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Genetic analysis pre-lung transplantation diagnosed a case of hereditary pulmonary alveolar proteinosis (PAP) complicated by fibrosis in adulthood. The need for genetic testing in GM-CSF autoantibody negative and unclassifiable PAP is highlighted. https://bit.ly/3QcsYwM., Competing Interests: Conflict of interest: S.A. Papiris reports grants or contracts as the Savara Impala 2 Trial Primary Investigator, outside the submitted work; payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events from Boehringer Ingelheim, DEMO, Hoffman la Roche and Elpen, outside the submitted work; and support for attending meetings and/or travel from Boehringer Ingelheim, outside the submitted work. Conflict of interest: R. Borie reports receiving consulting fees from Boehringer Ingelheim, Sanofi and Ferrer, outside the submitted work; payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events from Boehringer Ingelheim, Sanofi and Ferrer, outside the submitted work; support for attending meetings and/or travel from Boehringer Ingelheim, outside the submitted work; and participation on a data safety monitoring or advisory board for Savara, outside the submitted work. Conflict of interest: N. Nathan reports grants or contracts from the Orphan Disease Center, Genetic Basis of Neuroendocrine Cell Hyperplasia of Infancy (principal investigator (PI)), French Research and Innovation Grant 2023 (CORTICO-NEHI; PI) and Chancellerie des universités (PI), outside the submitted work; participation on a data safety monitoring or advisory board for Research and Innovation AP-HP, France (member), outside the submitted work; leadership or fiduciary role in other board, society, committee or advocacy group, paid or unpaid for a Clinical Research Collaboration on chILDEU of the European Respiratory Society (2022–present). Conflict of interest: M. Griese reports Boehringer Ingelheim support for an adjudication board, outside the submitted work. Conflict of interest: E.D. Manali reports grants or contracts as a Savara Impala 2 Trial Subinvestigator, outside the submitted work; payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events from Boehringer Ingelheim, CSL Behring, Hoffman la Roche and Elpen, outside the submitted work; and support for attending meetings and/or travel from Boehringer Ingelheim, outside the submitted work. Conflict of interest: The remaining authors have nothing to disclose., (Copyright ©The authors 2023.)

Aim: Inhaled corticosteroid (ICS)/long-acting β 2 agonist (LABA) combination therapy is used for the effective control of asthma. Aim of this study was to collect data on the effectiveness, safety, quality of life, and patient satisfaction from a fixed dose combination of budesonide/formoterol administered with the Elpenhaler ® device following 3-months' treatment. Methods: A 3-month real-life, multicentre, one-arm, prospective observational study (SKIRON study-NCT03055793) was conducted, using the following questionnaires: Asthma Control Questionnaire (ACQ-6) for asthma control assessment, MiniAQLQ questionnaire for QoL assessment, and Feeling of Satisfaction with Inhaler questionnaire (FSI-10) for patients' satisfaction with the inhaler device. Comorbidities and safety data were also recorded during the study. Results: We enrolled 1,174 asthmatic patients following standard clinical practice in primary care from 126 sites in urban and rural areas of Greece. The majority of patients (71.5%) had at least one comorbidity. A statistically significant improvement in the ACQ-6 score was noted at 3 months compared to the baseline evaluation (mean ± SD 2.19 ± 0.97 at baseline vs. 0.55 ± 0.56 at 3 months; mean change -1.64 (95%CI -1.69, -1.57), p  < 0.0001). MiniAQLQ score was statistically and clinically significantly improved, compared to baseline, (4.55 ± 1.04 at baseline vs. 6.37 ± 0.64 at 3 months; mean change 1.82 (95%CI 1.75, 1.87), p  < 0.0001). The mean FSI-10 score of 44.2 ± 5.4 indicated patient satisfaction and ease-of-use of the Elpenhaler ® device. Conclusions: In this large real-world study of inadequately-controlled asthma patients in primary care settings, the treatment with budesonide/formoterol FDC with the Elpenhaler ® device was associated with significant improvement in patients' asthma control and quality of life.

