Baseline Pulmonary Function Research Articles

Long-term outcome of interstitial lung disease in patients with primary Sjögren's syndrome: a retrospective observational study.

Interstitial lung disease (ILD) is a potentially serious but underdiagnosed manifestation of primary Sjögren's syndrome (pSS). This observational study investigated the prevalence and clinical course of ILD in pSS, together with prognostic factors. A multicenter, retrospective longitudinal study was performed using findings from baseline and follow-up pulmonary function tests and chest computed tomography. Predisposing factors for the development of ILD and acute exacerbation (AE) were identified using a logistic regression model. The risk factors for a significant decline of pulmonary function were determined by the Cox proportional hazard model. A total of 1,306 patients with pSS were included in this study (female, 98%; mean age, 54 years). Among them, 79 patients with pSS were comorbid with ILD. ILD was more frequently found in male, older patients. Nonspecific interstitial pneumonia was the most prevalent imaging pattern in pSS-ILD (51%), followed by usual interstitial pneumonia (22%). At diagnosis with pSS-ILD, 54% of patients had restrictive pulmonary function, and 41% of patients initiated pharmacological treatment. During the median 4-year follow-up period, AE, a significant decline in pulmonary function, and death occurred in 19%, 29%, and 9% of patients with pSS-ILD, respectively. The neutrophil-to-lymphocyte ratio (NLR) increased 3 months prior to AE, and it was associated with AE. Older age at pSS-ILD diagnosis was a prognostic factor for a significant decline in pulmonary function. ILD accounted for 6% of the comorbidity of pSS. AE was associated with a significant decline in pulmonary function, and the NLR may predict AE.

Average volume-assured pressure support versus fixed pressure support in chronic hypercapnic respiratory failure: a systematic review and meta-analysis

Rationale: Chronic hypercapnic respiratory failure occurs due to alveolar hypoventilation resulting in carbon dioxide retention. This is commonly managed with noninvasive ventilation (NIV) with modalities including fixed pressure support and average volume-assured pressure support (AVAPS). However, there is limited information comparing outcomes with these two modes of ventilator support in the management of chronic hypercapnic respiratory failure. Objective: This review and meta-analysis analyze the outcomes with fixed pressure NIV versus average volume-assured pressure support NIV in managing chronic obstructive pulmonary disease (COPD) with chronic hypercapnic respiratory failure, focusing on patients’ perception of symptom burden and gas exchange based on arterial blood gases. Search methods: PubMed, Embase, Cochrane Central Register of Controlled Trials, and Web of Science databases were searched; the latest search date was December 1, 2023. Inclusion criteria: randomized control trials and crossover studies in English in adults over the age of 19 with the diagnosis of COPD and chronic hypercapnic respiratory failure. Exclusion criteria: patients less than 19 years old, patients with acute exacerbations, and patients with central respiratory failure or neuromuscular disease. Outcomes included blood gas analysis after use of NIV measured in mmHg and patient perception of mental health, symptom burden, and comfort. Results for each outcome were analyzed in RevMan using an inverse variance statistical method with a fixed effect analysis. The final analysis included 7 studies with 252 participants. Results: The patients were 64 ± 9 years old. Baseline pulmonary function testing showed a forced expiratory volume in the first second (FEV1) of 34.6 ± 14.2 % predicted, consistent with severe COPD per GOLD criteria, and a baseline PaCO2 of 55.2 ± 9.2 mmHg. Primary outcomes for ventilation showed no statistical difference between AVAPS and fixed pressure support groups in PaCO2 (Odds Ratio [OR] -1.51; 95% Confidence Interval [CI]: -3.18, 0.16; p=0.08). Patient perceived outcomes were evaluated using several questionnaires, including St. George Respiratory Questionnaire (SGRQ), Short Form 36 Health Survey Questionnaire (SF-36), and Visual Analogue Scale (VAS). Comparable results were not available for all studies, but no statistically significant differences were found when comparing study results. Conclusions: There was little or no clinically significant difference between fixed pressure support and AVAPS in gas exchange. There are inadequate data to draw conclusions about the effect of fixed pressure support compared to AVAPS on patient perceived outcomes, such as comfort and symptom burden. No studies evaluated mortality benefit, cost effectiveness, or hospitalizations. Key words: COPD, chronic hypercapnic respiratory failure, non-invasive ventilation, average volume-assured pressure support ventilation

Diagnosis of renal tumors in Birt-Hogg-Dube syndrome: Clinical presentation and risk factors in a single-center retrospective cohort

