Advances in respiratory medicine最新文献

{"title":"Association of ICD-10-Coded Pneumonia Events with Interstitial Lung Disease Outcomes in Patients with Rheumatoid Arthritis: A Large Database Retrospective Cohort.","authors":"Esteban Kosak Lopez, Luis Rodriguez Donís, Justin Lam, Andrew Geller, Raul Leguizamon, Michael Vera Ricaurte, Priscilla Nethala, Maria Planchart Ferretto, Maria Laura Fernandez-Wever, Jose M Martinez-Manzano, Enrique Pacheco, Shahrzad Abdollahi","doi":"10.3390/arm94040049","DOIUrl":"10.3390/arm94040049","url":null,"abstract":"<p><p><b>Introduction:</b> Patients with rheumatoid arthritis (RA) have higher risk for pneumonia, interstitial lung disease (ILD) and pulmonary fibrosis (PF). However, the association between an ICD-10-coded pneumonia event (CPE) and the incidence of ILD or PF in the RA population remains unclear. <b>Methods:</b> We conducted a retrospective cohort study using the TriNetX database. Patients with ICD-10 for RA aged 50 or older who had a CPE within one year of RA diagnosis (CPE cohort, n = 4553) were matched 1:1 by propensity score for key factors, including demographics, comorbidities (i.e., COPD), and medication use (DMARDs, corticosteroids) to RA patients without a CPE (Control cohort, n = 4553). Cox proportional hazard models assessed the incidence of a composite ILD outcome, PF, and secondary complications over a 4-year follow-up after the index event defined as 1-year after RA diagnosis for both cohorts. <b>Results:</b> The CPE cohort showed an increased risk for all outcomes. Patients with CPE had a 2.48-fold increased risk for PF (HR = 2.48; 95% CI, 1.78-3.45; <i>p</i> < 0.01) and a 2.87-fold increased risk for the composite ILD outcome (HR = 2.87; 95% CI, 2.18-3.80; <i>p</i> < 0.01). The risk of rheumatoid lung disease was 4.15 times higher (HR = 4.15; 95% CI, 2.30-7.50; <i>p</i> < 0.01). Furthermore, the CPE group had a higher risk for all-cause mortality (HR = 1.82; 95% CI, 1.58-2.09; <i>p</i> < 0.01). <b>Conclusions:</b> The CPE within one year of RA diagnosis is associated with an increase in subsequent ILD-coded outcomes. While this retrospective design cannot establish causality, an unspecified pneumonia code in early RA may represent an early clinical manifestation of unrecognized ILD, serving as a high-risk marker that warrants pulmonary surveillance.</p>","PeriodicalId":7391,"journal":{"name":"Advances in respiratory medicine","volume":"94 4","pages":""},"PeriodicalIF":4.5,"publicationDate":"2026-07-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13509329/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148823534","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Artificial Intelligence-Induced Deskilling in Interventional Pulmonology: An International Cross-Sectional Survey on Risk Perception and Mitigation Strategies.","authors":"Guido Marchi, Lorenzo Corbetta","doi":"10.3390/arm94040048","DOIUrl":"10.3390/arm94040048","url":null,"abstract":"<p><p>Artificial intelligence (AI) is progressively reshaping interventional pulmonology (IP), yet its potential to erode procedural and cognitive competencies through AI-induced deskilling remains poorly characterized in this specialty. An international, observational, cross-sectional survey was conducted in May 2026 among 118 expert interventional pulmonologists from 10 different countries across 5 continents. Participants completed a structured questionnaire comprising five demographic items and 12 Likert-scale statements addressing deskilling risk perception and mitigation attitudes; percentage agreement was calculated for each item (scores 4-5). High perceived clinical value of AI was reported (87%), alongside substantial concern for procedural deskilling (73%) and upskilling inhibition (83%). Familiarity with automation bias was limited (38%), yet its clinical relevance was widely recognized after definition provision (81%)-a gap of 43 percentage points. Strong support emerged for AI-free training (84%), simulation-based training (86%), and longitudinal performance monitoring (78%). Concern for institutional fragility in the absence of AI was expressed by 74%, and governance frameworks, including minimum non-AI-assisted procedural volume requirements, were endorsed by 70%. Deskilling was identified as a high research priority by 89%. These findings indicate that AI-induced deskilling is perceived as a relevant and emerging risk by expert interventional pulmonologists internationally, even before the widespread clinical deployment of AI technologies. Although the extent to which these concerns will translate into measurable effects on procedural competence is currently uncertain, the results underscore the need for prospective research, educational initiatives, and appropriate governance frameworks to ensure the preservation of core procedural skills.