Assessing the quality of forced vital capacity measurement in patients with systemic sclerosis.

INTRODUCTION: Forced vital capacity (FVC) is an important tool for monitoring lung functions in patients with systemic sclerosis (SSc). However, several disease manifestations may influence the quality of FVC test in SSc. We aimed to assess the quality of FVC measurements according to current guidelines in patients with SSc and determine the factors that may affect results. METHOD: In this cross-sectional study, SSc patients and age/sex matched controls underwent spirometry. Quality of FVC measurements were graded according to updated American Thoracic Society (ATS) and European Respiratory Society (ERS) guidelines. Demographics, clinical features and parameters that may affect FVC test quality were compared between SSc patients with high and low quality FVC test. RESULTS: 98 SSc patients (90 female) and 100 controls were included. The rate of high quality FVC measurement in SSc patients was significantly lower in SSc patients compared to controls. (80 % vs 60.2 % p = 0.002). Among SSc patients; diffuse disease, ILD, anti-topoisomerase 1 antibody positivity, immunosuppressive use, flexion contractures of hands, reduced mouth opening and decreased chest expansion were more frequent in patients with low quality FVC (p < 0.05 for all). Patients with muscle weakness and medium/high risk of malnutrition were also numerically higher in low quality FVC group. Presence of more than one condition that may affect FVC quality was significantly higher among patients with low quality FVC. CONCLUSION: A significant percent of SSc patients had low quality FVC measurement. Physicians should be aware of this point while interpreting FVC test results especially in SSc patients with more than one condition that may affect the quality of the test.

The Effects of a Pulmonary Rehabilitation Programme on Functional Capacity and Strength of Respiratory Muscles in Patients with Post-COVID Syndrome.

Aim: The aim of this study was to estimate the effects of a pulmonary rehabilitation programme (PR) on the functional capacity and respiratory muscle strength of patients with post-COVID syndrome. Methods: A cross-sectional study was conducted using hospital data on patients who participated in a pulmonary rehabilitation programme at the Clinic for Lung Diseases, University Hospital Centre Zagreb, Croatia, between January 2021 and December 2022. Data on the spirometry, respiratory muscle strength, and functional exercise capacity of patients were collected at baseline and three weeks after the start of rehabilitation. The study included 80 patients (43 females, 37 males) with a mean age of 51±10 years. Results: A significant increase in respiratory muscle strength (P<0.001) was observed after pulmonary rehabilitation, with effect sizes ranging from small to large (Cohen's d from 0.39 to 1.07), whereas the effect for PImax expressed as a percentage was large (Cohen's d=0.99). In addition, the pulmonary rehabilitation programme significantly improved the parameters of the six-minute walk test in patients, and the parameters of lung function, FVC, FEV1, and DLCO also improved significantly after PR (P<0.05). Conclusion: The results showed that the pulmonary rehabilitation programme has clinically significant effects on functional capacity and respiratory muscle strength in patients with post-COVID syndrome.

Association of temperature and absolute humidity with incidence of exercise-induced bronchoconstriction in children.

AIM: Exercise test outdoors is widely used to diagnose asthma in children, but it is unclear how much outdoor air factors affect the results. METHODS: We analysed 321 outdoor exercise challenge tests with spirometry in children 6-16 years conducted due to suspicion of asthma or for assessing the effect of medication on asthma. We studied the association of FEV1 decrease and incidence of exercise-induced bronchoconstriction (EIB) with temperature, relative humidity (RH) and absolute humidity (AH). RESULTS: Asthma was diagnosed in 57% of the subjects. AH ≥5 g/m3, but not RH or temperature, was associated with the EIB incidence (p = 0.035). In multivariable logistic regression, AH ≥5 g/m3 was negatively associated (OR = 0.51, 95% CI [0.28─0.92], p = 0.026) while obstruction before exercise (OR = 2.11, 95% CI [1.16─3.86], p = 0.015) and IgE-mediated sensitisation were positively associated with EIB (OR = 2.24, 95% CI [1.11─4.51], p = 0.025). AH (r = -0.12, p = 0.028) and temperature (r = -0.13, p = 0.023) correlated with decrease in FEV1. In multivariable linear regression, only AH was associated with FEV1 decrease (coefficient = -0.044, 95% CI [-0.085 to -0.004], p = 0.033). CONCLUSION: AH of outdoor air associates with occurrence and severity of EIB in outdoor exercise tests in children. Care should be taken when interpreting negative outdoor exercise test results if AH of air is high.