Objectives: This study aimed to investigate whether positive serum autoantibodies (AAbs) have any impact on survival and time evolution of radiological findings and pulmonary function indices in patients with interstitial lung disease (ILD)., Patients and Methods: Ninety four patients with regular clinical, functional and high resolution computed tomography (HRCT) imaging follow-up for at least 12 consecutive months and complete testing for a panel of AAbs most commonly associated with ILD were enrolled in this retrospective two-center study. Eligible patients were divided into two groups based on the presence [ILD/AAb(+)] (n = 69) or absence [ILD/AAb(-)] (n = 25) of positive serum AAbs. All-cause mortality and longitudinal indicators of ILD progression such as a sustained decrease from baseline in absolute measurements of forced vital capacity (FVC) of ≥10% or single-breath diffusion capacity (DLCO SB ) of ≥15% were the primary study endpoints. DLCO SB  < 40% predicted on at least two consecutive measurements and progression of HRCT findings were our secondary endpoints. Kaplan-Meier (K-M) survival analysis and multivariate Cox proportional-hazards (PH) model were used to evaluate the prognostic significance of positive AAbs in the outcome of patients with ILD., Results: ILD/AAb(+) patients were predominantly female (71% vs 32%), were significantly younger (54.8 ± 14.6 vs 66.8 ± 10.1 years), and had longer duration of follow-up (78.1 ± 53.1 vs 41.6 ± 26.7 months), compared with ILD/AAb(-) patients (p < .01 for each comparison). Baseline measurements of FVC (% pred.) and DLCO SB (% pred.) did not differ significantly between the two groups. At the end of follow-up, mortality rates and the percentage of patients with a sustained FVC decrease were lower in the ILD/AAb(+) group (p < .05 for each comparison). With the exception of DLCO SB  < 40% pred., ILD/AAb(+) patients had a longer median time-to-event for each of the other studied outcomes (p < .01 for each K-M analysis). In addition, Cox PH models adjusted for age, smoking status, baseline pulmonary function tests and morphological pattern of ILD remained statistically significant in favor of the ILD/AAb(+) group (p < .05 for each comparison)., Conclusions: AAb(+) patients with ILD seem to have a more favorable prognosis regarding all-cause mortality, long-term deterioration in lung function parameters and progression of HRCT findings than their AAb (-) counterparts., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Background: Depression is a frequent comorbidity in COPD patients and is associated with greater physical impairment, increased health-care utilization, and worse outcomes. The presence of depressive symptoms in the partners of COPD patients has not been evaluated., Methods: We evaluated the partners of 230 consecutive COPD patients included in a prospective study. Depressive symptoms were evaluated using Beck's Depression Inventory (BDI) on the first day of admission for COPD exacerbation. Patients were followed-up for 1 year., Results: Significant depressive symptoms were present in 39.6 % of the COPD patients and in 40.9 % of their partners. Beck scores were higher in the partners of patients with severe airflow obstruction and in those with ≥2 exacerbations and ≥1 hospitalizations for COPD exacerbation during the 1-year follow-up. The BDI score of the patients' partners was significantly correlated with the BDI score of the COPD patients (r s = 0.422). In multivariate analysis, depressive symptoms in the COPD patients were an independent predictor of depressive symptoms in their partners (OR 4.136, 95 % CI 1.991-8.594; p < 0.001)., Conclusions: A large proportion of the partners of COPD patients present significant depressive symptoms. The identification of those patients and their partners represents a possible target for intervention.

Background: Respiratory symptoms are usually underestimated in patients with chronic kidney disease undergoing maintenance hemodialysis. Therefore, we set out to investigate the prevalence of patients chronic dyspnea and the relationship of the symptom to lung function indices., Methods: Twenty-five clinically stable hemodialysis patients were included. The mMRC dyspnea scale was applied before and after hemodialysis. Spirometry, single breath nitrogen test, arterial blood gases, static maximum inspiratory (P(imax)) and expiratory (P(emax)) muscle pressures, and mouth occlusion pressure (P 0.1) were also measured., Results: Despite normal spirometry, all patients (100%) reported mild to moderate degree of chronic dyspnea pre which was reduced after hemodialysis. The sole predictor of (Δ) mMRC was the (Δ) P 0.1 (r = 0.71, P < 0.001). The P(imax) was reduced before and correlated with the duration of hemodialysis (r = 0.614, P < 0.001), whilst after the session it was significantly increased (P < 0.001). Finally (Δ) weight was correlated with the (Δ) P(imax) %pred (r = 0.533, P = 0,006) and with the (Δ) CV (%pred) (r = 0.65, P < 0.001)., Conclusion: We conclude that dyspnea is the major symptom among the CKD patients that improves after hemodialysis. The neuromechanical dissociation observed probably is one of the major pathophysiologic mechanisms of dyspnea.