</p>","PeriodicalId":7391,"journal":{"name":"Advances in respiratory medicine","volume":"94 4","pages":""},"PeriodicalIF":4.5,"publicationDate":"2026-07-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13398125/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148576815","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cystic Fibrosis Mortality Trends 1999-2024-A CDC Wonder Study.","authors":"Palak Grover, Rahul Jain, Gurleen Kaur, Niroshan Ranjan, Bipneet Singh","doi":"10.3390/arm94040047","DOIUrl":"10.3390/arm94040047","url":null,"abstract":"&lt;p&gt;&lt;p&gt;Cystic fibrosis (CF) is an autosomal recessive disorder caused by mutations in the &lt;i&gt;CFTR&lt;/i&gt; gene. The sequential approval of CFTR modulators ivacaftor (2012), lumacaftor/ivacaftor (2015), tezacaftor/ivacaftor (2018), and elexacaftor/tezacaftor/ivacaftor (2019) has transformed CF care, but population-level mortality trends across therapeutic periods have not been comprehensively assessed. We conducted a retrospective analysis of CF mortality in the United States from 1999 to 2024 using CDC WONDER Underlying Cause of Death data (ICD-10 codes E84.0-E84.9). Age-adjusted mortality rates (AAMR) per 100,000 were calculated using the 2000 U.S. standard population. The study period was divided into three periods: pre-modulator (1999-2011), early modulator (2012-2018), and elexacaftor/tezacaftor/ivacaftor (2019-2024). Annual mortality trends were evaluated using segmented log-linear Poisson regression, with annual death counts as the outcome and the corresponding U.S. population as an offset. Candidate models with multiple change points were compared to identify distinct temporal segments. Annual percent changes (APCs) and 95% confidence intervals (CIs) were estimated for each segment. Prespecified therapeutic periods, including pre-modulator (1999-2011), early modulator (2012-2018), and ETI period (2019-2024), were retained for descriptive analyses. Trends were stratified by sex and U.S. Census Region. A total of 10,959 CF deaths were recorded over 26 years. In the pre-modulator period, mortality was stable at a mean of 478 deaths/year (AAMR 0.14-0.17). The early modulator period showed a modest 5.4% reduction in mean annual deaths (452/year). The period following ETI availability was associated with a decline to 263/year, a 47% reduction from the pre-modulator period (AAPC -8.6%/year). The AAMR declined from 0.17 (1999) to 0.07 (2024). The female-to-male death count ratio shifted from 1.05 to 0.97 across periods, though age-adjusted rates were identical between sexes within each period, and this finding should be interpreted cautiously. Regionally, the South's share of CF deaths grew from 37.2% to 41.6% despite absolute declines in all regions, suggesting potential geographic disparities that warrant further investigation with individual-level data. Segmented regression identified change points in 2005 and 2015. Mortality declined significantly from 1999 through 2005 (APC, -2.70%; 95% CI, -4.01% to -1.37%), remained stable from 2006 through 2015 (APC, +0.21%; 95% CI, -0.47% to +0.90%), and declined sharply from 2016 through 2024 (APC, -9.76%; 95% CI, -10.66% to -8.84%). CF mortality in the United States has declined by more than half since the introduction of CFTR modulators. The shift in the sex-based death count ratio and the concentration of remaining deaths in the South are hypothesis-generating observations that require confirmation with individual-level data. These ecological findings cannot establish causation, as concurrent changes in su","PeriodicalId":7391,"journal":{"name":"Advances in respiratory medicine","volume":"94 4","pages":""},"PeriodicalIF":4.5,"publicationDate":"2026-07-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13397906/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148576764","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Prevalence and Impact of Pulmonary Hypertension Associated with Arteriovenous Fistulas and Grafts in End-Stage Renal Disease: A Systematic Review and Meta-Analysis.","authors":"Ahmed A Zayed, Mohammad Aldalahmeh, Salim Barakat, Georges Khattar, Walid Sange, Elie Bou Sanayeh, Zaid Khamis, Bahy Abofrekha, Suzanne El-Sayegh, Michel