BACKGROUND:Idiopathic pulmonary fibrosis (IPF) is characterised by progressive loss of lung compliance, which raises the elastic work of breathing and alters resting ventilatory strategy. Clinical assessment of IPF still relies on forced manoeuvres, particularly forced vital capacity (FVC), which capture maximal performance rather than this resting adaptation. How tidal volume (VT) evolves over the IPF course is not well characterised. VT and the VT/FVC ratio offer an effort-independent window onto the same physiology, characterised here longitudinally. METHODS:We analysed 613 IPF patients enrolled at three European eurIPFreg centres (Giessen, Barcelona, Paris) between 2009 and 2025. Baseline VT, FVC, and VT/FVC were compared between survivors and non-survivors. Transplant-free survival was analysed by Cox regression including age, sex, antifibrotic treatment, log-transformed VT and FVC, and their interaction. Discrimination was assessed by Harrell's C-index with 20-fold cross-validation and time-dependent AUCs. Longitudinal trajectories were modelled over 60 months. RESULTS:VT followed a dynamic, non-monotonic trajectory consistent with progressive ventilatory adaptation, rising transiently to ~5% above baseline at months 18-21, coincident with the steepest segment of FVC decline (months 6-18), and returning slowly toward baseline thereafter. The VT/FVC ratio rose continuously (+17% at 60 months), the only marker to evolve monotonically, while FVC declined progressively (-8.8%). Higher baseline VT showed a trend toward improved survival (log-rank p = 0.092; HR 0.88, 95% CI 0.75-1.04), and the VT/FVC ratio stratified survival significantly (log-rank p < 0.001), as did baseline FVC (HR 0.59 per unit log-FVC, 95% CI 0.49-0.69; p < 0.001). The VT × FVC interaction approached significance (HR 1.12, 95% CI 0.98-1.30; p = 0.08), suggesting a joint physiological signal. Antifibrotic therapy reduced mortality (HR 0.63, 95% CI 0.50-0.81; p < 0.001). CONCLUSIONS:VT in IPF follows a dynamic, non-monotonic course, rising during the steepest phase of FVC decline and returning toward baseline thereafter, consistent with a ventilatory adaptation in which increased VT serves as a transient lower-cost strategy for maintaining minute ventilation before the elastic load makes it unsustainable. The VT/FVC ratio integrates this rising drive with falling vital capacity, evolves monotonically, and stratifies survival, identifying it as an effort-independent physiological descriptor of restrictive remodelling. Together, VT and VT/FVC enrich the standard FVC-based assessment of IPF by capturing the resting ventilatory dimension of disease progression.
INTRODUCTION:The clinical phenotype of pulmonary arterial hypertension (PAH) has shifted increasingly towards older patients with more cardiovascular comorbidities. Sodium-glucose cotransporter-2 inhibitors (SGLT2i) are used to treat symptomatic chronic heart failure. This study aimed to evaluate the impact of add-on SGLT2i in PAH patients with heart failure with preserved ejection fraction (HFpEF) as comorbidity. METHODS:PAH patients on stable therapy receiving SGLT2i as add-on were included in this single-center, retrospective cohort study. The primary endpoint comprised the change in echocardiographic right atrial (RA) area and right ventricular (RV) function after treatment with SGLT2i. Further clinical and echocardiographic parameters were analyzed. RESULTS:In total 198 patients on stable PAH therapy for at least three months receiving SGLT2i treatment and available follow-up assessments were analyzed (74±28 years, 61% female; 83.9% WHO functional class III-IV). During SGLT2i treatment, a significant reduction in RA area -1.93 ± 4.45 cm² (p<0.001) and improvement in RV function (p<0.001) during the mean follow-up of 6.7±3.3 months could be detected. RV area (-1.69±4.83 cm²), left atrial diameter (-2.17±4.43 mm) and systolic pulmonary arterial pressure (-9.26±16.10 mmHg) decreased significantly (all p<0.001). The mean 6-minute walking distance increased by 51.68±58.5 meters (p<0.001) and risk stratification significantly improved. Five patients discontinued the medication due to side effects. CONCLUSION:In patients with PAH and HFpEF as comorbidity SGLT2i as add-on to targeted therapy significantly improved right heart size and function, as well as further clinical parameters and was well tolerated. Further prospective studies are necessary to confirm these results.