Risk factors predicting need for the pediatric intensive care unit (PICU) post-hematopoietic cell transplant, PICU utilization, and outcomes following HCT: a single center retrospective analysis.

Hematopoietic cell transplant (HCT) is a curative treatment for multiple malignant and non-malignant disorders. While morbidity and mortality have decreased significantly over the years, some patients still require management in the pediatric intensive care unit (PICU) during their HCT course for additional respiratory, cardiovascular, and/or renal support. We retrospectively reviewed pediatric patients (0-18 years) who underwent HCT from January 2015-December 2020 at our institution to determine risk factors for PICU care and evaluate PICU utilization and outcomes. We also assessed pulmonary function testing (PFT) data to determine if differences were noted between PICU and non-PICU patients as well as potential evolution of pulmonary dysfunction over time. Risk factors of needing PICU care were lower age, lower weight, having an underlying inborn error of metabolism, and receiving busulfan-based conditioning. Nearly half of PICU encounters involved use of each of respiratory support types including high-flow nasal cannula, non-invasive positive pressure ventilation, and mechanical ventilation. Approximately one-fifth of PICU encounters involved renal replacement therapy. Pulmonary function test results largely did not differ between PICU and non-PICU patients at any timepoint aside from individuals who required PICU care having lower DLCO scores at one-year post-HCT. Future directions include consideration of combining our data with other centers for a multi-center retrospective analysis with the goal of gathering and reporting additional multi-center data to work toward continuing to decrease morbidity and mortality for patients undergoing HCT.

Clinician Barriers to Ordering Pulmonary Function Tests for Adults with Suspected Asthma.

BACKGROUND: Primary care clinicians do not adhere to national and international guidelines recommending pulmonary function testing (PFTs) in patients with suspected asthma. Little is known about why that occurs. Our objective was to assess clinician focused barriers to ordering PFTs. METHODS: An internet-based 11-item survey of primary care clinicians at a large safety-net institution was conducted between August 2021 and November 2021. This survey assessed barriers and possible electronic health record (EHR) solutions to ordering PFTs. One of the survey questions contained an open-ended question about barriers which was analyzed qualitatively. RESULTS: The survey response rate was 59% (117/200). The top 3 reported barriers included beliefs that testing will not change management, distance to testing site, and the physical effort it takes to complete testing. Clinicians were in favor of an EHR intervention to prompt them to order PFTs. Responses to the open-ended question also conveyed that objective testing does not change management. DISCUSSION: PFTs improve diagnostic accuracy and reduce inappropriate therapies. Of the barriers we identified, the most modifiable is to educate clinicians about how PFTs can change management. That in conjunction with an EHR prompt, which clinicians approved of, may lead to guideline congruent and improved quality in asthma care.

Diagnostic utility of spirometry for children with induced laryngeal obstruction or chronic non-specific cough.