BackgroundBirt-Hogg-Dube (BHD) syndrome is a rare genetic condition associated with the development of renal tumors. This study aims to determine typical age ranges for detecting renal abnormalities, risk factors for tumor development, and long-term outcomes based on current surveillance strategies.MethodsA single-center multi-site retrospective cohort study was performed on all patients with BHD diagnosed from 2000 to 2023. Baseline demographics, pulmonary function, laboratory, radiologic, and histopathologic findings were collected. Logistic regression was used to assess predictor variables for the development of renal tumors with survival analysis evaluated from the date of BHD diagnosis to date of death or last known follow-up.ResultsThe study included 149 patients with BHD, 39 (26%) with diagnosed renal tumors, of which 28 had histopathologic confirmation. Mean age at renal tumor detection was 53.61 years. Older age and male sex were predictive of renal tumor development ((odds ratio 1.05; 95% CI, 1.01–1.08, P = 0.002) and (odds ratio 2.59; 95% CI, 1.17–5.73, P = 0.02), respectively). Time to all-cause mortality appeared shorter in those with renal tumors (Log-rank P = 0.02), though no deaths were from cancer or cancer-related complications.ConclusionsCurrent screening protocols for renal tumors in BHD suggest the most common presenting age range for presentation is late 40s to early 50s, with older age and male sex as risk factors for tumor development.

EP.08F.01 Predicting Radiation Pneumonitis with Baseline Pulmonary Function Tests in Patients with Locally Advanced Non-Small Cell Lung Cancer (NSCLC)

EP.08F.01 Predicting Radiation Pneumonitis with Baseline Pulmonary Function Tests in Patients with Locally Advanced Non-Small Cell Lung Cancer (NSCLC)

Quantitative computed tomography assessment of pulmonary function and compensation after lobectomy and segmentectomy in lung cancer patients.

The effect of different surgical methods on postoperative lung function in patients with lung cancer is still inconclusive. The main objective of this study was to compare the effects of video-assisted thoracic surgery (VATS) lobectomy and segmentectomy on postoperative pulmonary function and compensatory changes in patients undergoing lung cancer surgery. A total of 120 patients (82 VATS lobectomy, 38 VATS segmentectomy) were assessed for demographic characteristics, baseline pulmonary function, tumor volume, T stage, and histological grade. Postoperative pulmonary function and compensatory changes [percentage of the well-aerated lung (WAL) total (or unilateral) lung volume (LV) (WAL%) and non-operated lung (NOL)] were measured at multiple time points up to 2 years. Logistic regression analysis identified factors associated with WAL% decline after 1 year. Both VATS lobectomy and segmentectomy led to a decrease in pulmonary function, with no significant difference in the extent of decline between the two groups (all P>0.05). Lobectomy triggered a more pronounced compensatory response, characterized by increased ipsilateral NOL volume over time. Segmentectomy induced minimal compensatory changes and had a minimal impact on pulmonary function. Factors associated with decreased pulmonary ventilation after 1 year differed between the lobectomy and segmentectomy groups. In the lobectomy group, a higher preoperative WAL% of ipsilateral NOL [odds ratio (OR) =1.073; 95% confidence interval (CI): 1.017-1.133; P=0.01] was associated with a higher risk of decline in pulmonary function, whereas in the segmentectomy group, the only influencing factor was the preoperative contralateral mean lung density (MLD) (OR =0.932; 95% CI: 0.884-0.984; P=0.01). Both lobectomy and segmentectomy after lung cancer surgery result in a decrease in WAL%, with lobectomy demonstrating a stronger pulmonary compensatory capacity. The application of quantitative computed tomography (CT) has shown significant value in predicting postoperative pulmonary function preservation and compensatory changes, providing strong support for personalized surgical decision-making.

Impact of Postoperative Prolonged Air Leakage on Long-Term Pulmonary Function after Lobectomy for Lung Cancer.

