Respir Med [JOURNAL]
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200 papers
Long-term noninvasive ventilation in children with fibrodysplasia ossificans progressiva.
La Regina DP, Griffon L, Poirault C … +8 more , Vedrenne-Cloquet M, Baujat G, Khirani S, Lascourrèges C, Midulla F, Cormier-Daire V, Bodemer C, Fauroux B
Respir Med · 2026 · PMID 42242566 · Publisher ↗
Fibrodysplasia ossificans progressiva (FOP) is an autosomal dominant rare disease characterized by progressive episodic multi-focal heterotopic ossification of skeletal muscle, ligaments, tendons, and fascia, associated...
Fibrodysplasia ossificans progressiva (FOP) is an autosomal dominant rare disease characterized by progressive episodic multi-focal heterotopic ossification of skeletal muscle, ligaments, tendons, and fascia, associated with congenital malformation of the great toes. Progressive thoracic deformity with scoliosis is present in approximately 65% of patients, causing restrictive lung disease, respiratory failure, and premature death. We report 3 children who developed severe scoliosis and sleep-disordered breathing with nocturnal hypoventilation, and were treated with long-term noninvasive ventilation (NIV) at home. Despite a non-optimal correction of nocturnal alveolar hypoventilation due to the severity of the disease, tolerance and compliance with NIV were excellent, with an improvement in sleep quality, a reduction in acute respiratory exacerbations requiring hospitalization, the maintenance of a good quality of life, and long-term survival. In conclusion, patients with FOP should be screened for nocturnal hypoventilation and recurrent or severe respiratory exacerbations, in order to propose long-term NIV, which may be associated with a better quality of life, and may eventually prolong life expectancy, even in case of uncomplete correction of nocturnal gas exchange.
Comparison of diaphragmatic function between pressure support and NAVA using ultrasonography.
Kotoulas SC, Soilemezi E, Pisanidou P … +5 more , Morfesis P, Angeloudi E, Mouratidou C, Mouloudi E, Liatsi D
Respir Med · 2026 · PMID 42242565 · Publisher ↗
BACKGROUND: Pressure support (PS) is the most widely used mode for weaning from mechanical ventilation, yet 20-30% of ICU patients fail to wean successfully. Neurally adjusted ventilatory assist (NAVA), which triggers an...
BACKGROUND: Pressure support (PS) is the most widely used mode for weaning from mechanical ventilation, yet 20-30% of ICU patients fail to wean successfully. Neurally adjusted ventilatory assist (NAVA), which triggers and supports breathing based on diaphragmatic electrical activity, improves patient-ventilator asynchrony and diaphragmatic efficiency. However, its effects on diaphragmatic function and weaning outcomes remain unclear. OBJECTIVES: To compare diaphragmatic function between PS and NAVA using ultrasonographic indices sensitive to predicting weaning failure. We hypothesized that NAVA would yield more favorable measurements even after brief implementation. METHODS: In this prospective study, 19 adult ICU patients with difficulty weaning were consecutively enrolled. Patients were initially ventilated with NAVA titrated to achieve ∼6 ml/kg tidal volume, followed by PS adjusted to match peak pressure and tidal volume. After stabilization in each mode, diaphragmatic ultrasound was performed by a blinded operator. Tissue Doppler parameters (peak contraction velocity, peak relaxation velocity, maximal relaxation rate) and thickening fraction were measured over 2-3 stable cycles per method. Ventilatory and diaphragmatic electrical activity parameters were also recorded. Paired-samples t-test or Wilcoxon signed-rank test was used to detect differences between modes. RESULTS: Two-hundred and nineteen breathing cycles were analyzed (109 PS, 110 NAVA). No significant differences were observed between PS and NAVA in ventilatory parameters, diaphragmatic electrical activity, tissue Doppler indices, or thickening fraction (all p > 0.2). CONCLUSION: In this first ultrasonographic comparison of diaphragmatic function between PS and NAVA, no clinically meaningful differences were detected despite matched ventilatory parameters. Larger studies with longer exposure are needed to clarify potential advantages of NAVA. REGISTRATION NUMBER OF CLINICAL TRIAL: The study protocol was approved prior to the initiation of enrolment by the Ethics Committee of Papageorgiou General Hospital of Thessaloniki, reference number ΕΣ 352η/16-03-2022.
Genotype-phenotype correlation of CFTR variants in cystic fibrosis patients.
Canitez Y, Ozemri Sag S, Nalbant N … +10 more , Alemdar A, Hayme S, Korkmaz M, Demirdogen E, Celebi S, Hacimustafaoglu MK, Ozgur T, Ozkan TM, Sapan N, Temel SG
Respir Med · 2026 · PMID 42242564 · Publisher ↗
INTRODUCTION: Cystic fibrosis (CF) is a life-limiting autosomal recessive disorder caused by mutations in the CFTR gene. In Turkiye, high genetic diversity and consanguinity contribute to a heterogeneous CFTR variant spe...
