Background:Cardiovascular and metabolic comorbidities in chronic obstructive pulmonary disease (COPD) are associated with higher symptoms burden. Few center-based studies have evaluated the impact of these comorbidities on short-term pulmonary rehabilitation outcomes with contrasting results. Research Question:This study aimed to determine whether cardiovascular diseases and metabolic comorbidities impacted long-term outcomes of a home-based PR program in COPD patients. Study Design and methods:Data of 419 consecutive COPD patients addressed to our pulmonary rehabilitation program between January 2010 and June 2016 were retrospectively analyzed. Our program consisted of once-weekly supervised home sessions, including therapeutic education and self-management support, with unsupervised retraining exercises and physical activities the other days for 8 weeks. Exercise capacity (6-min stepper test [6MST]), quality of life (visual simplified respiratory questionnaire), and anxiety and depression (hospital anxiety and depression scale) were assessed respectively, before (M0) and at the end (M2) of the pulmonary rehabilitation program, and at 6 (M8) and 12 months (M14) after its achievement. Results:Patients (mean age 64.1±11.2 years, 67% males, mean forced expiratory volume in one second (FEV1) 39.2±17.0% predicted) were classified as having cardiovascular comorbidities (n=195), only metabolic disorders (n=122) or none of these comorbidities (n=102). After adjustment, all outcomes appeared similar between groups at baseline and improved after pulmonary rehabilitation with a greater effect at M14 for patients with only metabolic disorders on anxiety and depression score (-5.0±0.7 vs -2.9±0.8 and -2.6±0.6, p=0.021). Quality of life and exercise capacity improvements were not significantly different between the three groups at M2 and M14. Conclusion:Cardiovascular and metabolic comorbidities do not preclude COPD patients from obtaining clinically meaningful improvements in exercise capacity, quality of life and anxiety-depression up to 1 year after a home-based pulmonary rehabilitation.

BACKGROUND:Low income, a known prognostic indicator of various chronic respiratory diseases, has not been properly studied in idiopathic pulmonary fibrosis (IPF). We hypothesize that a low income has an adverse prognostic impact on IPF. METHODS:Patients were selected from the French national prospective cohort COFI. Patients' income was assessed through the median city-level income provided by the French National Institute of Statistics and Economic Studies according to their residential address. Patients were classified in two groups as "low income" vs. "higher income" depending on whether their annual income was estimated to be < or ≥18 170 €/year (the first quartile of the income distribution in the study population). The survival and progression-free survival (PFS) of the groups were compared by a log-rank test and a Cox model in multivariate analysis. RESULTS:200 patients were included. The average follow-up was 33.8 ± 22.7 months. Patients in the low income group were significantly more likely to be of non-European origin (p < 0.006), and to have at least one occupational exposure (p < 0.0001), and they tended to have a higher cumulative exposure to fine particles PM2.5 (p = 0.057). After adjusting for age, gender, forced vital capacity at inclusion, geographical origin, and occupational exposure having a low-income level was a factor associated with a worse PFS (HR: 1.81; CI95%: 1.24-2.62, p = 0.001) and overall survival (HR: 1.49; CI95%: 1.0006-2.23, p = 0.049). CONCLUSIONS:Low income appears to be a prognostic factor in IPF. IPF patients with low incomes may also be exposed more frequently to occupational exposures.

Pulmonary rehabilitation, whether performed at home or in a specialised centre, is effective in the management of COPD. As gender-related differences in COPD were previously reported, we aimed to evaluate the impact of gender on pulmonary rehabilitation outcomes in the short, medium and long term. In this retrospective observational study of 170 women and 310 men with COPD, we compared the outcomes of an 8-week home-based pulmonary rehabilitation programme including an individualised plan of retraining exercises, physical activities, therapeutic education, and psychosocial and self-management support. Exercise tolerance, anxiety, depression, and quality of life (QOL) were assessed using the 6-min stepper test (6MST), Hospital Anxiety and Depression Scale (HADS) and Visual Simplified Respiratory Questionnaire (VSRQ), respectively. Assessments were carried out before, immediately after the pulmonary rehabilitation programme (T2) and at 8 (T8) and 14 months (T14). At baseline, women were younger (mean 62.1 versus 65.3 years), more often nonsmokers (24.7% versus 7.7%) and had a higher body mass index (28 versus 26.4 kg·m−2). They more often lived alone (50.6% versus 24.5%) and experienced social deprivation (66.7% versus 56.4%). They had significantly lower exercise tolerance (−34 strokes, 6MST) and higher anxiety and depression (+3.2 HADS total score), but there were no between gender differences in QOL (VSRQ). Both groups showed similar improvements in all outcome measures at T2, T8 and T14 with a tendency for men to lose QOL profits over time. Despite some differences in baseline characteristics, women and men with COPD had similar short-, medium- and long-term benefits of a home-based pulmonary rehabilitation programme.

