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Options to achieve oral corticosteroid (OCS)-sparing have been triggering increasing interest since the 1970s because of the side-effects of OCSs, and this has now become achievable with biologics. The Société de Pneumologie de Langue Française workshop on OCSs aimed to conduct a comprehensive review of the basics for OCS use in asthma and issue key research questions. Pharmacology and definition of regular use were reviewed by the first working group (WG1). WG2 examined whether regular OCS use is associated with T2 endotype. WG3 reported on the specificities of the paediatric area. Key "research statement proposals" were suggested by WG4. It was found that the benefits of regular OCS use in asthma outside episodes of exacerbations are poorly supported by the existing evidence. However, complete OCS elimination couldn't be achieved in any available studies for all patients and the panel felt that it was too early to conclude that regular OCS use could be declared criminal. Repeated or prolonged need for OCS beyond 1 g·year-1 should indicate the need for referral to secondary/tertiary care. A strategic sequential plan aiming at reducing overall exposure to OCS in severe asthma was then held as a conclusion of the workshop. [ABSTRACT FROM AUTHOR]

Chronic obstructive pulmonary disease (COPD) is characterised by nonreversible proximal bronchial obstruction leading to major respiratory disability. However, patient phenotypes better capture the heterogeneously reported complaints and symptoms of COPD. Recent studies provided evidence that classical bronchial obstruction does not properly reflect respiratory disability, and symptoms now form the new paradigm for assessment of disease severity and guidance of therapeutic strategies. The aim of this review was to explore pathways addressing COPD pathogenesis beyond proximal bronchial obstruction and to highlight innovative and promising tools for phenotyping and bedside assessment. Distal small airways imaging allows quantitative characterisation of emphysema and functional air trapping. Micro-computed tomography and parametric response mapping suggest small airways disease precedes emphysema destruction. Small airways can be assessed functionally using nitrogen washout, probing ventilation at conductive or acinar levels, and forced oscillation technique. These tests may better correlate with respiratory symptoms and may well capture bronchodilation effects beyond proximal obstruction. Knowledge of inflammation-based processes has not provided well-identified targets so far, and eosinophils probably play a minor role. Adaptative immunity or specific small airways secretory protein may provide new therapeutic targets. Pulmonary vasculature is involved in emphysema through capillary loss, microvascular lesions or hypoxia-induced remodelling, thereby impacting respiratory disability. [ABSTRACT FROM AUTHOR]

Objectives: Lumacaftor-ivacaftor is a cystic fibrosis transmembrane conductance regulator (CFTR) modulator known to improve clinical status in people with cystic fibrosis (CF). The aim of this study was to assess lung structural changes after 1 year of lumacaftor-ivacaftor treatment and to use unsupervised machine learning to identify morphological phenotypes of lung disease that are associated with response to lumacaftor-ivacaftor., Methods: Adolescents and adults with CF from a French multicentre real-world prospective observational study evaluating the first year of treatment with lumacaftor-ivacaftor were included if they had pre-therapeutic and follow-up chest computed tomography (CT) scans available. CT scans were visually scored using a modified Bhalla score. A k-means clustering method was performed based on 120 radiomics features extracted from unenhanced pre-therapeutic chest CT scans., Results: In total, 283 patients were included. The Bhalla score significantly decreased after 1 year of lumacaftor-ivacaftor (-1.40±1.53 points compared with pre-therapeutic CT, p<0.001). This finding was related to a significant decrease in mucus plugging (-0.58±0.88 points, p<0.001), bronchial wall thickening (-0.35±0.62 points, p<0.001) and parenchymal consolidations (-0.24±0.52 points, p<0.001). Cluster analysis identified three morphological clusters. Patients from cluster C were more likely to experience an increase in per cent predicted forced expiratory volume in 1 s (FEV 1 % pred) ≥5% under lumacaftor-ivacaftor than those in the other clusters (54% of responders versus 32% and 33%; p=0.02)., Conclusion: 1-year treatment with lumacaftor-ivacaftor was associated with a significant visual improvement of bronchial disease on chest CT. Radiomics features on pre-therapeutic CT scans may help to predict lung function response under lumacaftor-ivacaftor., Competing Interests: Conflict of interest: A. Campredon has nothing to disclose. Conflict of interest: E. Battistella has nothing to disclose. Conflict of interest: C. Martin reports lecture payments or honoraria from Chiesi and Zambon, outside the submitted work. Conflict of interest: I. Durieu has nothing to disclose. Conflict of interest: L. Mely has nothing to disclose. Conflict of interest: C. Marguet reports consulting fees from Gleamer; lecture payments or honoraria from Vertex, Viatis and Zambon; support for attending meetings and/or travel from Zambon; and participation on a Data Safety Monitoring Board or Advisory Board for Zambon and Viatis; outside the submitted work. Conflict of interest: C. Belleguic has nothing to disclose. Conflict of interest: M. Murris-Espin has nothing to disclose. Conflict of interest: R. Chiron has nothing to disclose. Conflict of interest: A. Fanton has nothing to disclose. Conflict of interest: S. Bui reports payment for expert testimony for inhaled antibiotics for Zambon, outside the submitted work. Conflict of interest: M. Reynaud-Gaubert has nothing to disclose. Conflict of interest: P. Reix has nothing to disclose. Conflict of interest: T-N. Hoang-Thi has nothing to disclose. Conflict of interest: M. Vakalopoulou has nothing to disclose. Conflict of interest: M-P. Revel has nothing to disclose. Conflict of interest: J. Da Silva has nothing to disclose. Conflict of interest: P-R. Burgel reports grants or contracts from Vertex and GSK; consulting fees from AstraZeneca, Chiesi, GSK, Insmed, Vertex and Zambon; and lecture payments or honoraria from Pfizer and Novartis; outside the submitted work. Conflict of interest: G. Chassagnon reports lecture payments or honoraria from Chiesi, outside the submitted work., (Copyright ©The authors 2022. For reproduction rights and permissions contact permissions@ersnet.org.)