N Chalhoub","doi":"10.3390/arm94040046","DOIUrl":"10.3390/arm94040046","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Background/objectives: &lt;/strong&gt;Pulmonary hypertension (PH) is an increasingly recognized complication in patients with end-stage renal disease (ESRD) undergoing hemodialysis, particularly those utilizing arteriovenous fistulas (AVF) or grafts (AVG) for vascular access. The prevalence and clinical impact of PH in this population remain unclear due to methodological heterogeneity and variable diagnostic criteria. This systematic review and meta-analysis aimed to quantify the association between AVF/AVG use and PH prevalence in ESRD patients and to explore sources of heterogeneity.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Methods: &lt;/strong&gt;A systematic search of PubMed, Embase, Scopus, and Web of Science was conducted for studies published through 31 December 2024, without language or date restrictions. Eligible studies included adults (≥18 years) with ESRD on dialysis, comparing those with AVF/AVG access to non-AVF/AVG controls (e.g., tunneled dialysis catheters or peritoneal dialysis), and reporting PH prevalence or mean pulmonary artery pressures. Study quality was assessed using the Newcastle-Ottawa Scale, and risk of bias was evaluated. A random-effects meta-analysis calculated pooled odds ratios (OR) for PH prevalence, with heterogeneity assessed by I&lt;sup&gt;2&lt;/sup&gt; and Cochran's Q. Sensitivity analyses and tests for publication bias (Egger's and Begg's) were performed. Secondary analysis compared pooled mean pulmonary artery pressures between groups.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results: &lt;/strong&gt;Eleven observational studies (1299 dialysis patients) met the inclusion criteria; ten studies (1224 patients) contributed to the quantitative meta-analysis after exclusion of one study with a zero-event control arm. Most studies were small, predominantly cross-sectional, and of moderate methodological quality. The pooled analysis showed a statistically significant association between AVF/AVG use and PH (OR 2.06, 95% CI: 1.69-2.52), with low statistical heterogeneity (I&lt;sup&gt;2&lt;/sup&gt; = 0%). This estimate was sensitive to individual studies: in leave-one-out analysis the association lost statistical significance when the single most influential study was removed indicating that the pooled result is driven in part by a small number of studies rather than being uniformly robust. No statistical evidence of publication bias was detected. Five studies reported continuous pulmonary artery pressures, which were directionally higher in AVF/AVG patients but were not pooled because of extreme heterogeneity (I&lt;sup&gt;2&lt;/sup&gt; = 99.4%).&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusions: &lt;/strong&gt;In this synthesis of observational data, AVF/AVG use was associated with higher odds of pulmonary hypertension than non-AVF/AVG access. Because all included studies were observational and the pooled estimate is sensitive to individual influential studies, these findings indicate a possible association rather than a causal effect and should be interpreted with caution. They support the rationale for prospective hemodynamic studies and ","PeriodicalId":7391,"journal":{"name":"Advances in respiratory medicine","volume":"94 4","pages":""},"PeriodicalIF":4.5,"publicationDate":"2026-07-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13397879/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148576828","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinical Impact of RSV Vaccination in Hemodialysis Patients: Real-World Evidence on Hospitalization Risk and the Role of Chronic Lung Disease.","authors":"Francesca K Martino, Francesca Fioretti, Lucia Federica Stefanelli, Gianni Carraro, Miriam Capuano, Giuseppe Scaparrotta, Federico Nalesso","doi":"10.3390/arm94040045","DOIUrl":"10.3390/arm94040045","url":null,"abstract":"<p><p><b>Background:</b> Respiratory syncytial virus (RSV) infection is a cause of respiratory morbidity in high-risk patients, including those with chronic lung disease (CLD) and those undergoing hemodialysis (HD). In HD patients, evidence on the clinical impact of RSV vaccination on respiratory complications remains limited. We aimed to assess the clinical impact of RSV vaccination in HD patients by comparing vaccinated and unvaccinated patients with a focus on CLD. <b>Methods:</b> We retrospectively evaluated 56 adult HD patients: 28 received the RSV