INTRODUCTION: Cystic fibrosis (CF) is a life-limiting autosomal recessive disorder caused by mutations in the CFTR gene. In Turkiye, high genetic diversity and consanguinity contribute to a heterogeneous CFTR variant spectrum; however, genotype-phenotype data remain limited. METHODS: This retrospective study analyzed 92 Turkish CF patients (36 homozygous, 56 compound heterozygous) to characterize CFTR variant diversity and clinical outcomes. Next-generation sequencing (NGS) was performed and confirmed by Sanger sequencing. Clinical data were analyzed using chi-square and nonparametric tests (p < 0.05). RESULTS: A total of 46 distinct CFTR variants were identified. The most frequent were F508del (24%), followed by Y515X (7.33%), G542X (6%), N1303K (5.33%) and D110H (4%). Meconium ileus (p = 0.017) and CF-related diabetes mellitus (p = 0.013) were significantly more frequent among homozygous patients. Pseudomonas aeruginosa and Staphylococcus aureus were the most prevalent pathogens. CFTR modulator therapy eligibility was significantly higher among compound heterozygous patients (p = 0.007). Non-missense variants were associated with higher sweat chloride levels and more frequent severe pancreatic insufficiency and abnormal fecal fat quantification compared with missense variants (p = 0.037; p = 0.001; p = 0.033). CONCLUSION: This study highlights the heterogeneous CFTR variants in Turkiye and suggests genotype-phenotype correlations. Homozygous patients tended to show more severe gastrointestinal and endocrine involvement, while compound heterozygotes may exhibit comparatively milder disease features and higher treatment eligibility.
Metronome-paced tachypnea test cutoff value for detecting dynamic hyperinflation in COPD: A diagnostic accuracy study.
Dos Santos ÁH, da Costa CC, de Souza RM … +2 more , Berton DC, Teixeira PJZ
Respir Med · 2026 · PMID 42235615 · Publisher ↗
BACKGROUND: Dynamic hyperinflation (DH) is a key mechanism of exertional dyspnea in COPD, but cardiopulmonary exercise testing (CPET) is not universally available. OBJECTIVE: To determine the diagnostic accuracy and opti...
BACKGROUND: Dynamic hyperinflation (DH) is a key mechanism of exertional dyspnea in COPD, but cardiopulmonary exercise testing (CPET) is not universally available. OBJECTIVE: To determine the diagnostic accuracy and optimal absolute cutoff value of the metronome-paced tachypnea (MPT) test for detecting exercise-induced DH in patients with moderate COPD, using constant-load CPET as the reference standard. METHODS: This diagnostic accuracy study included 18 patients with moderate COPD. Patients underwent pulmonary function testing, symptom-limited incremental CPET, and two paired sessions, 21 days apart, each including MPT followed by constant-load CPET after approximately 30-60 min. DH on CPET was defined as an inspiratory capacity (IC) reduction ≥10% and/or ≥0.15 L. IC was measured at rest, after MPT, and at peak exercise. ROC curve analysis and the Youden index were used to determine the optimal post-MPT IC cutoff. RESULTS: The MPT test showed good accuracy for identifying CPET-defined DH (AUC 0.862, 95% CI 0.675-0.963; p = 0.004). An IC decrease of 0.27 L after MPT was the optimal cutoff, with 94.7% sensitivity and 75% specificity. Mean bias between MPTΔIC and CPETΔIC was -0.25 L. No significant correlation was observed between MPTΔIC and baseline airflow obstruction or static lung-volume markers. CONCLUSIONS: The MPT test showed good diagnostic accuracy as a simple screening tool for detecting exercise-induced DH in COPD. An absolute IC decrease ≥0.27 L after MPT may represent a potentially useful cross-sectional screening threshold, although further validation is needed before it can be interpreted as a definitive longitudinal threshold.
Inspiratory muscle fatigue and pulmonary deposition/perfusion imaging predict sleep dysfunction in long COVID: Evidence from MTC scintigraphy and FIT performance metrics.
Nóbrega Júnior JC, Soares Brandão S, Formiga MF … +9 more , Xavier D, Torres R, de Souza SN, Fink JB, Ari A, Reinaux C, Brandão D, Campos S, Dornelas de Andrade A
Respir Med · 2026 · PMID 42225171 · Publisher ↗
BACKGROUND: Post-COVID-19 syndrome may impair respiratory function, inspiratory muscle performance, and sleep quality; however, the interaction between inspiratory muscle fatigue, regional deposition/perfusion, and sleep...
BACKGROUND: Post-COVID-19 syndrome may impair respiratory function, inspiratory muscle performance, and sleep quality; however, the interaction between inspiratory muscle fatigue, regional deposition/perfusion, and sleep disturbances remains unclear. OBJECTIVE: To analyze associations between inspiratory muscle fatigue, pulmonary radiopharmaceutical activity, and sleep disturbances in symptomatic and asymptomatic post-COVID-19 individuals. METHODS: This cross-sectional study included 33 post-COVID-19 individuals classified as symptomatic (n = 23) or asymptomatic (n = 10) according to symptom severity. Inspiratory muscle performance was assessed using maximal inspiratory pressure (MIP), sustained maximal inspiratory pressure (SMIP), and the inspiratory fatigue index (FIT) obtained from an incremental respiratory resistance test. Sleep was assessed by actigraphy, the Pittsburgh Sleep Quality Index (PSQI), and the Epworth Sleepiness Scale (ESS). Pulmonary aerosol deposition and perfusion were assessed by gamma scintigraphy using ᵐTc-DTPA and ᵐTc-MAA, respectively; total radiopharmaceutical activity was quantified for both lungs combined and for the right and left lungs separately. RESULTS: Symptomatic individuals had lower MIP (73 [37] vs 114 [22.50] cmHO), SMIP (502 [222] vs 935.50 [215] PTU), and FIT (22.30 [9.20] vs 46.25 [20.28]; all p < 0.001). Total aerosol deposition (327.16 [232.97] vs 618.26 [187.88] Kct) and total lung perfusion (765.66 [269.94] vs 1046.94 [447.41] Kct) were reduced. PSQI (9 [5] vs 6.50 [6]; p = 0.006) and ESS (12 [5] vs 4 [4]; p = 0.003) were worse. FIT correlated with total aerosol deposition (r = 0.93; p < 0.001). CONCLUSIONS: Long COVID is associated with reduced inspiratory muscle performance, impaired ventilation/perfusion, and worse sleep, supporting FIT and pulmonary scintigraphy as potential functional markers for assessment and rehabilitation monitoring.
