test increased by 34 m (-34 to 71) and St. George's Respiratory Questionnaire score dropped by 16 points (-4 to 23). At 6 months, pulmonary function changes were variable, while exercise capacity and symptom scores remained improved in selected cases.</p><p><strong>Conclusion: </strong>In this exploratory first-in-human case series, lobar PulmValve implantation was technically feasible and generated descriptive clinical signals. Larger controlled studies are required to define safety, durability, and clinical efficacy.</p>","PeriodicalId":21048,"journal":{"name":"Respiration","volume":" ","pages":"1-9"},"PeriodicalIF":2.6,"publicationDate":"2026-06-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148205926","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}