Mild-to-moderate haemoptysis (m-mH) is common in patients with cystic fibrosis but the risk of subsequent massive haemoptysis (MH) is not known. Allergic bronchopulmonary aspergillosis and diabetes were significant predictors of MH subsequent to m-mH. https://bit.ly/30093Hw., Competing Interests: Conflict of interest: G. Pavaut has nothing to disclose. Conflict of interest: M. Kyheng has nothing to disclose. Conflict of interest: O. Le Rouzic reports personal fees and nonfinancial support from AstraZeneca, Boehringer Ingelheim, Chiesi, Lilly and Novartis, and nonfinancial support from GlaxoSmithKline, MundiPharma, Pfizer, Teva, the Santelys Association, Vertex and Vitalaire, outside the submitted work. Conflict of interest: T. Perez reports nonfinancial support for clinical studies in cystic fibrosis from Physioassist and Antadir outside the submitted work. Conflict of interest: B. Wallaert reports personal fees and nonfinancial support from Roche and Boehringer Ingelheim, and nonfinancial support from Vitalaire, outside the submitted work. Conflict of interest: A. Prevotat has nothing to disclose., (Copyright ©ERS 2020.)

Background: The prevalence of Achromobacter xylosoxidans lung isolation in cystic fibrosis (CF) patients has increased, but the impact on lung function is controversial. The aim of this study was to evaluate the long-term effects of A. xylosoxidans isolation on respiratory function of adult patients with CF in the first 3 years after identification of A. xylosoxidans isolation., Methods: This was a case-control retrospective study performed at a single CF centre in Lille, France. Data for 36 patients with CF who had at least one sputum culture positive for A. xylosoxidans ( Ax+ ) were evaluated and compared with control CF patients uninfected by A. xylosoxidans ( Ax- ). Respiratory function and exacerbation frequency were evaluated between 1 year prior to and 3 years after A. xylosoxidans isolation., Results: Compared with the Ax - group, the Ax+ group had a lower forced expiratory volume in 1 s (FEV 1 ) at baseline (median (interquartile range): 55.2% (50.6-59.8%) versus 73.8% (67.2-80.4%); p=0.005), a greater decline in FEV 1 (±se) in the first year after A. xylosoxidans identification (-153.6±16.1 mL·year -1 versus -63.8±18.5 mL·year -1 ; p=0.0003), and more exacerbations in the first 3 years after A. xylosoxidans identification (9 (7-12) versus 7 (5-10); p=0.03). Ax+ patients co-colonised with Pseudomonas aeruginosa (n=27, 75%) had a greater FEV 1 decline (p=0.003) and more exacerbations in the year after A. xylosoxidans identification (p=0.037) compared with patients colonised with A. xylosoxidans alone. Patients with chronic A. xylosoxidans isolation (n=23, 64%) had more exacerbations than intermittently colonised patients in the 3 years after A. xylosoxidans identification (p=0.012)., Conclusion: A. xylosoxidans isolation is associated with a decline in respiratory function in patients with CF. Chronic A. xylosoxidans isolation and P. aeruginosa co-isolation may be markers of more severe respiratory disease in Ax + patients., Competing Interests: Conflict of interest: M. Tetart has nothing to disclose. Conflict of interest: F. Wallet has nothing to disclose. Conflict of interest: M. Kyheng has nothing to disclose. Conflict of interest: S. Leroy has nothing to disclose. Conflict of interest: T. Perez reports being the principal investigator of a clinical study evaluating a device for chest clearance in cystic fibrosis (Simeox, PhysioAssist). Conflict of interest: O. Le Rouzic reports personal fees and nonfinancial support from AstraZeneca, Boehringer Ingelheim, Chiesi, Lilly and Novartis, and nonfinancial support from GlaxoSmithKline, MundiPharma, Pfizer, Teva, the Santelys Association, Vertex and Vitalaire, outside the submitted work. Conflict of interest: B. Wallaert reports personal fees from Roche and Boehringer Ingelheim, outside the submitted work. Conflict of interest: A. Prevotat reports personal fees from Vertex and GSK, and congress invitations from Teva and Novartis, outside the submitted work., (Copyright ©ERS 2019.)