Testosterone treatment and respiratory medications and exacerbations in people prescribed inhaled corticosteroids: a nationwide cohort study.
Hansen ESH, Håkansson KEJ, Brustad N … +5 more , Meteran H, Jacobsen PA, McDonald VM, Juul A, Ulrik CS
Respir Med · 2026 · PMID 42217835 · Publisher ↗
BACKGROUND: After puberty asthma is more common and often more severe in females, indicating a potential role of sex steroids. Observational and genetic studies suggest that higher testosterone levels are associated with...
BACKGROUND: After puberty asthma is more common and often more severe in females, indicating a potential role of sex steroids. Observational and genetic studies suggest that higher testosterone levels are associated with improved lung function and reduced asthma risk, but evidence on the clinical efficacy of exogenous androgen supplementation is limited. We aimed to investigate changes in use of inhaled corticosteroid (ICS) and short-acting β2-agonist (SABA), and exacerbation rates among individuals treated with ICS who initiate systemic androgen treatment. METHODS: We conducted a nationwide, population-based register study comprising individuals treated with inhaled corticosteroid who initiated systemic testosterone (ATC G03B) between 1995 and 2022. Primary outcomes were changes in daily doses of inhaled corticosteroids, use of reliever medication, and exacerbation rates (defined as oral corticosteroid courses ≥125 mg or hospital admissions with obstructive airway disease). Outcomes were compared before and after androgen initiation using a within-subject design. RESULTS: A total of 858 male individuals on ICS (mean age 51 years) initiated testosterone therapy and were included. Their ICS dose was stable for the first 18 months after starting testosterone but decreased during months 18-24 (mean reduction 54 μg; SE 19; P = 0.03). SABA use increased among those >45 years (mean increase 111 puffs; SE 34; P = 0.009). Exacerbation rates did not change. CONCLUSION: Systemic testosterone treatment was associated with a reduction in ICS use after 18-24 months of therapy whereas use of SABA increased among older adults. These findings indicate a complex, age-dependent relationship between testosterone therapy and airway symptoms.
CHG index and carotid atherosclerosis in relatively young adults with obstructive sleep apnea: a cross-sectional study.
Han Y
Respir Med · 2026 Aug · PMID 42217834 · Publisher ↗
BACKGROUND: Obstructive sleep apnea (OSA) predisposes to cardiovascular disease through complex metabolic interactions. The Cholesterol-High-density lipoprotein-Glucose (CHG) index, integrating total cholesterol, HDL-C,...
BACKGROUND: Obstructive sleep apnea (OSA) predisposes to cardiovascular disease through complex metabolic interactions. The Cholesterol-High-density lipoprotein-Glucose (CHG) index, integrating total cholesterol, HDL-C, and fasting glucose, may serve as a novel metabolic biomarker for subclinical atherosclerosis in OSA. This cross-sectional study aimed to evaluate its association with carotid intima-media thickness (IMT). METHODS AND RESULTS: A total of 648 patients with OSA (mean age 37 years; range 21-58 years) were analysed. Using Least Absolute Shrinkage and Selection Operator (LASSO) regression, the CHG index, age, and hypertension were identified as key predictors of carotid IMT. Restricted cubic spline models adjusted for these covariates revealed a significant nonlinear association between the CHG index and carotid IMT (P for overall <0.001; P for nonlinearity = 0.001), which was more pronounced than the non-significant association observed for the AHI (P = 0.404). The association was particularly evident in patients with severe OSA (P for overall = 0.019 and 0.025, respectively). Logistic regression showed a synergistic effect: patients with both high CHG and severe OSA had the greatest odds of high carotid IMT (>0.8 mm; adjusted OR = 3.69, 95% CI: 1.26-13.63). CONCLUSION: The CHG index is an independent cross-sectional marker of subclinical atherosclerosis in OSA patients, with risk amplified by severe OSA. Due to the cross-sectional design, these findings do not imply that the CHG index can predict individual IMT progression or serve as a direct intervention target. Nevertheless, integrating the CHG index into OSA management could help identify patients at high cardiovascular risk. Future prospective studies are needed to establish temporality.
High ASA-PS as an independent predictor of refractoriness and poor prognosis in secondary spontaneous pneumothorax.
Nonomura R, Niikawa H, Yabe R … +6 more , Nagata H, Ueda K, Oshima Y, Sasaki T, Ishibashi N, Metoki H
Respir Med · 2026 Aug · PMID 42214726 · Publisher ↗
BACKGROUND: Secondary spontaneous pneumothorax (SSP) is a challenging disease with a poor prognosis, particularly in elderly patients with comorbidities, and its management is often difficult, sometimes leading to a refr...