vaccine in autumn 2024 and 28 did not. Clinical data were collected from electronic medical records. Outcomes included influenza-like illness (ILI), pneumonia, and respiratory infection requiring hospitalization between September 2024 and September 2025. <b>Results:</b> Patients had a mean age of 74.4 years and a median Charlson Comorbidity Index (CCI) of 10. The RSV-vaccinated group had a greater comorbidity burden than the unvaccinated group (CCI 11 IQR 10-12 vs. 9 IQR 8-11, <i>p</i> = 0.02) and a higher prevalence of CLD (46.4% vs. 25.0%, <i>p</i> = 0.09). During follow-up, 28 patients (50.0%) had at least one ILI episode, 23 (41.1%) developed pneumonia, and 15 (26.8%) were hospitalized for respiratory infection. The incidence of ILI was 46.4% in vaccinated patients and 53.6% in unvaccinated patients (<i>p</i> = 0.28), while the incidence of pneumonia was 39.3% and 42.9%, respectively (<i>p</i> = 0.78). Respiratory infection requiring hospitalization occurred in 14.3% of vaccinated patients and 39.3% of unvaccinated patients (<i>p</i> = 0.035). CLD was significantly associated with pneumonia (<i>p</i> = 0.001) and showed trends toward higher rates of ILI (<i>p</i> = 0.09) and hospitalization for respiratory infection (<i>p</i> = 0.1). <b>Conclusions:</b> In our exploratory study, RSV vaccination in HD patients was associated with fewer hospitalizations for respiratory infection, despite greater comorbidity in vaccinated patients. CLD was associated with a higher incidence of respiratory complications, particularly pneumonia. The retrospective design and small sample size do not allow definitive conclusions; future prospective studies with an adequate sample size are needed to confirm our results.</p>","PeriodicalId":7391,"journal":{"name":"Advances in respiratory medicine","volume":"94 4","pages":""},"PeriodicalIF":4.5,"publicationDate":"2026-07-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13398179/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148576834","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Guidelines for Minimizing Bleeding Risk During Bronchoscopic Procedures.","authors":"Adam Barczyk, Anna Andrychiewicz, Małgorzata Czajkowska-Malinowska, Katarzyna Górska, Bartosz Hudzik, Piotr Korczyński, Rafał Krenke, Wojciech Naumnik, Wojciech J Piotrowski, Cezary Piwkowski, Jerzy Soja, Artur Szlubowski, Jerzy Windyga, Joanna Zając, Filip Mejza","doi":"10.3390/arm94040044","DOIUrl":"10.3390/arm94040044","url":null,"abstract":"<p><p>This article presents recommendations aimed at reducing the risk of bleeding during bronchoscopy. The document was developed by a working group convened by the Polish Respiratory Society, which included pulmonologists experienced in bronchoscopic procedures, an anesthesiologist, a thoracic surgeon, a cardiologist, a hematologist, a nurse, and methodologists. Clinical questions were formulated according to the PICO (Population, Intervention, Comparison, Outcome) framework, followed by a systematic literature search and critical appraisal of the selected studies. Based on these data, 13 recommendations/good clinical practice points were developed addressing bronchoscopy in patients with thrombocytopenia, abnormal activated partial thromboplastin time or international normalized ratio results, and in those receiving antiplatelet agents, oral anticoagulants, or low-molecular-weight heparin.</p>","PeriodicalId":7391,"journal":{"name":"Advances in respiratory medicine","volume":"94 4","pages":""},"PeriodicalIF":4.5,"publicationDate":"2026-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13397931/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148576782","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Utilization Patterns of Nebulized Glycopyrronium in Patients Hospitalized for Acute Exacerbations of Obstructive Airway Disease (AEOAD)-Indian Expert Perspectives.","authors":"Arjun Khanna, Pradyut Waghray, Ashok Kr Singh, Jinay Mehta, Rithik, Sagar Bhagat, Saiprasad Patil, Hanmant Barkate","doi":"10.3390/arm94040043","DOIUrl":"10.3390/arm94040043","url":null,"abstract":"<p><p><b>Background:</b> Acute exacerbation of obstructive airway disease (AEOAD) is a major cause of hospitalization, morbidity, and premature mortality in India. Hospitalized patients for the same are predominantly treated with short-acting bronchodilators, which require frequent administration and are associated with systemic adverse effects. Despite the availability of nebulized long-acting muscarinic antagonists (LAMAs) with quick onset