This study aimed to evaluate the long-term impact of postoperative prolonged air leak (PAL) on pulmonary function. We enrolled 1,316 patients with pathologic stage I-III lung cancer who underwent lobectomy. The cohort was divided into 2 groups: those who experienced PAL (n=55) and those who did not (n=1,261). Propensity score matching was conducted at a 1:4 ratio, resulting in 49 patients in the PAL group and 189 in the non-PAL group. Changes in pulmonary function were compared among preoperative, 6-month postoperative, and 12-month postoperative measurements between the 2 groups. The variables used for propensity score matching included age, sex, smoking history, body mass index, baseline pulmonary function, pathologic stage, and surgical approach. All standardized mean differences were less than 0.1. Six months postoperatively, the PAL group showed a greater reduction in both forced expiratory volume in 1 second (FEV1) (-13.0% vs. -10.0%, p=0.041) and forced vital capacity (FVC) (-15.0% vs. -9.0%, p<0.001) than the non-PAL group. In cases of upper lobectomy, there were no significant differences in FEV1 changes between the PAL and non-PAL groups at both 6 and 12 months. However, in lower lobectomy, the PAL group demonstrated a more pronounced decrease in FEV1 (-14.0% vs. -11.0%, p=0.057) and FVC (-20.0% vs. -13.0%, p=0.006) than the non-PAL group at 6 months postoperatively. Postoperative PAL delayed the recovery of pulmonary function after lobectomy. These effects were markedly more pronounced after lower lobectomy than after upper lobectomy.

The Impact of a Diet Rich in Omega-3 Fatty Acids on the Quality of Life of Patients with Squamous Cell Lung Cancer and Comorbid Depression: A Retrospective Study.

Lung cancer is a significant health concern, and is often accompanied by comorbid depression, leading to worsened prognosis and decreased quality of life for patients. This study aimed to investigate the potential influence of a diet rich in Omega-3 fatty acids on the quality of life of patients with squamous cell lung cancer and comorbid depression. A retroactive analysis of clinical information from patients with squamous cell lung cancer and comorbid depression admitted to Hongqi Hospital Affiliated to Mudanjiang Medical University from June 2022 to June 2023 was conducted. The patients were classified into two groups on the basis of different dietary care approaches: the Routine Dietary Group and the Omega-3 Fatty Acids Group. Baseline characteristics, pulmonary function tests, dietary intake, depression scoring, and quality of life scores were compared between the two groups. 103 patients in total were included, with 51 in the Routine Dietary Group and 52 in the Omega-3 Fatty Acids Group. The Omega-3 Fatty Acids Group exhibited significantly higher ingestion of Omega-3 fatty acids in comparison with the Routine Dietary Group (3.15 ± 0.64 g/day vs. 2.93 ± 0.28 g/day, p = 0.022). Despite similar baseline pulmonary function tests, patients in the Omega-3 Fatty Acids Group showed significantly higher scores in physical (70.17 ± 4.81 vs. 68.18 ± 5.03, p = 0.043) and emotional (71.29 ± 4.58 vs. 69.38 ± 4.25, p = 0.030) functioning, as well as lower scores in insomnia (27.41 ± 4.51 vs. 29.34 ± 4.21, p = 0.027) and constipation (7.34 ± 1.66 vs. 8.43 ± 3.36, p = 0.040). The study provided insights into the potential impact of a diet rich in Omega-3 fatty acids on the quality of life of patients with squamous cell lung cancer and complicating depression, suggesting that dietary interventions emphasizing Omega-3 fatty acids may be conducive to improving physical and emotional functioning, as well as symptom management, in this patient population.

Comparison of Asthma Phenotypes in Severe Asthma Cohorts (SARP, U-BIOPRED, ProAR and COREA) From 4 Continents.

Asthma is a clinical syndrome with various underlying pathomechanisms and clinical phenotypes. Genetic, ethnic, and geographic factors may influence the differences in clinical presentation, severity, and prognosis. We compared the characteristics of asthma based on the geographical background by analyzing representative cohorts from the United States, Europe, South America, and Asia using the Severe Asthma Research Program (SARP), Unbiased Biomarkers for the Prediction of Respiratory Disease Outcomes (U-BIOPRED), Program for Control of Asthma in Bahia (ProAR), and Cohort for Reality and Evolution of Adult Asthma in Korea (COREA), respectively. The clinical characteristics and medications for the SARP (n = 669), U-BIOPRED (n = 509), ProAR (n = 996), and COREA (n = 3,748) were analyzed. Subgroup analysis was performed for severe asthma. The mean age was highest and lowest in the COREA and SARP, respectively. The asthma onset age was lowest in the ProAR. The mean body mass index was highest and lowest in the SARP and COREA, respectively. Baseline pulmonary function was lowest and highest in the U-BIOPRED and COREA, respectively. The number of patients with acute exacerbation in the previous year was highest in U-BIOPRED. The mean blood eosinophil count was highest in COREA. The total immunoglobulin E was highest in the ProAR. The frequency of atopy was highest in the SARP. The principal component analysis plot revealed differences among all cohorts. The cohorts from 4 different continents exhibited different clinical and physiological characteristics, probably resulting from the interplay between genetic susceptibility and geographical factors.