BACKGROUND: Secondary spontaneous pneumothorax (SSP) is a challenging disease with a poor prognosis, particularly in elderly patients with comorbidities, and its management is often difficult, sometimes leading to a refractory state. This study aimed to identify independent predictors for refractory Persistant Air Leakage (R-PAL) and evaluate respiratory disease-specific survival. METHODS: We retrospectively reviewed 292 patients treated for SSP between 2018 and 2024. R-PAL was strictly defined as an air leak persisting for 7 days following a definitive procedure. To adjust for treatment selection bias, we incorporated the initial treatment modality into our multivariate logistic regression and Cox proportional hazards models, directly comparing the predictive values of ASA-PS and ECOG-PS. RESULTS: R-PAL occurred in 39 patients (13.4%). Multivariate logistic regression identified high ASA-PS (3) (Odds Ratio 6.13, p < 0.001) and non-surgical initial treatment (Odds Ratio 2.99, p = 0.007) as independent predictors of R-PAL. The ASA-PS model demonstrated superior fit and discrimination (AIC 183.4, AUC 0.84) compared to the ECOG-PS model. Furthermore, the 1-year respiratory disease-specific survival rate was significantly worse in the ASA-PS 3 group (67.4%) compared to the ASA-PS <3 group (98.0%). Cox analysis confirmed ASA-PS 3 as a significant independent predictor of mortality (Hazard Ratio 8.30, p = 0.007). CONCLUSIONS: High ASA-PS independently predicts both refractoriness and poor prognosis in SSP, outperforming ECOG-PS. These findings suggest that physiological organ reserve is more critical for pleural healing than daily activity levels, necessitating a multidisciplinary approach for high-risk patients.
Unmasking the genetic heterogeneity of cystic fibrosis in the Torbat Jam region: From common variants to large deletions and the challenge of missing heritability.
Ghaeninejhad H, DinMohammadi H, Tavakoli S … +1 more , Hamzehloei T
Respir Med · 2026 · PMID 42208689 · Publisher ↗
BACKGROUND: Cystic Fibrosis (CF) is an autosomal recessive disorder caused by mutations in the CFTR gene. The genetic spectrum of CF in Iran is highly heterogeneous due to ethnic diversity and consanguinity, particularly...
BACKGROUND: Cystic Fibrosis (CF) is an autosomal recessive disorder caused by mutations in the CFTR gene. The genetic spectrum of CF in Iran is highly heterogeneous due to ethnic diversity and consanguinity, particularly in the Khorasan province. Data regarding the Torbat Jam region is scarce. METHODS: We analyzed 7 unrelated patients with a clinical diagnosis of CF (sweat chloride >60 mmol/L). Genomic DNA was subjected to Sanger sequencing of all 27 CFTR exons. In cases with no identifiable mutations, Multiplex Ligation-dependent Probe Amplification (MLPA) was performed to detect Copy Number Variations (CNVs). Whole Exome Sequencing (WES) was utilized for patients who remained negative after Sanger and MLPA. This work has been approved with the Approval number: IR.ZUMS.BLC.1404-051. RESULTS: Mutations were identified in 4 out of 7 patients (57.1%). We detected the hallmark c.1521_1523delCTT (p.Phe508del) in a homozygous state in one patient and heterozygous in another. We identified the c.1210-11T > G (5T; TG12 variant) splice-site mutation in two compound heterozygous patients. Additionally, a missense mutation c.547C > A (p.Leu183Ile) was found. Notably, one patient with no sequence variants harbored a homozygous deletion of the entire Exon 2, detected via MLPA. Three patients (42.9%) remained genetically unresolved despite WES analysis. CONCLUSION: This study highlights a distinct mutational profile in Torbat Jam, characterized by severe alleles and large deletions. The high rate of unresolved cases despite WES suggests the involvement of deep intronic variants or extra-genic modifiers, necessitating Whole Genome Sequencing (WGS) for comprehensive diagnosis in this population.
Bridging the global survival gap in cystic fibrosis: Evidence from a 30-year longitudinal Mexican cohort.
Reyes-Apodaca M, Vázquez-Frias R, Núñez-Barrera I … +1 more , Lezana-Fernández JL
Respir Med · 2026 Aug · PMID 42208688 · Publisher ↗
BACKGROUND: Survival in cystic fibrosis (CF) has improved markedly in high-income countries over recent decades; however, important disparities persist across regions. Long-term data describing survival patterns and thei...