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.

Background:Methotrexate (MTX) is a key anchor drug for rheumatoid arthritis (RA) management. Its use has been associated with hypersensitivity pneumonitis and diffuse lung disease. Whether MTX exposure increases the risk of interstitial lung disease (ILD) in patients with RA is disputed.Objectives:We aimed to evaluate the association of antecedent MTX use with development of RA-ILD.Methods:Through a case-control study design with derivation and international validation samples, we examined the association of MTX exposure with ILD in 482 patients with RA-ILD and 741 patients with RA without ILD. Estimates were pooled over the different samples using meta-analysis techniques.Results:Analysis of the derivation sample revealed an inverse relationship between MTX exposure and RA-ILD (adjusted odds ratio [OR], 0.48; 95% confidence interval [CI], 0.25 to 0.92; P=0.028), which was confirmed in the validation samples (pooled adjusted OR, 0.39; 95% CI, 0.23 to 0.68; P<0.001). The combined estimate using both the derivation and validation samples revealed an adjusted OR of 0.43 (95% CI, 0.28 to 0.65; P<0.0001). MTX ever users were less frequent among patients with RA-ILD compared to those without ILD, irrespective of chest high resolution computed tomography pattern. In patients with RA-ILD, ILD onset was significantly delayed in MTX ever users compared to never users (11.5 ± 10.6 years and 3.7 ± 7.1 years, respectively; P<0.001).Conclusion:Our results suggest that MTX is not a risk factor for RA-ILD and support a possible disease modifying effect of MTX on development of RA-ILD.Disclosure of Interests:Pierre-Antoine Juge: None declared, Joyce S. Lee Consultant of: Celgene, Genentech, Boehringer Ingelheim, Jessica Lau: None declared, Leticia Kawano: None declared, Jorge Rojas-Serrano: None declared, Marco Sebastiani: None declared, Gouri Koduri: None declared, Eric Matteson Grant/research support from: Pfizer, Consultant of: Boehringer Ingelheim, Gilead, TympoBio, Arena Pharmaceuticals, Speakers bureau: Simply Speaking, Karina Bonfiglioli Consultant of: Roche, Abbvie, Pfizer, Janssen and BMS, Marcio Sawamura: None declared, Ronaldo Kairalla: None declared, Lorenzo Cavagna: None declared, Emanuele Bozzalla Cassione: None declared, Andreina Manfredi: None declared, Mayra Mejia: None declared, Pedro Rodríguez Henríquez: None declared, Montserrat I. Gonzalez-Perez: None declared, Ramcés Falfan-Valencia: None declared, Ivette Buendia-Roldan: None declared, Glora Perez-Rubio: None declared, Esther Ebstein Consultant of: BMS, Employee of: BMS, Steven Gazal: None declared, Raphael Borie Consultant of: Roche, Boehringer Ingelheim, Sebastien Ottaviani: None declared, Caroline Kannengiesser: None declared, Benoît Wallaert Consultant of: Roche, Boehringer Ingelheim, Yurdagul Uzunhan Consultant of: Roche, Boehringer Ingelheim,, Hilario Nunes: None declared, Dominique Valeyre Consultant of: Astra Zeneca, Roche, Boehringer Ingelheim, Nathalie Saidenberg Kermanac’h: None declared, marie-Christophe Boissier: None declared, Lidwine Wemeau Stervinou Consultant of: Roche, Janssen, BMS, Boehringer Ingelheim, Rene-Marc Flipo Speakers bureau: Novartis, Janssen, Lilly, Sylvain Marchand-Adam Consultant of: Roche, Novartis, Boehringer Ingelheim, Pascal Richette: None declared, Yannick Allanore Shareholder of: Sanofi, Roche, Consultant of: Actelion, Bayer, BMS, Boehringer Ingelheim, Inventiva, Sanofi, Claire Dromer: None declared, Marie-Elise Truchetet: None declared, Christophe Richez Consultant of: Abbvie, Amgen, Mylan, Pfizer, Sandoz and UCB., Thierry Schaeverbeke: None declared, Huguette Lioté: None declared, Gabriel Thabut Employee of: Astra Zeneca, Kevin Deane Grant/research support from: Janssen, Consultant of: Inova, ThermoFisher, Janseen, BMS and Microdrop, Joshua Solomon: None declared, Tracy Doyle: None declared, Jay H. Ryu: None declared, Ivan O. Rosas Consultant of: Boehringer Ingelheim, Gerentech, Three Lakes Partners, V. Michael Holers Grant/research support from: Janssen, Celgene, and BMS, Catherine Boileau: None declared, Marie-Pierre Debray: None declared, Raphaël Porcher: None declared, David A. Schwartz Consultant of: NuMedii, Robert Vassallo Shareholder of: Pfizer, BMS, SunPharma, Bruno Crestani Shareholder of: Apellis, Boehringer Inghelheim, Medillune, Roche, Consultant of: Boehringer Ingelhiem, Astra Zeneca, Roche, Sanofi, Philippe Dieudé: None declared