PURPOSE: To determine the diagnostic utility of spirometry in distinguishing children with Induced Laryngeal Obstruction (ILO) or chronic non-specific cough (a.k.a. tic cough) from those with mild or moderate to severe asthma. METHODS: Retrospective cross sectional design. Children diagnosed with ILO (N = 70), chronic non-specific cough (N = 70), mild asthma (N = 60), or moderate to severe asthma (N = 60) were identified from the electronic medical record of a large children's hospital. Spirometry was completed before ILO, non-specific cough, or asthma diagnoses were made by pediatric laryngologists or pulmonologists. Spirometry was performed following American Thoracic Society guidelines and was interpreted by a pediatric pulmonologist. Forced Vital Capacity (FVC), Forced Expiratory Volume in 1 Second (FEV1), FEV1/FVC Ratio (FEV1/FVC), Forced Mid-Expiratory Flow 25--75 % (FEF25-75%), pulmonologist interpretation of flow volume loops, and overall exam findings were extracted from the medical record. RESULTS: Ninety seven percent of children with ILO or chronic non-specific cough presented with spirometry values within normative range. Patients with ILO, non-specific cough, and mild asthma presented with FVC, FEV1, FEV1/FVC, and FEF25-75% values in statistically similar range. Children with moderate to severe asthma presented with significantly reduced FVC (p < .001), FEV1 (p < .001), FEV1/FVC (p < .001), and FEF25-75% (p < .001) values when compared with patients in the other groups. Flow volume loops were predominantly normal for children with ILO and non-specific cough. CONCLUSIONS: Findings indicate that ILO and chronic non-specific cough can neither be diagnosed nor differentiated from mild asthma using spirometry alone. Spirometry should therefore be used judiciously with this population, bearing in mind the limitations of the procedure. Future research should determine the most effective and efficient ways of delineating ILO and non-specific cough from other respiratory conditions in children.

Application of the ERS/ATS Spirometry Standards and Race-Neutral Equations in the COPDGene Study.

RATIONALE: The European Respiratory Society (ERS) and the American Thoracic Society (ATS) recommend using z-scores, and the ATS has recommended using Global Lung Initiative (GLI)- "Global" race-neutral reference equations for spirometry interpretation. However, these recommendations have been variably implemented and the impact has not been widely assessed, both in clinical and research settings. OBJECTIVES: We evaluated the ERS/ATS airflow obstruction severity classification. METHODS: In the COPDGene Study (n = 10,108), airflow obstruction has been defined as a forced expiratory volume in one second to forced vital capacity (FEV1/FVC) ratio <0.70, with spirometry severity graded from class 1 to 4 based on race-specific percent predicted (pp) FEV1 cut-points as recommended by the Global Initiative for Chronic Obstructive Lung Disease (GOLD). We compared the GOLD approach, using NHANES III race-specific equations, to the application of GLI-Global equations using the ERS/ATS definition of airflow obstruction as FEV1/FVC ratio < lower limit of normal (LLN) and z-FEV1 cut-points of -1.645, -2.5, and -4 ("zGLI Global"). We tested the four-tier severity scheme for association with COPD outcomes. MEASUREMENTS AND MAIN RESULTS: The lowest agreement between ERS/ATS with zGLI Global and the GOLD classification was observed in individuals with milder disease (56.9% and 42.5% in GOLD 1 and 2) and race was a major determinant of redistribution. After adjustment for relevant covariates, zGLI Global distinguished all-cause mortality risk between normal spirometry and the first grade of COPD (Hazard Ratio 1.23, 95% CI 1.04-1.44, p=0.014), and showed a linear increase in exacerbation rates with increasing disease severity, in comparison to GOLD. CONCLUSIONS: The zGLI Global severity classification outperformed GOLD in the discrimination of survival, exacerbations, and imaging characteristics.

Impact of Isolated Exercise-Induced Small Airway Dysfunction on Exercise Performance in Professional Male Cyclists.