Screening is important to determine whether patients with systemic sclerosis (SSc) have pulmonary hypertension because earlier pulmonary hypertension treatment can improve survival in these patients. Although decreased transfer factor of the lung for carbon monoxide ( T LCO ) is currently considered the best pulmonary function test for screening for pulmonary hypertension in SSc, small series have suggested that partitioning T LCO into membrane conductance (diffusing capacity) for carbon monoxide ( D MCO ) and alveolar capillary blood volume ( V C ) through combined measurement of T LCO and transfer factor of the lung for nitric oxide ( T LNO ) is more effective to identify pulmonary hypertension in SSc patients compared with T LCO alone. Here, the objective was to determine whether combined T LCO - T LNO partitioned with recently refined equations could more accurately detect pulmonary hypertension than T LCO alone in SSc.For that purpose, 572 unselected consecutive SSc patients were retrospectively recruited in seven French centres.Pulmonary hypertension was diagnosed with right heart catheterisation in 58 patients. T LCO , T LNO and V C were all lower in SSc patients with pulmonary hypertension than in SSc patients without pulmonary hypertension. The area under the receiver operating characteristic curve for the presence of pulmonary hypertension was equivalent for T LCO (0.82, 95% CI 0.79-0.85) and T LNO (0.80, 95% CI 0.76-0.83), but lower for V C (0.75, 95% CI 0.71-0.78) and D MCO (0.66, 95% CI 0.62-0.70).Compared with T LCO alone, combined T LCO - T LNO does not add capability to detect pulmonary hypertension in unselected SSc patients., Competing Interests: Conflict of interest: None declared., (Copyright ©ERS 2017.)

Dyspnoea is a prominent symptom of chronic obstructive pulmonary disease (COPD). Recent multidimensional dyspnoea questionnaires like the Multidimensional Dyspnea Profile (MDP) individualise the sensory and affective dimensions of dyspnoea. We tested the MDP in COPD outpatients based on the hypothesis that the importance of the affective dimension of dyspnoea would vary according to clinical characteristics.A multicentre, prospective, observational, real-life study was conducted in 276 patients. MDP data were compared across various categories of patients (modified Medical Research Council (mMRC) dyspnoea score, COPD Assessment Test (CAT) score, Global Initiative for Chronic Obstructive Lung Disease (GOLD) airflow obstruction categories, GOLD "ABCD" categories, and Hospital Anxiety and Depression Scale (HADS)). Univariate and multivariate regressions were conducted to explore factors influencing the affective dimension of dyspnoea. Cluster analysis was conducted to create homogeneous patient profiles.The MDP identified a more marked affective dimension of dyspnoea with more severe mMRC, CAT, 12-item Short-Form Health Survey mental component, airflow obstruction and HADS. Multivariate analysis identified airflow obstruction, depressive symptoms and physical activity as determinants of the affective dimension of dyspnoea. Patients clustered into an "elderly, ex-smoker, severe disease, no rehabilitation" group exhibited the most marked affective dimension of dyspnoea.An affective/emotional dimension of dyspnoea can be identified in routine clinical practice. It can contribute to the phenotypic description of patients. Studies are needed to determine whether targeted therapeutic interventions can be designed and whether they are useful., (Copyright ©ERS 2016.)