BACKGROUND:Despite the rising recognition of chronic obstructive pulmonary disease (COPD) in younger adults (20-50 years), the clinical heterogeneity of this population during acute exacerbations (AECOPD) remains undefined, obscuring precision risk stratification and personalized management. METHODS:This study analyzed data from a nationwide, multicenter, prospective cohort across 10 tertiary hospitals in China (Sep 2017-Jul 2021). From 13,993 eligible patients, we identified 334 young AECOPD cases. Unsupervised K-means clustering was applied using nine biomarkers of inflammation, hematologic function, and structural lung damage. To contextualize its clinical relevance, the disease severity of the identified high-risk phenotype was benchmarked against the elderly AECOPD cohort (n=13,659). RESULTS:Cluster analysis identified two distinct phenotypes. The "Systemic Inflammatory Phenotype" (n=179) was characterized by a pervasive inflammatory state (elevated CRP, neutrophils, WBC, PCT, and fibrinogen) and significantly worse lung function. In contrast, the "Eosinophilic Phenotype" (n=155) exhibited an eosinophil-predominant profile. Critically, the Systemic Inflammatory Phenotype experienced a more severe hospital course, with significantly higher rates of invasive mechanical ventilation (3.9% vs. 0.0%, p=0.017), non-invasive ventilation (23.5% vs. 10.3%, p=0.003), and ICU admission (6.7% vs. 1.3%, p=0.026). Notably, the overall disease severity of this high-risk phenotype were comparable to the elderly AECOPD cohort. CONCLUSION:We identified a high-risk systemic inflammatory phenotype among young AECOPD inpatients, demonstrating clinical acuity on par with elderly patients. These findings challenge the conventional age-centric risk paradigm and advocate for a shift towards phenotype-driven assessment to enable early identification and targeted management of young AECOPD patients at the greatest risk.
Background: Advances in medical research, early diagnosis, and therapeutic strategies have significantly improved survival among children with chronic pulmonary diseases (CPDs), leading to a growing population of adolescents and young adults transitioning to adult care. Despite this demographic shift, healthcare systems, including Italy’s, remain inadequately equipped to manage the evolving needs of these patients. This study aims to identify key barriers and unmet needs within the Italian healthcare system regarding the transition from pediatric to adult care for individuals with CPDs. Methods: A nationwide survey was conducted among members of SIMRI (Italian Society for Childhood Respiratory Diseases) through a questionnaire consisting of thirteen questions. The survey was sent between June and August 2023, with responses collected until July 2024. Results: A total of Fifty-one responses were collected from pulmonologists, hospital-based pediatricians, primary care pediatricians, and adult pulmonologists from 14 Italian regions. Of these, 64.7% reported the absence of a structured transition program within their institutions. Where programs existed, they primarily targeted severe asthma (60.6%) and PCD or non-CF bronchiectasis (24.2%). Only 15.7% of respondents consistently addressed transition with patients and families, and 13.8% of centers had dedicated transition clinics. Proposed improvements included the establishment of specialized clinics (76.5%) and targeted professional training (72.5%). Conclusions: The transition of CPDs patients in Italy is currently fragmented and underdeveloped. Standardized protocols, dedicated transition services, interdisciplinary collaboration, continuous education, and involvement of patient associations are essential to ensure continuity of care and optimize long-term health outcomes.