BACKGROUND: Professional cycling puts significant demands on the respiratory system. Exercise-induced bronchoconstriction (EIB) is a common problem in professional athletes. Small airways may be affected in isolation or in combination with a reduction in forced expiratory volume at the first second (FEV1). This study aimed to investigate isolated exercise-induced small airway dysfunction (SAD) in professional cyclists and assess the impact of this phenomenon on exercise capacity in this population. MATERIALS AND METHODS: This research was conducted on professional cyclists with no history of asthma or atopy. Anthropometric characteristics were recorded, the training age was determined, and spirometry and specific markers, such as fractional exhaled nitric oxide (FeNO) and immunoglobulin E (IgE), were measured for all participants. All of the cyclists underwent cardiopulmonary exercise testing (CPET) followed by spirometry. RESULTS: Compared with the controls, 1-FEV3/FVC (the fraction of the FVC that was not expired during the first 3 s of the FVC) was greater in athletes with EIB, but also in those with isolated exercise-induced SAD. The exercise capacity was lower in cyclists with isolated exercise-induced SAD than in the controls, but was similar to that in cyclists with EIB. This phenomenon appeared to be associated with a worse ventilatory reserve (VE/MVV%). CONCLUSIONS: According to our data, it appears that professional cyclists may experience no beneficial impacts on their respiratory system. Strenuous endurance exercise can induce airway injury, which is followed by a restorative process. The repeated cycle of injury and repair can trigger the release of pro-inflammatory mediators, the disruption of the airway epithelial barrier, and plasma exudation, which gradually give rise to airway hyper-responsiveness, exercise-induced bronchoconstriction, intrabronchial inflammation, peribronchial fibrosis, and respiratory symptoms. The small airways may be affected in isolation or in combination with a reduction in FEV1. Cyclists with isolated exercise-induced SAD had lower exercise capacity than those in the control group.

Association of pulmonary function test abnormalities and quality-of-life measures after COVID-19 infection.

BACKGROUND: Long-COVID is a multisystem disease that can lead to significant impairments in health-related quality of life (HRQoL). Following COVID-19 infection, abnormalities on pulmonary function tests (PFT) are common. The primary aim of this study is to evaluate for any correlation between PFT abnormalities and impairment in HRQoL scores following COVID-19 infection. METHODS: This is an analysis of a prospective cohort of patients in Louisville, KY who were infected with COVID-19. Data collected included demographics, past medical history, laboratory tests, PFTs, and several HRQoL questionnaires such as the EuroQol 5 Dimension HRQoL questionnaire (EQ-5D-5 L), Generalized Anxiety Disorder 7 (GAD-7), Patient Health Questionnaire (PHQ-9), and Posttraumatic stress disorder checklist for DSM-5 (PCL-5). Descriptive statistics were performed, comparing PFTs (normal vs abnormal) and time since COVID-19 infection (3- vs 6- vs ≥ 12 months). RESULTS: There were no significant differences in FEV1, FVC, or the percentage of patients with abnormal PFTs over time after COVID-19 infection. Following COVID-19, patients with normal PFTs had worse impairment in mobility HRQoL scores and change in GAD-7 scores over time. There were no differences over time in any of the HRQoL scores among patients with abnormal PFTs. CONCLUSIONS: Among patients with an abnormal PFT, there was no temporal association with HRQoL scores as measured by EQ-5D-5 L, GAD-7, PHQ-9, and PCL-5. Among patients with a normal PFT, mobility impairment and anxiety may be associated with COVID-19 infection. Following COVID-19 infection, impairment in HRQoL scores is not completely explained by the presence of abnormalities on spirometry.

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.

Resolving the Complexity: A Comprehensive Review on Carbon Monoxide Diffusion Capacity in Chronic Obstructive Pulmonary Disease Patients.

This review comprehensively examines the intricate relationship between carbon monoxide (CO) diffusion capacity and chronic obstructive pulmonary disease (COPD). COPD, comprising chronic bronchitis and emphysema, presents a substantial global health burden, necessitating a nuanced understanding of pulmonary function parameters for effective diagnosis and management. The review delves into the physiological underpinnings, measurement techniques, and factors influencing CO diffusion capacity, emphasizing its pivotal role in assessing alveolar gas exchange efficiency. Key findings elucidate correlations between altered diffusion capacity and the severity of COPD, providing clinicians with valuable insights into disease progression. Considerations of age, gender, and ethnic disparities in normal CO diffusion capacity values highlight the importance of personalized interpretations. The clinical implications extend beyond diagnosis, influencing COPD management and prognostication, with potential applications in predicting treatment response. The review outlines avenues for future research, including advancements in measurement technologies and the exploration of novel imaging modalities and biomarkers. Ultimately, this review serves as a foundation for refining diagnostic approaches and treatment strategies and enhancing patient care in the dynamic landscape of COPD.