BACKGROUND: Survival in cystic fibrosis (CF) has improved markedly in high-income countries over recent decades; however, important disparities persist across regions. Long-term data describing survival patterns and their determinants in middle-income settings remain limited. The aim of this study was to estimate survival in Mexican patients with CF over three decades and to explore the clinical and health-system factors associated with mortality. METHODS: we conducted a retrospective longitudinal cohort study including patients with a confirmed diagnosis of CF followed at a national tertiary referral center in Mexico between 1990 and 2020. Survival was estimated using the Kaplan-Meier method. Potential predictors of mortality were evaluated through univariate analyses and multivariable Cox proportional hazards regression. Temporal changes in demographic, clinical, nutritional, microbiological, and treatment-related characteristics were also examined. RESULTS: a total of 341 patients were included. Median estimated survival was 20 years (95% CI 17.3-23.0) and appeared to improve over time. In multivariable analyses, early detection of P. aeruginosa ≤7 years, malnutrition, and reduced pulmonary function were associated with increased mortality, whereas access to complete standard CF treatment was associated with lower mortality. CONCLUSIONS: survival among Mexican patients with CF has improved over the past three decades but continues to lag that reported in high-income countries. The findings suggest that long-term outcomes are strongly influenced by modifiable clinical factors and access to comprehensive care. Strengthening multidisciplinary management, ensuring equitable treatment availability, and developing national CF registries may be essential steps toward reducing avoidable mortality and narrowing global survival disparities.
People with cystic fibrosis' experience of symptom burden while taking elexacaftor/tezacaftor/ivacaftor.
Muther EF, Gibson SL, Ford C … +7 more , Duncan C, Riekert KA, Lyons E, Hoppe JE, Prickett M, Isenburg MV, Everhart RS
Respir Med · 2026 Aug · PMID 42208687 · Publisher ↗
BACKGROUND: Despite improvements seen with elexacaftor/tezacaftor/ivacaftor (ETI), many people with CF (PWCF) still experience symptom burden. This study aimed to evaluate current perceived symptom burden and contrast it...
BACKGROUND: Despite improvements seen with elexacaftor/tezacaftor/ivacaftor (ETI), many people with CF (PWCF) still experience symptom burden. This study aimed to evaluate current perceived symptom burden and contrast it with PWCF's recollection of symptoms before starting ETI. METHODS: Participants were 367 PWCF, ages 7-74 years (M age = 26.2), in the "Your ETI" (yETI) study. PWCF or caregivers of children (7-12 years) completed the Change in Perceptions of Health on ETI (CHETI) survey which assessed how often they were bothered by symptoms (respiratory, gastrointestinal/nutrition, sinus/pain, sleep/fatigue, and mental health/cognitive symptoms) before starting ETI and in the past month. A change score was calculated and categorized as less bothered, stable, or more bothered. Age differences were investigated. RESULTS: Participants were on ETI for an average of 3.9 years. A notable percentage reported being less bothered by respiratory symptoms including cough (72%) and chest tightness (66%), and gastrointestinal symptoms including stomach pain/discomfort (48%), constipation, gas, bloating (46%), and difficulty gaining weight (51%). A smaller proportion indicated more bother related to weight gain (26%), increased appetite (23%), sleep difficulties (17%), and mental health/cognitive symptoms (14-22%). Participants reported the most current bother from constipation/gas/bloating (48%), anxiety (44%), sleep difficulties (44%), and anger/irritability (43%). Adults, notably those >36 years old, typically reported these symptoms more burdensome. CONCLUSIONS: While PWCF were bothered less by symptoms now compared to before starting ETI, some symptoms still caused a high degree of bother, particularly among 'older' adults with CF. Findings underscore the need for ongoing strategies to monitor and manage non-respiratory symptoms.
Sonographic pleural line excursion in children with bronchospasm: a prospective single-center study.
Scheier E, AbuKishk K
Respir Med · 2026 Aug · PMID 42203185 · Publisher ↗
Pediatric asthma scoring relies on subjective findings with poor interrater reliability. We prospectively studied children presenting to a pediatric emergency department with acute wheeze requiring beta-agonist therapy,...
Pediatric asthma scoring relies on subjective findings with poor interrater reliability. We prospectively studied children presenting to a pediatric emergency department with acute wheeze requiring beta-agonist therapy, measuring pleural line excursion (maximum minus minimum vertical displacement of the pleural line) from M-mode ultrasound video using a custom algorithm. Fifty children were analyzed. A multivariate logistic regression model constructed to predict hospital admission, combining excursion with peripheral capillary oxygen saturation (SpO), age-adjusted heart-rate percentile and age, achieved an area under the receiver operating characteristic curve (AUC) of 0.81. Among hypoxic children (SpO ≤91.5%), excursion ≤2.0 mm conferred a 73% admission rate versus 17% for excursion >2.0 mm. These exploratory findings require prospective validation.
Mucus plugs in COPD and cardiovascular mortality: An emerging risk pathway and the therapeutic potential of mucolytic strategies.
Cazzola M, Calzetta L, Parente V … +2 more , Rogliani P, Matera MG
Respir Med · 2026 Aug · PMID 42203184 · Publisher ↗
Airway mucus plugging is an underrecognized feature of COPD that is increasingly associated with adverse outcomes, including higher mortality rates. Computed tomography studies demonstrate that mucus plugs are prevalent,...