Introduction: 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. Case Series: Six male patients underwent successful lobar valve implantation, with a median of one valve per treated lobe. No intraprocedural complications, device malfunctions, or valve migration were observed during follow-up. Four serious adverse events occurred, including one hydropneumothorax, one acute exacerbation of COPD, one pulmonary infection in a non-treated lobe, and one 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 second (FEV1) improved by 80 mL (30 to 310) and residual volume (RV) decreased by 200 mL (-890 to 430), accompanied by 6-minute walk test (6MWT) increased by 34 m (-34 to 71) and St. George's Respiratory Questionnaire (SGRQ) 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. Conclusion: 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.
INTRODUCTION:Obstructive sleep apnoea (OSA) is a respiratory disorder with comorbidities of several nature, from cardiovascular to renal ones. OSA is typically treated through continuous positive airway pressure (CPAP), but more investigations are needed to confirm its benefits. METHODS:In this work, survival analysis has been exploited and enhanced with eXplainable Artifical Intelligence (XAI) to investigate the impact of comorbidities and compare the model reliability. The dataset encompasses both clinical and polisomnography-based data for a total of 45 different features. RESULTS:1394 OSA patients followed for 15 years were enrolled. All the selected features have been studied by means of DL-models and time-dependent XAI techniques. The variables impacting on mortality are reported below in descending order with respect their importance: 1. Age, Years of CPAP, Renal dysfunction, COPD, BMI categories, Sex, and Anemia for the CoxTime; 2. Age, AHI, Renal dysfunction, SaO2 min, Years of CPAP, COPD, and Anemia for the LogHazard. CONCLUSION:Advancing age, severity of OSA, comorbidity, including chronic kidney disease, COPD and anaemia, are crucial contributors to increased mortality.
BACKGROUND:Short-term airway stenting is performed in patients with severe excessive central airway collapse (ECAC) to assess whether airway stabilization leads to symptomatic improvement thereby supporting surgical management. Interpretation of stent trials, however, may be confounded by complications such as bacterial colonization and granulation tissue formation. METHODS:Retrospective review of patients who underwent airway stenting at our institution between 2016 and 2022. We assessed the incidence of airway bacterial colonization, defined as conversion from a negative pre-stent to a positive post-stent bronchial wash (BW) culture, and the development of granulation tissue 7-14 days after stent placement, as well as their correlation with clinical outcomes. RESULTS:Pre- and post-stent bronchial washings were available from 101 stent trials. Airway bacterial colonization was observed in 40% of cases, with S.aureus accounting for 79%. Granulation tissue developed in 63% of cases, and it was significantly more frequent in silicone stents compared to Uncovered Self-Expanding Metallic Airway Stents (USEMAS) (odds ratio [OR] = 16; 95% CI, 1.6-166.7, P = .02). It was also more frequent in patients with positive post-stent BW (adjusted OR = 2.6; 95% CI, 0.9-6.6, P = 0.05) and in cases of early colonization (adjusted OR = 2.98; 95% CI, 0.9-9.5, P = 0.065). Although colonization and granulation tissue occurred more frequently in patients with no clinical improvement, neither was independently associated with increased odds of a negative stent trial. CONCLUSIONS:Bacterial colonization, particularly with S. aureus, occurs early after stent deployment and is associated with granulation tissue formation. However, these complications did not significantly affect clinical stent trial outcomes.