of action, such as glycopyrronium, their role in acute care remains unclear in India. <b>Methods:</b> A pan-India expert opinion-building initiative was conducted among 220 pulmonologists across Tier I-II cities through 13 structured advisory meetings between April 2025 and July 2025. The final expert perspectives were then categorized into recurrent insights, raised in 75% or more meetings, and variable insights, raised in <75% of all meetings. <b>Results</b>: Experts reported that AEOAD management commonly involved initial stabilization with SABA/SAMA followed by transition to triple therapy with nebulized glycopyrronium, formoterol, and budesonide. Nebulized glycopyrronium was perceived to provide rapid and sustained bronchodilation with fewer cardiovascular side effects compared to short-acting agents. Benefits were reported in patients with frequent exacerbations, high sputum burden, and bronchiectasis. Operational advantages included reduced dosing frequency and nursing workload. Experts also noted potential improvements in hospital stay and readmissions; however, these observations were based on clinical experience rather than controlled data. <b>Conclusions:</b> Indian pulmonologists agreed that early initiation of nebulized glycopyrronium (with formoterol and budesonide) in hospitalized AEOAD may improve symptom control, lower exacerbation burden, reduce reliance on short-acting bronchodilators and corticosteroids, and shorten hospital stays.</p>","PeriodicalId":7391,"journal":{"name":"Advances in respiratory medicine","volume":"94 4","pages":""},"PeriodicalIF":4.5,"publicationDate":"2026-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13398247/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148576767","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Vertical Displacement Index and Early Treatment-Related Physiological Improvement in Acute Respiratory Failure: An Exploratory Ultrasound-Based Study.","authors":"Bedriye Müge Sönmez, İlker Şirin, Gülşen Akçay, Murat Özdemir, Necip Gökhan Güner","doi":"10.3390/arm94030040","DOIUrl":"10.3390/arm94030040","url":null,"abstract":"<p><p><b>Objective</b>: Rapid assessment of early treatment-related physiological improvement in emergency department (ED) patients with respiratory failure (RF) remains challenging. Blood gas analysis is informative but invasive and not ideal for repeated use. The vertical displacement index (VDI), an ultrasound-derived parameter based on pleural motion, may provide dynamic bedside information on early physiological change. This study evaluated whether changes in VDI are associated with early physiological improvement in ED patients with RF. <b>Methods</b>: This prospective observational study was conducted in the EDs of two tertiary care hospitals. Adult patients presenting with dyspnea and clinical evidence of RF were included. VDI was measured by lung ultrasound at baseline and 30 min after initial treatment. The primary endpoint was the change in VDI 30 min after the initial treatment, calculated as the difference between pre-treatment and post-treatment VDI. The expected direction was a post-treatment decrease in VDI, with greater VDI reduction expected to be associated with greater early physiological improvement. Secondary analyses included comparisons of VDI changes across oxygen saturation and diagnostic groups, as well as correlations between ΔVDI and physiological changes. Patients were grouped by admission oxygen saturation (<80%, 80-90%, and ≥90%). <b>Results</b>: Seventy-nine patients were included. Pre-treatment VDI differed significantly between oxygen saturation groups, with the highest values in the most hypoxemic patients (<i>p</i> = 0.028). VDI decreased significantly after treatment in all groups (<i>p</i> < 0.001 for all), with the greatest reduction in the <80% group. By diagnosis, VDI decreased significantly in pulmonary edema, COPD/asthma, and pneumonia, but not in pulmonary embolism (<i>p</i> = 0.138). VDI reduction correlated positively with improvements in oxygen saturation (r = 0.27, <i>p</i> = 0.016) and pH (r = 0.24, <i>p</i> = 0.037), but not with CO₂. <b>Conclusions:</b> VDI may be explored as a practical ultrasound-derived bedside parameter associated with early physiological improvement in ED patients with RF.