Airway mucus plugging is an underrecognized feature of COPD that is increasingly associated with adverse outcomes, including higher mortality rates. Computed tomography studies demonstrate that mucus plugs are prevalent, variable, and frequently persistent or dynamic over time. The presence of mucus plugs is independently linked to a worse prognosis, even after adjusting for cardiovascular and respiratory risk factors. Patients with mild COPD appear to be at particularly high risk, suggesting that mucus plugging may represent an early and potentially modifiable disease trait. Mucus plugging contributes to COPD progression through several mechanisms, including airflow obstruction, ventilation/perfusion mismatch, hypoxemia, airway inflammation, and increased susceptibility to infectious exacerbations. These exacerbations are strongly associated with acute cardiovascular events caused by systemic inflammation, endothelial dysfunction, and vascular stress. Additionally, chronic hypoxia and inflammatory signaling related to mucus dysfunction may independently increase cardiovascular risk. Within the "treatable traits" framework, mucus dysfunction is an important therapeutic target. Mucolytic and mucoactive therapies aim to improve mucus properties and clearance, which could reduce exacerbations and their systemic consequences. Some meta-analytic evidence suggests that, among thiol-based mucolytics, erdosteine is the most effective at reducing exacerbation frequency and duration. It also demonstrates antioxidant and anti-inflammatory effects that may influence systemic disease pathways. However, its comparative advantages over other agents remain uncertain. This narrative review summarizes the current evidence regarding the epidemiology, pathophysiology, and clinical implications of mucus plugging in COPD, with a focus on the proposed mucus plug-exacerbation-cardiovascular event axis and the potential role of mucus-directed therapies.
Impact of concurrent COPD and cardiovascular disease on mortality.
Lee HW, Kim SH, Rhee CK … +2 more , Kim DK, Choi JY
Respir Med · 2026 Aug · PMID 42203183 · Publisher ↗
BACKGROUND: Chronic obstructive pulmonary disease (COPD) and cardiovascular disease (CVD) frequently coexist and share common risk factors. However, the mortality burden associated with their co-occurrence has not been f...
BACKGROUND: Chronic obstructive pulmonary disease (COPD) and cardiovascular disease (CVD) frequently coexist and share common risk factors. However, the mortality burden associated with their co-occurrence has not been fully characterized at the population level, particularly with respect to cause-specific and absolute risks. METHODS: We conducted a population-based cohort study using data from UK Biobank. Participants were classified into four groups according to baseline COPD and CVD status. The primary outcome was all-cause mortality, and secondary outcomes included respiratory, cardiovascular, and lung cancer mortality. Multivariable Cox proportional-hazards models and competing-risk analyses were used to estimate adjusted hazard ratios (HRs). Absolute risks, risk differences, and numbers needed to harm (NNH) were calculated to quantify excess mortality burden. RESULTS: Among 293,948 participants followed for a median of 12.9 years, 3.9% had coexisting COPD and CVD at baseline. Compared to participants without either condition, those with both had a markedly increased risk of all-cause mortality (adjusted HR, 2.207 [95% confidence interval, 2.113-2.305]). Risks of respiratory-specific and cardiovascular-specific mortality were also substantially elevated. Although the COPD/CVD interaction was less than multiplicative on the HR scale, the absolute mortality burden was greatest among participants with both conditions. At 10 years, the excess absolute risk of all-cause mortality associated with coexisting COPD and CVD was 13.9 percentage points, corresponding to an NNH of 7.2. CONCLUSION: Coexisting COPD and CVD defines a distinct high-risk phenotype with a significantly increased absolute burden of all-cause and cause-specific mortality, underscoring the need for integrated cardiopulmonary risk management.
Acute COPD Exacerbation and long-term cardiopulmonary outcomes: Real-world EXACOS-CP evidence study in Israel.
Hayek S, Gottlieb U, Treves N … +8 more , Yahalom R, Rhodes K, Livnat I, Yarden A, Silverbeck J, Cabrera C, Zwas DR, Berkman N
Respir Med · 2026 Aug · PMID 42203182 · Publisher ↗
BACKGROUND: Chronic obstructive pulmonary disease (COPD) is a common and debilitating respiratory disease. Acute exacerbations of COPD (AECOPD), characterized by a sudden worsening of symptoms, are associated with cardio...
BACKGROUND: Chronic obstructive pulmonary disease (COPD) is a common and debilitating respiratory disease. Acute exacerbations of COPD (AECOPD), characterized by a sudden worsening of symptoms, are associated with cardiovascular (CV) morbidity and mortality. However, their impact on cardiopulmonary outcomes (CP) has not been evaluated in Israel. STUDY OBJECTIVE: To determine the association between AECOPD and risk of CP outcomes in newly diagnosed COPD patients and to estimate incidence rates across time intervals and exacerbation severity. STUDY DESIGN AND METHODS: This retrospective cohort study analyzed data from Clalit Health Services database. Patients aged ≥40 years who were newly diagnosed with COPD between 2010 and 2019 were included. Those who experienced a first moderate or severe AECOPD were compared to those without prior exacerbations. Matching (1:1) on COPD duration, and inverse probability weighting (IPW) further balanced baseline characteristics between groups. Cox proportional hazards models estimated hazard ratios (HRs) with 95% Confidence Interval (CI). RESULTS: The matched cohort comprised 51,480 patients. In the IPW-analysis, AECOPD was associated with an increased risk of composite CP event (HR 1.19; 95% CI: 1.16-1.23), CV events (HR 1.12; 95% CI: 1.08-1.16), respiratory outcomes (HR 1.35; 95% CI: 1.30-1.39), severe AECOPD (HR 1.51; 95% CI: 1.44-1.58), mechanical ventilation (HR 1.58; 95% CI: 1.43-1.75), and acute respiratory failure (HR 1.82; 95% CI: 1.65-2.01). CONCLUSIONS: Moderate or severe AECOPD significantly increases the risk of CP complications, especially respiratory events. These findings underscore the need for proactive, multidisciplinary management and close follow-up after an exacerbation.