INTRODUCTION:Home noninvasive ventilation (HNIV) is an established therapy for chronic obstructive pulmonary disease (COPD) with chronic respiratory failure. However, outcomes following HNIV initiation after discharge from the hospital after acute COPD exacerbation remain poorly investigated, in particular sex-related factors. METHODS:We conducted a single-center, retrospective cohort study on COPD patients admitted to our respiratory care unit (RCU) for acute exacerbation with respiratory failure who were discharged home with newly established HNIV between 2013 and 2019. The primary endpoint was all-cause mortality after discharge from RCU. Sex-stratified survival analysis was performed using Kaplan-Meier curves, Cox proportional hazards models, and interaction models incorporating sex and clinical covariates, including lung function test and blood gases assessed at discharge. RESULTS:A total of 84 patients fulfilled all inclusion and exclusion criteria. The median age was 74 (IQR: 68-77) years, the male-to-female ratio was 1.4:1, and the median forced expiratory volume in 1 s (FEV1) at discharge was 25 (19.3-35.6)% predicted. Median survival was 158 (47-301) weeks, and Kaplan-Meier analysis demonstrated a longer survival time in males than in females (217 [77-434] vs. 130 [26-226] weeks; p = 0.036). After adjustment for age, FEV1% and other significant sex-dependent variables, female sex remained an independent predictor of increased mortality (HR [95% CI], 2.13 [1.01-4.49]; p = 0.046). Interaction analysis confirmed improved model fit with sex-by-covariate terms (χ2 = 12.85, p = 0.045; C-index = 0.71). Higher FEV1% was protective overall (HR [95% CI], 0.94 [0.86-0.99]; p = 0.028), but this benefit was only present in men (interaction p = 0.016). In a subgroup of patients, diffusing capacity of the lung for carbon monoxide (DLCO) data were available. Compared to males, females had lower DLCO and higher pCO2 levels at hospital discharge, which may explain the observed sex difference in survival. CONCLUSION:In COPD patients released home with newly established HNIV after hospitalization for acute exacerbation with respiratory failure, even after adjustment for established clinical covariates, female sex was associated with worse survival. FEV1% was also associated with survival; however, only in males and not in females, indicating sex-specific disease trajectories. These findings highlight the importance of incorporating sex-by-covariate interactions into prognostic models and the need for sex-tailored management strategies in advanced COPD.
INTRODUCTION: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. METHODS: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. RESULTS: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%). CONCLUSION: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.
Background. Sex-related differences in interstitial lung disease (ILD) phenotypes are well recognized, but it remains unclear whether sex itself independently influences outcomes in non-idiopathic pulmonary fibrosis (non-IPF) ILD once comorbidities, lung function, and treatment are considered. Methods. In the prospective INSIGHTS-ILD registry (data cut 17 September 2025), we compared men and women with non-IPF ILD using descriptive analyses and Cox models with prespecified adjustment steps: Model A (age, comorbidity count, smoking), Model B (A + forced vital capacity [FVC] and diffusing capacity of the lung for carbon monoxide [DLCO]), and Model C (B + antifibrotic therapy). Prespecified subgroup analyses included age strata (≤55 and >55 years) and ILD entities. Longitudinal FVC and DLCO trajectories were assessed over 24 months. Results. Among 883 patients (483 men, 400 women), exposures and disease entities differed significantly by sex: men reported more occupational/environmental exposures and had higher rates of fibrotic idiopathic interstitial pneumonia, whereas women more frequently had autoimmune-related ILD and a family history of ILD. Men had a higher comorbidity burden and more often received antifibrotic therapy at baseline. Survival was shorter in men (HR 1.51; 95% CI 1.03–2.21), but this association disappeared after adjustment in Model A (HR 1.04; 0.65–1.68), Model B (HR 1.03; 0.61–1.74), and Model C (HR 1.04; 0.62–1.77). Progression-free survival and transplant-free survival showed no consistent sex-related differences. Longitudinal FVC and DLCO declines were modest and largely parallel in both sexes, with no significant between-group differences. Findings were similar across age groups and ILD entities. Conclusion. Men and women with non-IPF ILD differ in exposures, phenotypes, and comorbidities, but after accounting for these factors, sex is not an independent predictor of survival or functional progression. Risk assessment should therefore primarily be based on objective disease characteristics rather than sex alone.
INTRODUCTION: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. METHODS: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. RESULTS: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). CONCLUSION: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.
Introduction: 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. Methods: 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. Results: 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). Conclusion: 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.
INTRODUCTION: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. METHODS: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. RESULTS: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. CONCLUSION: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.