Calculated Maximal Volume Ventilation (cMVV) as a Marker of Early Respiratory Failure in Amyotrophic Lateral Sclerosis (ALS).

Respiratory failure assessment is among the most debatable research topics in amyotrophic lateral sclerosis (ALS) clinical research due to the wide heterogeneity of its presentation. Among the different pulmonary function tests (PFTs), maximal voluntary ventilation (MVV) has shown potential utility as a diagnostic and monitoring marker, able to capture early respiratory modification in neuromuscular disorders. In the present study, we explored calculated MVV (cMVV) as a prognostic biomarker in a center-based, retrospective ALS population belonging to the Piemonte and Valle d'Aosta registry for ALS (PARALS). A Spearman's correlation analysis with clinical data and PFTs showed a good correlation of cMVV with forced vital capacity (FVC) and a moderate correlation with some other features such as bulbar involvement, ALSFRS-R total score, blood oxygen (pO2), carbonate (HCO3-), and base excess (BE), measured with arterial blood gas analysis. Both the Cox proportional hazard models for survival and the time to non-invasive ventilation (NIV) measurement highlighted that cMVV at diagnosis (considering cMVV(40) ≥ 80) is able to stratify patients across different risk levels for death/tracheostomy and NIV indication, especially considering patients with FVC% ≥ 80. In conclusion, cMVV is a useful marker of early respiratory failure in ALS, and is easily derivable from standard PFTs, especially in asymptomatic ALS patients with normal FVC measures.

Clinical and functional outcomes associated with pulmonary complications after coronary artery bypass grafting.

INTRODUCTION: Coronary artery bypass grafting(CABG) is a surgical treatment for coronary artery disease aiming at improving symptoms and life expectancy. Despite this, there are pulmonary and functional complications that may arise during the postoperative period due to invasive mechanical ventilation(IMV), cardiopulmonary bypass and immobility, leading to longer hospital stays. OBJECTIVE: To evaluate the clinical and functional outcomes related to pulmonary complications in the postoperative period of CABG. METHODS: Prospective cohort. During the ICU stay the patients were divided into: Non Complicated Group(NCG) who did not present complications and Complicated Group(CG) who presented complication. Functional variables were applied as the six-minute walk test(6MWT), gait speed, sit up and stand up test, Timed Up and Go, peripheral muscle strength, ventilatory, pulmonary function and Functional Independence Measure. These tests were applied preoperatively, at ICU discharge, hospital discharge and six months after surgery. RESULTS: The study evaluated 90 patients, 59 in the NCG and 31 CG. In the 6MWT there was a 2%(p = 0.43) decrease in the NCG, while the decrease was 13%(p < 0.01) in the CG. In the MRC the drop was 2%(p = < 0.01) in the CNG, while in the CG the drop was 14%(p = < 0.01). In MIP the NCG had a 6%(p = 0.67) decrease, while the CG had a 16%(p = < 0.01) decrease. CONCLUSION: Patients with postoperative complications of CABG may have reduced functional performance, muscle strength, and pulmonary function at hospital discharge and after six months.

Remotely supervised spirometry versus laboratory-based spirometry during the COVID-19 pandemic: a retrospective analysis.