The impact of antifibrotic use on long-term clinical outcomes in the pulmonary fibrosis foundation registry

BackgroundIdiopathic pulmonary fibrosis (IPF) is a devastating interstitial lung disease (ILD) with a high mortality rate. The antifibrotic medications pirfenidone and nintedanib have been in use since 2014 for this disorder and are associated with improved rate of lung function decline. Less is known about their long-term outcomes outside of the clinical trial context.MethodsThe Pulmonary Fibrosis Foundation Patient Registry was used for this study. Patients with an IPF diagnosis made within a year of enrollment were included. The treated group was defined as patients receiving either pirfenidone or nintedanib for at least 180 days. The untreated group did not have any record of antifibrotic use. Demographic data, comorbidities, serial lung function, hospitalization, and vital status data were collected from the registry database. The primary outcomes were transplant-free survival, time to first respiratory hospitalization, and time to 10% absolute FVC decline. Time-to-event analyses were performed utilizing Cox proportional hazards models and the log-rank test. Model covariates included age, gender, smoking history, baseline lung function, comorbidities, and oxygen use.ResultsThe registry contained 1212 patients with IPF; ultimately 288 patients met inclusion criteria for the treated group, and 101 patients were designated as untreated. Patients treated with antifibrotics were significantly younger (69.8 vs. 72.6 years, p = 0.008) and less likely to have smoked (61.1% ever smokers vs. 72.3% never smokers, p = 0.04). No significant differences were seen in race, gender, comorbidities, or baseline pulmonary function between groups. The primary outcome of transplant-free survival was not significantly different between the two groups (adjusted HR 0.799, 95% CI 0.534–1.197, p = 0.28). Time to respiratory hospitalization was significantly shorter in the treated group (adjusted HR 2.12, 95% CI 1.05–4.30, p = 0.04). No significant difference in time to pulmonary function decline was seen between groups.ConclusionsThis multicenter study demonstrated 63% of newly diagnosed IPF patients had continuous antifibrotic usage. Antifibrotics were not associated with improved transplant-free survival or pulmonary function change but was associated with an increased hazard of respiratory hospitalization. Future studies should further investigate the role of antifibrotic therapy in clinically important outcomes in real-world patients with IPF and other progressive ILDs.

Proteomic profiling of bronchoalveolar lavage fluid uncovers protein clusters linked to survival in idiopathic forms of interstitial lung disease

BackgroundIdiopathic interstitial pneumonias (IIPs) such as idiopathic pulmonary fibrosis (IPF) and interstitial pneumonia with autoimmune features (IPAF), present diagnostic and therapeutic challenges due to their heterogeneous nature. This study aimed to identify intrinsic molecular signatures within the lung microenvironment of these IIPs through proteomic analysis of bronchoalveolar lavage fluid (BALF).MethodsPatients with IIP (n=23) underwent comprehensive clinical evaluation including pre-treatment bronchoscopy and were compared to controls without lung disease (n=5). Proteomic profiling of BALF was conducted using label-free quantitative methods. Unsupervised cluster analyses identified protein expression profiles which were then analyzed to predict survival outcomes and investigate associated pathways.ResultsProteomic profiling successfully differentiated IIP from controls.k-means clustering, based on protein expression revealed three distinct IIP clusters, which were not associated with age, smoking history, or baseline pulmonary function. These clusters had unique survival trajectories and provided more accurate survival predictions than the Gender Age Physiology (GAP) index (C-index 0.794versus0.709). The cluster with the worst prognosis featured decreased inflammatory signaling and complement activation, with pathway analysis highlighting altered immune response pathways related to immunoglobulin production and B cell-mediated immunity.ConclusionsThe unsupervised clustering of BALF proteomics provided a novel stratification of IIP patients, with potential implications for prognostic and therapeutic targeting. The identified molecular phenotypes underscore the diversity within the IIP classification and the potential importance of personalized treatments for these conditions. Future validation in larger, multi-ethnic cohorts is essential to confirm these findings and to explore their utility in clinical decision-making for patients with IIP.

Proteomic profiling of bronchoalveolar lavage fluid uncovers protein clusters linked to survival in idiopathic forms of interstitial lung disease.