Background:Long-term effectiveness of pulmonary rehabilitation (PR) is still uncertain in older people with severe chronic obstructive pulmonary disease (COPD). The objective was to compare the effects of home-based PR in people with COPD above and below the age of 70 years. Methods:In this retrospective study, 480 people with COPD were recruited and divided into those ≤70 (n=341) and those >70 years of age (n=139). All participants underwent an 8 weeks of home-based PR, consisting of a weekly supervised 90-minute home session. Six-minute stepper test (6MST), timed-up and go test (TUG), Hospital Anxiety and Depression Scale, and Visual Simplified Respiratory Questionnaire (VSRQ) were assessed at baseline (M0), at 2 (M2), 8 (M8), 14 (M14) months after baseline. Results:The older group was described by fewer current smokers (p <0.001), more long-term oxygen therapy use (p = 0.024), higher prevalence of comorbidities (p<0.001), lower 6MST score and higher TUG score (p<0.001), compared to the younger group. Both groups improved every outcome at M2 compared to baseline. At M2, 88% of people ≤70 years of age and 79% of those above 70 were considered as responders in at least one evaluated parameter (p = 0.013). Both groups maintained the benefits at M14, except for the VSRQ score and the number of responders to this outcome in the older group. Conclusion:Regardless of the age, personalized home-based PR was effective for people with COPD in the short term. Above 70 years, an ageing effect appeared on the long-term effectiveness of quality of life benefit.

Background: Idiopathic pulmonary fibrosis (IPF) is characterized by a male predominance. The aim of the study was to explore gender differences in a well-designed French multicentre prospective IPF cohort (COhorte FIbrose, COFI) with a 5-year follow-up. Methods: Between 2007 and 2010, 236 patients with incident IPF were included in COFI. Gender characteristics were compared using a t -test, Chi-squared test and ANOVA, as appropriate. Survival analyses were performed. Results: Fifty-one (22%) females and 185 (78%) males with an average age at diagnosis of 70.1 ± 9.20 and 67.4 ± 10.9 years, respectively, were included in the cohort. Women were significantly less exposed to tobacco smoke [never n = 32 (62.7%) vs. n = 39 (21.1%), p < 0.001] and to occupational exposure [ n = 7 (13.7%) vs. n = 63 (34.1%), p = 0.012]. Baseline forced vital capacity, % of predicted (FVC%) was significantly better in women compare to men (83.0% ± 25.0 v. 75.4% ± 18.7 p = 0.046). At presentation honeycombing and emphysema on CT scan were less common in women [ n = 40 (78.4%) vs. n = 167 (90.3%) p = 0.041] and [ n = 6 (11.8%) vs. n = 48 (25.9%) p = 0.029], respectively. During follow-up fewer women were transplanted compared to men [ n = 1 (1.96%) vs. n = 20 (10.8%) p = 0.039]. Medians of survival were comparable by gender [31 months (CI 95%: 28–40) vs. 40 months (CI 95%: 33–72) p = 0.2]. After adjusting for age and FVC at inclusion, being a woman was not associated to a better survival. Conclusions: Women appear to have less advanced disease at diagnosis, maybe due to less exposure history compare to men. Disease progression and overall survival remains comparable regardless gender, but women have less access to lung transplantation.