The impact of depressive symptoms on outcomes of acute exacerbations of chronic obstructive pulmonary disease (AECOPD) has not been thoroughly evaluated in prospective studies. We prospectively enrolled 230 consecutive patients hospitalised for AECOPD, without previous diagnosis of depression. Depressive symptoms were evaluated with Beck's depression inventory. Pulmonary function tests, arterial blood gases, COPD assessment test (CAT) and Borg dyspnoea scale were recorded on admission and on days 3, 10 and 40. Patients were evaluated monthly for 1 year. Patients with depressive symptoms required longer hospitalisation (mean ± sd 11.6 ± 3.7 versus 5.6 ± 4.1 days, p<0.001). Clinical variables improved during the course of AECOPD, but depressive symptoms on admission had a significant impact on dyspnoea (p<0.001) and CAT score (p = 0.012) improvement. Patients with depressive symptoms presented more AECOPD (p<0.001) and more hospitalisations for AECOPD (p<0.001) in 1 year. In multivariate analysis, depressive symptoms were an independent predictor of mortality (hazard ratio 3.568, 95% CI 1.302-9.780) and risk for AECOPD (incidence rate ratio (IRR) 2.221, 95% CI 1.573-3.135) and AECOPD hospitalisations (IRR 3.589, 95% CI 2.319-5.556) in 1 year. The presence of depressive symptoms in patients admitted for AECOPD has a significant impact on recovery and is related to worse survival and increased risk for subsequent COPD exacerbations and hospitalisations in 1 year.

Background. Cognitive deterioration may impair COPD patient's ability to perform tasks like driving vehicles. We investigated: (a) whether subclinical neuropsychological deficits occur in stable COPD patients with mild hypoxemia (PaO(2) > 55 mmHg), and (b) whether these deficits affect their driving performance. Methods. We recruited 35 stable COPD patients and 10 normal subjects matched for age, IQ, and level of education. All subjects underwent an attention/alertness battery of tests for assessing driving performance based on the Vienna Test System. Pulmonary function tests, arterial blood gases, and dyspnea severity were also recorded. Results. COPD patients performed significantly worse than normal subjects on tests suitable for evaluating driving ability. Therefore, many (22/35) COPD patients were classified as having inadequate driving ability (failure at least in one of the tests), whereas most (8/10) healthy individuals were classified as safe drivers (P = 0.029). PaO(2) and FEV1 were correlated with almost all neuropsychological tests. Conclusions. COPD patients should be warned of the potential danger and risk they face when they drive any kind of vehicle, even when they do not exhibit overt symptoms related to driving inability. This is due to the fact that stable COPD patients may manifest impaired information processing operations.

Background: The slope of phase III (single breath nitrogen test), an index of ventilation inhomogeneity, has been used for early detection of COPD. Tidal airway closure (cyclic opening and closure of the peripheral airways during tidal breathing; AC(T)) and expiratory flow limitation (attainment of maximal expiratory flow during tidal expiration; EFL(T)) cause small airways disease (SAD). The relationships of these indices with COPD severity may reflect the progress from SAD to overt COPD., Methods: In this cross-sectional study we have assessed for the first time the phase III slope, AC(T) and EFL(T) in 10 smokers with normal spirometry (group O) and 40 COPD patients with GOLD scores from I to IV., Results: In most group O smokers the phase III slope was increased, and further increased with GOLD severity (up to 800%pred in GOLD IV). A close correlation was found of slope with GOLD (r = 0.77). AC(T) was absent in smokers with normal spirometry and in most patients with mild COPD. EFL(T) first appeared in GOLD II patients and its prevalence progressively increased in GOLD III and IV patients., Conclusions: Most group O smokers exhibit increased phase III. With overt COPD there is a progressive increase in phase III and reduction of FEV(1)/FVC ratio from GOLD I to IV. A reduction of FEV(1) occurs from GOLD stage II. As the disease progresses from moderate to severe, there is an increasing presence of AC(T). Tidal EFL, with dynamic hyperinflation and severe dyspnea is present only in GOLD III and IV.