BACKGROUND: The COVID-19 pandemic has constrained access to spirometry, and the inherent risk of infectious transmission during aerosol-generating procedures has necessitated the rapid development of Remotely Supervised Spirometry (RSS). This innovative approach enables patients to perform spirometry tests at home, using a mobile connected spirometer, all under the real-time supervision of a technician through an online audio or video call. METHODS: In this retrospective study, we examined the quality of RSS in comparison to conventional Laboratory-based Spirometry (LS), using the same device and technician. Our sample included 242 patients, with 129 undergoing RSS and 113 participating in LS. The RSS group comprised 51 females (39.5%) with a median age of 37 years (range: 13-76 years). The LS group included 63 females (55.8%) with a median age of 36 years (range: 12-80 years). RESULTS: When comparing the RSS group to the LS group, the percentage of accurate Forced Expiratory Volume in one second (FEV1) measurements was 78% (n = 101) vs. 86% (n = 97), p = 0.177; for Forced Vital Capacity (FVC) it was 77% (n = 99) vs. 82% (n = 93), p = 0.365; and for both FEV1 and FVC, it was 75% (n = 97) vs. 81% (n = 92), p = 0.312, respectively. CONCLUSIONS: Our findings demonstrate no significant difference in the quality of spirometry testing between RSS and LS, a result that held true across all age groups, including patients aged over 65 years. The principal advantages of remote spirometry include improved access to pulmonary function tests, reduced infectious risk to curtail disease spread, and enhanced convenience for patients.

Pulmonary Function Tests Post-Stroke. Correlation between Lung Function, Severity of Stroke, and Improvement after Respiratory Muscle Training.

Stroke is a significant cause of mortality and chronic morbidity caused by cardiovascular disease. Respiratory muscles can be affected in stroke survivors, leading to stroke complications, such as respiratory infections. Respiratory function can be assessed using pulmonary function tests (PFTs). Data regarding PFTs in stroke survivors are limited. We reviewed the correlation between PFTs and stroke severity or degree of disability. Furthermore, we reviewed the PFT change in stroke patients undergoing a respiratory muscle training program. We searched PubMed until September 2023 using inclusion and exclusion criteria in order to identify studies reporting PFTs post-stroke and their change after a respiratory muscle training program. Outcomes included lung function parameters (FEV1, FVC, PEF, MIP and MEP) were measured in acute or chronic stroke survivors. We identified 22 studies of stroke patients, who had undergone PFTs and 24 randomised controlled trials in stroke patients having PFTs after respiratory muscle training. The number of patients included was limited and studies were characterised by great heterogeneity regarding the studied population and the applied intervention. In general, PFTs were significantly reduced compared to healthy controls and predicted normal values and associated with stroke severity. Furthermore, we found that respiratory muscle training was associated with significant improvement in various PFT parameters and functional stroke parameters. PFTs are associated with stroke severity and are improved after respiratory muscle training.

Functional Progression after Dose Suspension or Discontinuation of Nintedanib in Idiopathic Pulmonary Fibrosis: A Real-Life Multicentre Study.

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a chronic interstitial lung disease with rapidly progressive evolution and an unfavorable outcome. Nintedanib (NTD) is an antifibrotic drug that has been shown to be effective in slowing down the progression of the disease. The aim of our study was to examine the efficacy, especially in terms of the functional decline, and the safety profile of NTD in patients treated with the recommended dose and subjects who reduced or suspended the therapy due to the occurrence of adverse reactions. METHODS: We conducted a real-life retrospective study based on the experience of NTD use in two centers between 2015 and 2022. Clinical data were evaluated at baseline, at 6 and 12 months after the NTD introduction in the whole population and in subgroups of patients who continued the full-dose treatment, at a reduced dosage, and at the discontinuation of treatment. The following data were recorded: the demographic features, IPF clinical features, NTD therapeutic dosage, tolerability and adverse events, pulmonary function tests (PFTs), the duration of treatment upon discontinuation, and the causes of interruption. RESULTS: There were 54 IPF patients who were included (29.6% females, with a median (IQR) age at baseline of 75 (69.0-79.0) years). Twelve months after the introduction of the NTD therapy, 20 (37%) patients were still taking the full dose, 11 (20.4%) had reduced it to 200 mg daily, and 15 (27.8%) had stopped treatment. Gastrointestinal intolerance predominantly led to the dose reduction (13.0%) and treatment cessation (20.4%). There were two deaths within the initial 6 months (3.7%) and seven (13.0%) within 12 months. Compared to the baseline, the results of the PFTs remained stable at 6 and 12 months for the entire NTD-treated population, except for a significant decline in the DLCO (% predicted value) at both 6 (38.0 ± 17.8 vs. 43.0 ± 26.0; p = 0.041) and 12 months (41.5 ± 15.3 vs. 44.0 ± 26.8; p = 0.048). The patients who continued treatment at the full dose or a reduced dosage showed no significant differences in the FVC and the DLCO at 12 months. Conversely, those discontinuing the NTD exhibited a statistically significant decline in the FVC (% predicted value) at 12 months compared to the baseline (55.0 ± 13.5 vs. 70.0 ± 23.0; p = 0.035). CONCLUSIONS: This study highlights the functional decline of the FVC at 12 months after the NTD initiation among patients discontinuing therapy but not among those reducing their dosage.