</p>","PeriodicalId":7391,"journal":{"name":"Advances in respiratory medicine","volume":"94 3","pages":""},"PeriodicalIF":4.5,"publicationDate":"2026-06-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13295631/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148315072","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Advances in Therapeutic Options for Pulmonary and Sleep Disorders in Mucopolysaccharidosis (MPS) Patients: A Narrative Review.","authors":"Bimaje Akpa","doi":"10.3390/arm94030041","DOIUrl":"10.3390/arm94030041","url":null,"abstract":"<p><p>Mucopolysaccharidosis (MPS) are a group of inherited lysosomal storage genetic disorders that affect the body's ability to break down glycosaminoglycans (GAGs) due to the deficiency of required enzymes. This leads to depositions of these GAGs in various tissues and organs resulting in multi-systemic manifestations including pulmonary and sleep related issues. In recent years, there have been significant advancements in therapeutic options and supportive management which have led to the overall improvement in respiratory care, culminating in improved quality of life for MPS patients. Management of pulmonary and sleep disorders in mucopolysaccharidosis requires a multidisciplinary approach due to the multi-systemic affectation of the genetic disorders. Therapeutic options such as enzyme replacement therapy (ERT) and hematopoietic stem cell transplantation (HSCT) have yielded varying success in mitigating respiratory complications. Emerging treatments such as gene therapies have shown exciting and promising results thus far. Supportive therapies such as airway clearance, regular vaccination and use of positive airway pressure devices are also essential. Pre-operative airway and anesthesia planning is critical to mitigate peri-operative and post-operative complications. Early diagnosis, close monitoring and a patient focused individualized approach are essential for respiratory optimization and overall improvement in clinical outcomes. This review article aims to discuss these advancements in a comprehensive format, making it accessible to medical providers who care for this subset of patients.</p>","PeriodicalId":7391,"journal":{"name":"Advances in respiratory medicine","volume":"94 3","pages":""},"PeriodicalIF":4.5,"publicationDate":"2026-06-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13295430/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148315246","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Acute Exacerbation of Interstitial Lung Disease: A Case Series and a Narrative Literature Review.","authors":"Bartłomiej Czyżak, Adam Lasota, Sebastian Majewski","doi":"10.3390/arm94030042","DOIUrl":"10.3390/arm94030042","url":null,"abstract":"<p><p>Acute exacerbation of interstitial lung disease (AE-ILD) represents sudden, severe deterioration in patients with pre-existing ILD and is associated with high morbidity and mortality. Our work presents a case series of AE-ILD in patients with idiopathic pulmonary fibrosis (IPF), idiopathic non-specific interstitial pneumonia (iNSIP), and connective tissue disease-associated ILD (CTD-ILD) managed at our institution and provides a narrative review of AE-ILD. Across cases, AE-ILD manifested as rapid progression of dyspnea and extensive ground-glass opacities (GGOs) on imaging, often triggered by infections or immune-mediated processes. Despite treatment, all cases were fatal, confirming that mortality remains high in AE-ILD. In our literature review, we focus on dysregulated innate immunity, an altered microbiome, potential microaspiration, surgical procedures, and autoantibody-mediated inflammation as triggers, as well as the risk factors for and prevalence of AE-ILD. We also examine pharmacological and non-pharmacological interventions, with particular emphasis on the role of antifibrotic agents as a key protective factor. Evidence for and against corticosteroid use in AE-IPF and non-IPF AE-ILD is discussed, highlighting the radically different treatment approach for AE in melanoma differentiation-associated gene 5 (MDA5)-positive dermatomyositis (DM)-associated ILD compared to AE-IPF. Our findings underscore the heterogeneous presentation and poor prognosis of AE-ILD, emphasizing the urgent need for standardized diagnostic criteria, risk stratification, and prospective studies with larger cohorts to establish evidence-based therapeutic strategies.</p>","PeriodicalId":7391,"journal":{"name":"Advances in respiratory medicine","volume":"94 3","pages":""},"PeriodicalIF":4.5,"publicationDate":"2026-06-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13296277/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148315258","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}