Association between corticosteroid therapy during acute COVID-19 and Long COVID symptoms: a propensity score overlap-weighted analysis.
Fukunaga N, Asakura T, Terai H … +29 more , Tanaka H, Azekawa S, Iizuka S, Ozawa T, Nagao G, Nakagawara K, Morita A, Atsumi A, Yagi K, Kaji M, Ogata A, Konishi S, Matsuyama E, Ito F, Takaoka H, Shigematsu L, Shimada T, Otake S, Sunata K, Okada M, Fukushima T, Ohgino K, Masaki K, Miyata J, Japan COVID-19 Task Force, Hasegawa N, Chubachi S, Namkoong H, Fukunaga K
Respir Med · 2026 May · PMID 42190841 · Publisher ↗
BACKGROUND: The association between acute-phase corticosteroid therapy and subsequent Long COVID symptoms remains uncertain. Therefore, we aimed to evaluate the association between systemic corticosteroid therapy during...
BACKGROUND: The association between acute-phase corticosteroid therapy and subsequent Long COVID symptoms remains uncertain. Therefore, we aimed to evaluate the association between systemic corticosteroid therapy during hospitalization and patient-reported Long COVID symptoms during follow-up. METHODS: In this prospective multicenter observational study, we enrolled adults hospitalized with laboratory-confirmed severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection at 26 hospitals in Japan between January 2020 and February 2021. To account for evolving corticosteroid prescription practices, we used propensity score overlap weighting with calendar time adjustment relative to the RECOVERY trial announcement and fit overlap-weighted mixed-effects logistic regression models to estimate longitudinal associations across follow-up (3, 6, and 12 months). Additionally, symptom-specific outcomes were evaluated at 3 months. RESULTS: Among the 1044 participants, 373 received systemic corticosteroids and 671 did not. Corticosteroid therapy was associated with higher odds of any Long COVID symptom during follow-up (OR: 1.71; 95% CI: 1.12-2.63). In exploratory subgroup analyses stratified by oxygen requirement, no clear association was observed among patients who required oxygen therapy during hospitalization, whereas the association was more apparent among those who did not require oxygen therapy. At 3 months, corticosteroid therapy was associated with muscle weakness (OR: 2.43; 95% CI: 1.27-4.65). CONCLUSIONS: In this observational overlap-weighted analysis, acute-phase systemic corticosteroid therapy during COVID-19 hospitalization was associated with higher odds of patient-reported Long COVID symptoms during follow-up. However, these findings should not be interpreted as evidence against appropriately indicated corticosteroid therapy in patients with hypoxemic COVID-19 or respiratory failure.
The co-existence of asthma and neurodevelopmental or mental health disorders and its association with health-related outcomes in a national sample of Canadian children.
Lawson JA, Kim M, Jandaghi P … +9 more , Goodridge D, Balbuena L, Cockcroft D, Adamko D, Philipenko B, Pylypow J, Pavloff M, Ferro MA, Khanam U
Respir Med · 2026 Aug · PMID 42190840 · Publisher ↗
BACKGROUND: Asthma and neurodevelopmental and mental disorders (NMD) are associated. It is important to understand how having both asthma and a NMD is associated with health outcomes. We sought to determine if having bot...
BACKGROUND: Asthma and neurodevelopmental and mental disorders (NMD) are associated. It is important to understand how having both asthma and a NMD is associated with health outcomes. We sought to determine if having both asthma and a NMD was associated with poorer health outcomes in children. METHODS: We used data from the Canadian Health Survey of Children and Youth 2019, a cross-sectional, nationally representative Statistics Canada dataset. We compared health-related outcomes by condition status (no asthma or NMD; asthma only; NMD only; both asthma and NMD) in children (5-17 year old). RESULTS: Perceptions of health were lowest in the those with both asthma and a NMD while the likelihood of anxiety-like feelings and requiring/receiving services from a doctor were highest in those with both asthma and a NMD. Requiring/receiving services for NMD related outcomes were associated with NMD only or both asthma and a NMD (Odds ratios (OR) from 6.00 to 26.03, p < 0.05). Frequency of depression-like symptoms, functional difficulties, and lower academic achievement were also associated with both the NMD only and both asthma and a NMD groups. The presence of asthma only was associated with the frequency of anxiety-like and depression-like symptoms; requiring/receiving services for mental health, from a psychologist/counsellor, and for focusing/controlling behaviour. CONCLUSION: Having both asthma and a NMD was associated with worse well-being, functioning and requiring or receiving healthcare services. Results were strongest in those with a NMD or both asthma and a NMD suggesting that the presence of a NMD has an important role.
Guidance for chest-CT in children and adults with cystic fibrosis: A European perspective (part 2: special situations/respiratory complications).
Fayon M, Hill K, Riberi L … +21 more , Dournes G, Burgel PR, Messore B, Waldron M, Svedberg M, Lammertyn E, Fustik S, Gramegna A, Stahl M, Kerpel-Fronius A, Balbi M, Ciet P, Chassagnon G, Ferrero C, Sutharsan S, Opitz M, Andrinopoulou ER, Maher M, Duckers J, Tiddens H, Sermet-Gaudelus I
Respir Med · 2026 Aug · PMID 42190839 · Publisher ↗
This manuscript covers guidance proposed by The European Cystic Fibrosis Society Clinical Trials Network (ECFS-CTN) for the use of chest computed tomography (CT) scans in special situations such as haemoptysis, aspergill...