Referral rates and barriers to lung transplantation based on pulmonary function criteria in interstitial lung diseases: a retrospective cohort study.

BACKGROUND: Interstitial lung diseases (ILD) unresponsive to medical therapy often require lung transplantation (LTx), which prolongs quality of life and survival. Ideal timing for referral for LTx remains challenging, with late referral associated with significant morbidity and mortality. Among other criteria, patients with ILD should be considered for LTx if forced vital capacity (FVC) is less than 80% or diffusion capacity for carbon monoxide (DLCO) is less than 40%. However, data on referral rates are lacking. OBJECTIVES: To evaluate referral rates for LTx based on pulmonary function tests (PFTs) and identify barriers associated with non-referral. DESIGN: A single-center retrospective cohort study. METHODS: The study consisted of ILD patients who performed PFT between 2014 and 2020. Patients with FVC < 80% or a DLCO < 40% were included in the study. Patients with absolute contraindications to LTx were excluded. Referral rates were computed, and a comparison was made between referred and non-referred subjects. RESULTS: Out of 114 ILD patients meeting criteria for referral to LTx, 35 were referred (30.7%), and 7 proceeded to undergo LTx. Median time from PFT to referral for assessment was 255 days [interquartile range (IQR) 35-1077]. Median time from referral to LTx was 89 days (IQR 59-143). Referred patients were younger (p = 0.003), had lower FVC (p < 0.001), DLCO (p < 0.001), and a higher rate of pulmonary hypertension (p = 0.04). Relatively better PFT, and older age, were significantly associated with non-referral of patients. CONCLUSION: There is under-referral of ILD patients who are eligible for LTx, which is associated with severe disease and missed opportunities for LTx. Further research is required to validate these findings.

Lung transplants: addressing referral gaps for lung disease patientsPatients with severe lung diseases that are unresponsive to medical treatments often require lung transplants to enhance their quality of life and survival. Determining the optimal timing for considering a transplant is challenging, as delaying it can lead to complications. Our study aimed to assess how frequently individuals with lung problems, particularly interstitial lung diseases, were referred for lung transplants based on lung function tests. We conducted a retrospective analysis of medical records for patients with lung diseases who underwent lung function tests between 2014 and 2020. We selected patients whose test results indicated impaired lung function, excluding those who were ineligible for lung transplants due to other medical reasons. Subsequently, we examined the number of patients referred for a lung transplant and compared them to those who were not referred. Our findings revealed that out of 114 patients eligible for a lung transplant, only 35 were referred, representing a referral rate of approximately 31%. Among these, only 7 patients actually underwent the transplant procedure. The time elapsed between the lung function test and the referral for a transplant assessment was notably long, averaging around 255 days. Additionally, once referred, patients waited an average of 89 days for the transplant assessment. Referred patients tended to be younger and had more severe lung disease, characterized by lower lung function test results and a higher likelihood of pulmonary hypertension. Conversely, patients who were not referred generally enjoyed better overall health and were older. This discrepancy highlights the missed opportunities for patients to improve their health and quality of life through lung transplantation. Further research is essential to verify the accuracy of these findings, but this study represents a crucial step toward ensuring that individuals with lung diseases receive the appropriate care they require.