Idiopathic interstitial pneumonias (IIPs) such as idiopathic pulmonary fibrosis (IPF) and interstitial pneumonia with autoimmune features (IPAF), present diagnostic and therapeutic challenges due to their heterogeneous nature. This study aimed to identify intrinsic molecular signatures within the lung microenvironment of these IIPs through proteomic analysis of bronchoalveolar lavage fluid (BALF). Patients with IIP (n=23) underwent comprehensive clinical evaluation including pre-treatment bronchoscopy and were compared to controls without lung disease (n=5). Proteomic profiling of BALF was conducted using label-free quantitative methods. Unsupervised cluster analyses identified protein expression profiles which were then analyzed to predict survival outcomes and investigate associated pathways. Proteomic profiling successfully differentiated IIP from controls. k-means clustering, based on protein expression revealed three distinct IIP clusters, which were not associated with age, smoking history, or baseline pulmonary function. These clusters had unique survival trajectories and provided more accurate survival predictions than the Gender Age Physiology (GAP) index (C-index 0.794 vs. 0.709). The cluster with the worst prognosis featured decreased inflammatory signaling and complement activation, with pathway analysis highlighting altered immune response pathways related to immunoglobulin production and B cell-mediated immunity. The unsupervised clustering of BALF proteomics provided a novel stratification of IIP patients, with potential implications for prognostic and therapeutic targeting. The identified molecular phenotypes underscore the diversity within the IIP classification and the potential importance of personalized treatments for these conditions. Future validation in larger, multi-ethnic cohorts is essential to confirm these findings and to explore their utility in clinical decision-making for patients with IIP.

Control status and follow-up of acute attacks in children with bronchial asthma with normal pulmonary ventilation function

To investigate the control status of bronchial asthma (referred to as "asthma") in school-age children with normal pulmonary ventilation function and the occurrence of acute attacks within 1 year of follow-up. A retrospective analysis was conducted on clinical data of 327 children aged 6-14 years with bronchial asthma and normal pulmonary ventilation function from April to September 2021. Based on the measured value of one second rate (FEV1/FVC), the children were divided into the ≥80% group (267 cases) and the <80% group (60 cases). The pulmonary ventilation function, asthma control level, and occurrence of acute attacks within 1 year were compared between the two groups. The baseline pulmonary ventilation function in the <80% group was lower than that in the ≥80% group, and the proportion of small airway dysfunction was higher than that in the ≥80% group (P<0.05). After standardized treatment for 1 year, the small airway function indices in the <80% group improved but remained lower than those in the ≥80% group (P<0.05). The rate of incomplete asthma control at baseline was 34.6% (113/327), and the asthma control level in the <80% group was lower than that in the ≥80% group (P<0.05). After standardized treatment for 1 year, the asthma control level in the <80% group remained lower than that in the ≥80% group, and the proportion of acute asthma attacks was higher than that in the ≥80% group (P<0.05). Approximately one-third of school-age children with asthma still have incomplete asthma control when their pulmonary ventilation function is normal. Among them, children with measured FEV1/FVC<80% have an increased risk of acute asthma attacks and require close follow-up and strengthened asthma management.

Is improved pulmonary vasodilation why endurance trained individuals have elevated diffusing capacity compared to untrained individuals?

Contrary to our traditional understanding, the pulmonary vasculature may adapt to endurance training. Improved pulmonary vasodilation may be the principal mediator for trained individuals who exhibit greater diffusing capacity (DLCO), capillary blood volume (VC; representing recruitment and distension) and membrane diffusing capacity (DM; representing recruitment) at rest and during exercise as compared to untrained. Potential functional differences may be investigated using pulmonary vasodilators ( e.g., oral sildenafil). The purpose of this study was to examine the effects of sildenafil on DLCO, VC, and DM, in trained and untrained participants. We hypothesized that sildenafil would increase rest and exercise DLCO, VC, and DM, with a lesser relative response in trained due to improved baseline pulmonary vascular function. In this randomized, double-blind, placebo-controlled cross-over design (NCT: 04985929), DLCO was measured at rest (supine and seated) and during cycle ergometry (60 Watts, 30%, 60%, and 90% of V̇O2peak). DLCO was conducted using the modified Roughton-Forster multiple FiO2 breath-hold technique over 6 visits, where participants were given either oral placebo or sildenafil (50 mg, single dose, 30 min wash-in). To date, 18 participants (6 female) (56% target sample size) have been recruited into two groups: trained (T) and untrained (UT) (Mean V̇O2peak = 66.6 ± 7.2 mL.kg−1.min−1 and 37.8 ± 3.2 mL.kg−1.min−1, respectively). Estimated PASP was reduced between placebo and sildenafil across all participants at rest (mean Δ = -2.7 mmHg; p&lt;0.01), indicating effective pulmonary vasodilation. Under placebo, T had higher DLCO at rest (supine), 30%, 60%, and 90% of V̇O2peak (p&lt;0.05), consistent with previous literature. There was no effect of sildenafil on DLCO, VC, or DM (all p&gt;0.200) or drug by workload interactions (all p&gt;0.189) in either group. These preliminary results suggest that pharmacological augmentation of the nitric oxide vasodilatory pathway does not increase DLCO regardless of training status. We speculate that this pathway may not be the primary driver responsible for the elevated DLCO observed in endurance trained participants. Natural Sciences and Engineering Research Council of Canada. This is the full abstract presented at the American Physiology Summit 2024 meeting and is only available in HTML format. There are no additional versions or additional content available for this abstract. Physiology was not involved in the peer review process.