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.
INTRODUCTION:Transbronchial lung cryobiopsy (TBLC) is increasingly used to diagnose interstitial lung disease (ILD). However, the learning curve associated with this technique remains unclear. METHODS: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. RESULTS: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. CONCLUSION: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.
INTRODUCTION: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. METHODS: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. RESULTS: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). CONCLUSION: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.
INTRODUCTION: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. CASE PRESENTATION: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. CONCLUSION: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.
INTRODUCTION:Lung volume reduction surgery (LVRS) is an established treatment for selected emphysema patients. Because Chronic obstructive pulmonary disease differs by sex in aetiology, symptom perception, and treatment response, we examined whether these differences affect LVRS outcomes. METHODS:All consecutive LVRS patients (August 2019-May 2025) were included and stratified by sex (111 men, 96 women). Propensity-score matching (PSM) (caliper 0.01, optimal matching) balanced age, body mass index, forced expiratory volume in 1 s (FEV1), RV, type of surgery, oxygen use, azithromycin, and emphysema morphology between groups. Endpoints were changes from baseline in pulmonary function (FEV1, RV), exercise capacity (6-min walking distance [6MWD] and Quality of Life [QoL]) (St. George's respiratory questionnaire [SGRQ]) at 3 and 6 months; as well as transplant-free survival. RESULTS:After PSM, 81 men and 81 women were included. Pack-years were similar (40 vs. 39; p = 0.060). Baseline characteristics were comparable after matching, but men had longer 6MWD (382 vs. 332 m; p = 0.011). At 3 months, improvements were comparable for FEV1 %pred (+11% vs. +9%; p = 0.22), RV (-59% vs. -48%; p = 0.49), and 6MWD (+57 m vs. +54 m; p = 0.47), whereas SGRQ improved more in men (-19 vs. -11 points; p = 0.020). At 6 months, changes in FEV1 %pred, 6MWD and SGRQ were similar between sexes. Three-year transplant-free survival was comparable (81% [95% CI: 70-94] vs. 83% [95% CI: 73-93], p = 0.70). CONCLUSION:Sex did not influence short-term postoperative functional outcome, QoL, or survival. Both sexes improved, with only a brief early QoL advantage in men. These findings indicate that sex should not guide expectations, as men and women benefit equally from LVRS.
INTRODUCTION:Challenges associated with decannulation following tracheostomy frequently arise from diminished cough efficacy. Active-passive lower limb training is a rehabilitative strategy that is commonly employed but insufficiently studied in this context. This study aimed to assess the effectiveness of active-passive lower limb training in potentially enhancing diaphragm engagement, thereby improving cough capacity in tracheostomized patients with central nervous system injuries. METHODS:A total of 36 patients were enrolled in this study and stratified into three groups (n = 12) for a 14-day intervention assessment. Utilizing a randomized crossover design combine with a control group. In intervention groups, participants were allocated to one of two intervention sequences: (1) the CA sequence, which involved an initial phase of active-passive lower limb training followed by an additional physiotherapy (PT) course; or (2) the AC sequence, which applied these interventions in the reverse order. A standard care control group (Con) was monitored for an equivalent duration. Assessments were conducted on days 1, 7, and 14. Primary and secondary outcomes included peak cough flow (PCF), Glasgow Coma Scale score, and ultrasound-derived measurements of cough-related respiratory muscle thickness. RESULTS:All groups showed significantly improved PCF post-intervention (p = 0.011, 0.007, 0.007). Active-passive lower limb training led to a greater PCF increase compared to additional PT or standard care (p = 0.011). Both interventions increased parasternal intercostal muscle thickness (p < 0.05). CONCLUSIONS:Active-passive lower limb training significantly improved cough capacity in tracheostomized patients with CNS injuries, being as effective as additional PT and potentially reducing staffing needs. This intervention is a promising approach to enhance cough effectiveness in tracheostomy patients.