This manuscript covers guidance proposed by The European Cystic Fibrosis Society Clinical Trials Network (ECFS-CTN) for the use of chest computed tomography (CT) scans in special situations such as haemoptysis, aspergillus-related lung disease, non-tuberculous mycobacterial (NTM) infection and pneumothorax in people with CF. Guidance development followed a three-step process. The most important questions were first identified via anonymous online survey, then a comprehensive literature search was made to support a first set of statements. They were finally validated by a Delphi process to obtain collective opinion of participants. Consensus supported the use of chest CT in the presence of haemoptysis, Allergic Bronchopulmonary Aspergillosis, and NTM-positive cultures (>2 separate expectorated sputum samples, or 1 positive bronchoscopic result). The ultimate responsibility for justifying a chest CT scan lies with the healthcare professionals directly involved in patient management.
Accelerometry daily steps count is associated with overall survival in malignant pleural effusion patients.
Lopez P, Lam DCL, Muruganandan S … +7 more , Jeffery E, McVeigh JA, Chan KKP, Newton RU, Fitzgerald DB, Gary Lee YC, Peddle-McIntyre CJ
Respir Med · 2026 · PMID 42184886 · Publisher ↗
AIM: To investigate the association of accelerometry daily step count with 3-year overall survival (OS) in patients with malignant pleural effusion (MPE). METHODS: This is a pooled analysis of four studies in patients wi...
AIM: To investigate the association of accelerometry daily step count with 3-year overall survival (OS) in patients with malignant pleural effusion (MPE). METHODS: This is a pooled analysis of four studies in patients with MPE. Activity behaviour was captured using 7-day accelerometry (Actigraph GT3X+) and survival information was collected from medical records. Participants were divided in two groups based on median number of steps: high (n = 81, median of 7509 [interquartile range (IQR): 5699; 9844] steps) and low steps (n = 82, 2940 [IQR: 1676; 3947] steps). Data were analysed with Cox regression analyses (hazard ratio [HR] and 95% confidence intervals [95% CI]) and Kaplan-Meier method to explore the association of daily step count with 3-year OS. RESULTS: A total of 163 MPE patients had 57 and 72 deaths recorded in the high and low steps group, respectively. An unadjusted HR of 0.50 (95% CI: 0.35-0.71) was observed for those in the high steps group. High steps group presented a median 3-year OS of 18.9 (95% CI: 13.4-24.3) months vs. 9.3 (95% CI: 6.2-12.4) months in low steps (p < 0.001). An adjusted HR of 0.48 (95% CI: 0.31-0.74) was observed for patients presenting with high number of steps after controlling for age, cancer type, serum albumin, Eastern Cooperative Oncology Group performance status (0, 1, 2, and 3) and sedentary time. CONCLUSION: This exploratory study indicates accelerometry-based physical activity measurement is a meaningful prognostic marker in patients with MPE, even after controlling for established prognostic factors. Accelerometry may add value to existing prognostic models.
Diagnostic yield of gravity based diagnostic thoracentesis in suspected malignant pleural effusion (GBTIME): A pilot study.
Sharma P, Sivaramakrishnan P, Dahal S … +7 more , Banerjee S, Sanjan G, Singh A, Saini L, Dua R, Mishra M, Sindhwani G
Respir Med · 2026 Aug · PMID 42176822 · Publisher ↗
In malignant pleural effusion (MPE), malignant cells may settle in a gravity-dependent gradient due to the weight of cells. We aimed to compare diagnostic yield of cytology between aspirates from lower, upper and 5th int...
In malignant pleural effusion (MPE), malignant cells may settle in a gravity-dependent gradient due to the weight of cells. We aimed to compare diagnostic yield of cytology between aspirates from lower, upper and 5th intercostal spaces in suspected malignant moderate to massive effusion under transthoracic ultrasound guidance (TUS). This single-center, analytical, cross-sectional study was conducted over 6 months. 62 subjects with suspected MPE having moderate to massive effusion on TUS were asked to either sit or recline on bed at 45 - 90 inclination for 2 h. Under TUS guidance, diagnostic thoracentesis was done from lowermost space (2 intercostal spaces [ICS] above the level of diaphragm), uppermost space (1-2 ICS below highest level) and triangle of safety. Diagnostic yields for malignancy on cytology were compared between three sites. 36 subject's cytology returned positive for malignancy. The median lowest and highest ICS of aspiration was 7th and 4th ICS respectively. Lower space aspirate showed higher positivity (88.9%) compared to upper space (58.3%; Mcnemar critical value 3.84, p = 0.013). It showed poor agreement (k = -0.085 p = 0.473) to upper space and moderate level (k = 0.321, p = 0.025) with mid axillary line aspirate (72%, n = 26). Lung malignancies showed better lower space aspirate positivity than extra-pulmonary (p = 0.018). Pleural nodule, central/peripheral lung mass and cancer sub-type did not affect the diagnostic yield. No complications were noted in any subject. Diagnostic thoracentesis from lower ICS may provide higher cytological yield in suspected MPE, however, this must be done only under TUS guidance to prevent complications.
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