Monitoring of Sarcoidosis.

This article focuses on the monitoring of pulmonary sarcoidosis. The monitoring of sarcoidosis is, in part, focused on serial change in major organ involvement but also includes diagnostic re-evaluation and review of change in quality of life. Recent criteria for progression of fibrotic interstitial lung disease are adapted to pulmonary sarcoidosis. The frequency and nature of monitoring are discussed, integrating baseline risk stratification and strategic treatment goals. Individual variables used to identify changes in pulmonary disease severity are discussed with a focus on their flaws and the need for a multidimensional approach. Other key monitoring issues are covered briefly.

Optimizing lung cancer radiation therapy: A systematic review of multifactorial risk assessment for radiation-induced lung toxicity.

BACKGROUND: Radiation therapy (RT) is essential in treating advanced lung cancer, but may lead to radiation pneumonitis (RP). This systematic review investigates the use of pulmonary function tests (PFT) and other parameters to predict and mitigate RP, thereby improving RT planning. METHODS: A systematic review sifted through PubMed and on BioMed Central, targeting articles from September 2005 to December 2022 containing the keywords: Lung Cancer, Radiotherapy, and pulmonary function test. RESULTS: From 1153 articles, 80 were included. RP was assessed using CTCAEv.4 in 30 % of these. Six studies evaluated post-RT quality of life in lung cancer patients, reporting no decline. Patients with RP and chronic obstructive pulmonary disease (COPD) generally exhibited poorer overall survival. Notably, forced expiratory volume in one second (FEV1) and diffusing capacity of the lung for carbon monoxide (DLCO) declined 24 months post-RT, while forced vital capacity (FVC) stayed stable. In the majority of studies, age over 60, tumors located in the lower part of the lung, and low FEV1 before RT were associated with a higher risk of RP. Dosimetric factors (V5, V20, MLD) and metabolic imaging emerged as significant predictors of RP risk. A clinical checklist blending patient and tumor characteristics, PFT results, and dosimetric criteria was proposed for assessing RP risk before RT. CONCLUSION: The review reveals the multifactorial nature of RP development following RT in lung cancer. This approach should guide individualized management and calls for a prospective study to validate these findings and enhance RP prevention strategies.

Estimating the effect of nintedanib on forced vital capacity in children and adolescents with fibrosing interstitial lung disease using a Bayesian dynamic borrowing approach.

BACKGROUND: The rarity of childhood interstitial lung disease (chILD) makes it challenging to conduct powered trials. In the InPedILD trial, among 39 children and adolescents with fibrosing ILD, there was a numerical benefit of nintedanib versus placebo on change in forced vital capacity (FVC) over 24 weeks (difference in mean change in FVC % predicted of 1.21 [95% confidence interval: -3.40, 5.81]). Nintedanib has shown a consistent effect on FVC across populations of adults with different diagnoses of fibrosing ILD. METHODS: In a Bayesian dynamic borrowing analysis, prespecified before data unblinding, we incorporated data on the effect of nintedanib in adults and the data from the InPedILD trial to estimate the effect of nintedanib on FVC in children and adolescents with fibrosing ILD. The data from adults were represented as a meta-analytic predictive (MAP) prior distribution with mean 1.69 (95% credible interval: 0.49, 3.08). The adult data were weighted according to expert judgment on their relevance to the efficacy of nintedanib in chILD, obtained in a formal elicitation exercise. RESULTS: Combined data from the MAP prior and InPedILD trial analyzed within the Bayesian framework resulted in a median difference between nintedanib and placebo in change in FVC % predicted at Week 24 of 1.63 (95% credible interval: -0.69, 3.40). The posterior probability for superiority of nintedanib versus placebo was 95.5%, reaching the predefined success criterion of at least 90%. CONCLUSION: These findings, together with the safety data from the InPedILD trial, support the use of nintedanib in children and adolescents with fibrosing ILDs.