P151 Nailfold capillaroscopy for prediction of novel severe organ involvement and mortality in systemic sclerosis

Abstract Background/Aims Nailfold capillaroscopy (NFC) has been suggested as a potential biomarker of disease severity in systemic sclerosis (SSc). Several studies report the association between capillary loss and disease severity however, the association of NFC abnormalities with novel severe organ involvement/progression and mortality in SSc has not been evaluated. We aim to evaluate the association of nailfold capillaroscopy (NFC) abnormalities with novel major organ involvement/progression and mortality in SSc. Methods Follow-up data from patients with SSc registered between 2000 and 2022 were analyzed. Patients underwent NFC at baseline. Baseline demographic data, antibody status, baseline pulmonary function, novel severe organ involvement/progression and mortality were registered. Novel organ involvement/progression was defined as new or progressive involvement of peripheral vasculature, lungs, heart, skin, gastrointestinal, kidney, musculoskeletal at 12 and 24 months of follow-up. The following NFC parameters were evaluated: capillary density, hemorrhages, enlarged and giant capillaries, avascular areas, organization of capillary architecture and scleroderma pattern (early/active/late). Logistic regression modelling was run to assess associations between NFC and clinical parameters, the occurrence of novel severe organ involvement and/or progression and mortality. Results 113 patients were included: 28 (25%) were male, 86 (76%) lcSSc and 27 (23%) dcSSc. 70 patients (61%) developed novel overall severe organ involvement/progression and 40 patients died (36%) during follow-up. Table 1 and table 2 summarizes the associations between NFC and novel severe organ involvement/progression and mortality during follow-up. Loss of capillary density was associated with overall severe organ involvement (p = 0.002), peripheral vascular involvement (p = 0.03), new ILD (p = 0.04) and skin progression (p = 0.01); avascular areas were associated with overall severe organ involvement (p = 0.03), new ILD (p = 0.03) and progression of ILD (p = 0.02) and scleroderma pattern was associated with overall severe organinvolvement (p = 0.03), peripheral vascular involvement (OR p = 0.04), new ILD (p = 0.004), progression of ILD (p = 0.03) and skin progression (p = 0.04). Mortality was associated with ILD (OR 2.56 CI 95% 1.5-7.2, p 0.04), DLCO &amp;lt;70% (2.34 CI 93% 1.42-6.23, p 0.03), a late pattern in NFC (3.42 95% CI 1.42-7.25, p 0.002), presence of hemorrhages (OR 1.77 95% CI 1.24-8.23, p0.034) and avascular zones (OR 6.22 95% CI 2.54-10.3, p 0.021). Survival rates from diagnosis at 10 years were lower in patients with NFC with active a late pattern (40% and 20%) compared with those with normal, unspecific and early pattern (100%, 90% and 70%) (p = 0.008 by log rank test) (figure 1). Conclusion NFC may be a potential biomarker in SSc for predicting novel severe organ involvement and/or progression and mortality. Abnormal capillary density, avascular areas and scleroderma pattern are predictors of overall severe organ involvement, peripheral vascular involvement, novel and progression of ILD and skin progression. Disclosure C. Sieiro Santos: None. P. Pérez García: None. J. Ordas Martínez: None. C. Álvarez Castro: None. E. Díez Álvarez: None.

Predicting Exacerbations in Alpha-1 Antitrypsin Deficiency Using Clinical and Pulmonary Function Tests: Portuguese EARCO Registry

Introduction: Exacerbations are common in individuals with alpha-1 antitrypsin deficiency (AATD)-related lung disease. This study intended to identify independent predictive factors for exacerbations in AATD using the Portuguese European Alpha-1 Research Collaboration (EARCO) registry. Methods: This study includes patients from the Portuguese EARCO registry, a prospective multicenter cohort (NCT04180319). From October 2020 to April 2023, this registry enrolled 137 patients, 14 of whom were excluded for analysis for either missing 12 months of follow-up or baseline pulmonary function. Results: Among the 123 AATD patients, 27 (22.0%) had at least one exacerbation in the last 12 months of follow-up. Patients with Pi*ZZ phenotype were three times more likely than the rest of the population to experience any exacerbation (32.7 vs. 14.1%, p = 0.014; OR 3.0). BODE index was significantly higher in exacerbators than in non-exacerbators (3.9 ± 2.4 vs. 1.3 ± 1.2; p < 0.001), including on multivariate analysis (p = 0.002). Similar results were found for BODEx (multivariate p < 0.001). DLCO was the only functional parameter independently associated with exacerbations (p = 0.024). Conclusions: DLCO, BODE, and BODEx were independent predictors of exacerbations at 12 months in AATD patients. Understanding these risk factors can aid decision-making on AATD-related lung disease management and improve patient outcomes.

A Review of Muscular Dystrophies.

Muscular dystrophy encompasses a group of genetic conditions with progressive muscle damage and weakness. Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) are X-linked recessive disorders that affect the production of the protein dystrophin. Emery-Dreifuss muscular dystrophy (EDMD) is typically an X-linked-recessive disorder involving the gene that codes for emerin. Facioscapulohumeral muscular dystrophy and oculopharyngeal muscular dystrophy (OPMD) are both autosomal dominant disorders. Although commonly mistaken as a condition in which patients are susceptible to malignant hyperthermia with volatile inhalational anesthetics, muscular dystrophy is more closely associated with rhabdomyolysis. Providers developing an anesthetic plan for dental patients with muscular dystrophy must take into consideration the patient's baseline cardiac and pulmonary function as well as the potential for abnormalities. Nondepolarizing neuromuscular blocker use is safe but likely to result in prolonged skeletal muscle relaxation. Succinylcholine and volatile anesthetics are generally contraindicated due to the risks of rhabdomyolysis and hyperkalemia with subsequent ventricular fibrillation, cardiac arrest, and death if left untreated. In-depth understanding of the more commonly encountered forms of muscular dystrophy is vital to providing safe and effective ambulatory anesthesia care for patients undergoing dental treatment outside the traditional hospital operating room setting.

Wedge resection, segmentectomy, and lobectomy: oncologic outcomes based on extent of surgical resection for ≤2 cm stage IA non-small cell lung cancer.

Long-standing controversy has existed over whether sublobar resection is an adequate oncological procedure for clinical stage IA non-small cell lung cancer (NSCLC) ≤2 cm, despite the recent randomized trial reports of Japanese Clinical Oncology Group (JCOG) 0802 and Cancer and Leukemia Group B (CALGB) 140503 demonstrating non-inferior outcomes with sublobar resection compared to lobectomy. As practice patterns shift, we sought to compare oncologic outcomes in patients with these early-stage tumors after wedge resection, segmentectomy, or lobectomy in a contemporary, real-world, cohort. A retrospective review of a prospectively maintained database from a single institution was conducted from 2011 to 2020 to identify all patients with clinically staged IA1 or IA2 NSCLC (tumors ≤2 cm with no nodal involvement). The primary outcomes of interest were overall survival (OS) and disease-free survival (DFS), with secondary outcomes of lung cancer-specific survival (LCSS), recurrence patterns, and perioperative morbidity and mortality. A total of 480 patients were identified; 93 (19.4%) patients underwent wedge resection, 90 (18.7%) received segmentectomy, and 297 (61.9%) underwent lobectomy. Patients who underwent wedge resection had worse Eastern Cooperative Oncology Group (ECOG) performance status (23.7% ECOG 1 or 2 vs. 5.6% among segmentectomy and 5.4% among lobectomy, P<0.05). Both wedge resection and segmentectomy patients had lower preoperative mean percentage of predicted forced expiratory volume in one second (%FEV1) compared to the lobectomy group (81.8% and 82.6% vs. 89.6%, P=0.002), a higher proportion of patients with chronic obstructive pulmonary disease (COPD) and interstitial lung disease (ILD), and a higher Charlson Comorbidity Index. There were no statistically significant differences in 5-year OS, DFS, or LCSS between groups: 90%, 61%, 78% for wedge resections compared with 85%, 75%, 86% for segmentectomy, and 87%, 77%, 87% for lobectomy, respectively. Recurrence was observed in 17 patients who underwent wedge resection (18.3%, 8 local, 9 distant), 12 patients who received segmentectomy (13.4%, 6 local, 6 distant), and 38 patients who underwent lobectomy (12.8%, 11 local, 27 distant), which was not significantly different (P=0.36). Patients with inferior performance status or lower baseline pulmonary function are more likely to receive wedge resection for clinical stage IA NSCLC ≤2 cm in size. For these small tumors, lobectomy, segmentectomy, and wedge resection provide comparable oncologic outcomes.

Factors that Influence the Need to Start Adaptive Radiotherapy.

Adaptive radiotherapy (ART) is an essential approach to account for anatomical and biological uncertainties. Adaptive radiotherapy is, however, time-consuming, and it is unclear which patients are eligible or when is the best time to start ART. This prospective study was conducted at Kasr El-Aini Center of Clinical Oncology and Nuclear Medicine, Cairo, Egypt from January 2019 to December 2020. Thirty patients with pathologically proven, limited-stage small cell or stage I-II non-small cell lung cancer who were either not fit for or refused surgery or had stage III disease were recruited and underwent treatment planning to receive 60 Gy on a conventional 3D conformal radiation schedule with platinum-based chemotherapy. All patients underwent computed tomography (CT) planning within 2 and 4 weeks of starting radiation therapy to assess the need for adaptation. Pulmonary function test and echocardiography findings were assessed at the end of treatment and at 3 and 6 months after treatment, and were compared to the baseline. We found a significant reduction in mean value of the planning target volume (PTV) in the CT scans at the second (331 cm3) and fourth (257 cm3) weeks of treatment as compared to baseline (342 cm3) (p-value < 0.0001). Adaptation decreased the dose to the organ at risk with statistical significance and with improvement of the target coverage. At week 2 of radiotherapy, the need for adaptation was correlated to the conformity index (p = 0.0473), esophageal V35 (p = 0.0488), esophageal V50 (p = 0.0295), and its mean dose (p = 0.0087). At week 4 it was correlated to forced expiratory volume in 1 second (FEV1) (p = 0.0303), ratio between the forced expiratory volume in 1 second and the forced vital capacity (FEV1/FVC) (p = 0.0024), and echocardiography (p = 0.0183). Conformity index and esophageal dose constraints can predict the need for adaptation at week 2, whereas baseline pulmonary function parameters and echocardiography can predict the need for adaptation at week 4 of radiotherapy.

Early-life pulmonary viral infection leads to long-term functional and lower airway structural changes in the lungs.

Early-life respiratory virus infections have been correlated with enhanced development of childhood asthma. In particular, significant numbers of respiratory syncytial virus (RSV)-hospitalized infants go on to develop lung disease. It has been suggested that early-life viral infections may lead to altered lung development or repair that negatively impacts lung function later in life. Our data demonstrate that early-life RSV infection modifies lung structure, leading to decreased lung function. At 5 wk postneonatal RSV infection, significant defects are observed in baseline pulmonary function test (PFT) parameters consistent with decreased lung function as well as enlarged alveolar spaces. Lung function changes in the early-life RSV-infected group continue at 3 mo of age. The altered PFT and structural changes induced by early-life RSV were mitigated in TSLPR-/- mice that have previously been shown to have reduced immune cell accumulation associated with a persistent Th2 environment. Importantly, long-term effects were demonstrated using a secondary RSV infection 3 mo following the initial early-life RSV infection and led to significant additional defects in lung function, with severe mucus deposition within the airways, and consolidation of the alveolar spaces. These studies suggest that early-life respiratory viral infection leads to alterations in lung structure/repair that predispose to diminished lung function later in life.NEW & NOTEWORTHY These studies outline a novel finding that early-life respiratory virus infection can alter lung structure and function long-term. Importantly, the data also indicate that there are critical links between inflammatory responses and subsequent events that produce a more severe pathogenic response later in life. The findings provide additional data to support that early-life infections during lung development can alter the trajectory of airway function.

Impact of Smoking Cessation Therapy on Pulmonary Function: Identification of Factors Predicting Improvement of Forced Expiratory Volume in 1s

Background: Stopping smoking has beneficial effects on pulmonary function, but few studies have investigated which factors predict a favorable outcome. Methods: We analyzed the results of a prospective 3-month smoking cessation program. Smokers who had quit were defined as those abstaining from smoking between visits at 8 and 12 weeks (verified by exhaled carbon monoxide ≤10 ppm). Baseline pulmonary function was compared with that at the end of the program.