International guidelines recommend that annual influenza vaccination be performed in patients with idiopathic pulmonary fibrosis (IPF). Viral infections are recognized to be triggers of acute exacerbation (AE). The purpose of this study was to investigate the role of annual influenza vaccination on AE occurrence. 236 patients with incident IPF were included in the multicentric longitudinal prospective cohort COhorte FIbrose (COFI), and followed for 5 years. Influenza vaccination status was requested annually. We have defined aone-year vaccination coverage from September the first to August the 30th of the following year. 638 annual observations were available with an informed vaccination status. To evaluate the association between annual influenza vaccination and AE occurrence, two mixed-effect Cox regression was used. For the second model we divided patients in 3 groups: – patients vaccinated every year; – patients vaccinated at least half of the years of follow-up; – patients vaccinated less than half the years of follow-up. Only the first AE was considered. The definition of AE was that of Akira et al. Mean follow-up was 33.2 ± 23.6 months. 36 AEs occurred in 33 patients. Of the 33 patients, only 27 had an informed vaccination status in the year of their AE. Nine (33%) patients who experienced an AE weren’t vaccinated. For 141 (22%) annual observations, the patient didn’t receive the vaccine. In this model, influenza vaccination was associated with a decreased occurrence of AE but this did not reach the level of significance in this small population (HR:0.58 CI95%0.23-1.46, P = 0.25). In the first group patients were vaccinated every year and consisted of hundred and thirty-three individuals. The second group was composed of patients vaccinated at least half of the years of follow-up, with 35 patients. And 36 patients were vaccinated less than half the years of follow-up in the last group. In the second model, patients vaccinated less than half the years of follow-up were significantly more at risk to present AE compared to patients who were vaccinated every year (HR:2.77 CI95%1.11-6.93, P = 0.03). In our IPF cohort, only about half of the patients had influenza vaccination coverage throughout the follow-up period. Annual influenza vaccination seems to reduce the risk of AE but due to a lack of power this association was not significant. However patients vaccinated less than half the years of follow-up were significantly more at risk to present AE compared to patients who were vaccinated every year. A study with a larger number of patients is highly desirable to confirm this association.

Objective. Rituximab was proven superior to azathioprine for maintenance treatment of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). The high cost of rituximab might, however, limit its routine use. This study determined the cost-effectiveness of intravenous rituximab (5 x 500 mg until month 18), versus oral azathioprine (2 mg/kg per day, gradually decreased between month 12 and 22), for maintenance treatment of patients with granulomatosis with polyangiitis, microscopic polyangiitis, or renal-limited vasculitis, aged 18-75. Methods. We performed a single-trial based economic evaluation. MAIN-RITSAN was a 28-month multicentre, prospective, randomised, controlled open-label trial. We estimated the cost of healthcare resources and quality of life using prospectively collected data. Healthcare costs were estimated from the perspective of the French Social Health Insurance's perspective, using 2016 tariffs for reimbursement. Utilities were derived from Short Form 36 scores. We estimated total average cost, incremental cost per incremental relapse averted and per quality-adjusted life-year (QALY) gained. Sensitivity analyses were performed to assess uncertainty over relapses, severe adverse events, discount rate, utility weights, time horizon and the cost of rituximab. Costs drivers were tested using a generalised linear model. Results. Total average costs were (sic)13,387 ((sic)11,605-(sic)15,646) and (sic)10,217 ((sic)7,567-12,949) in the rituximab and azathioprine groups respectively. The incremental cost-effectiveness ratio (ICER) was (sic)12,824 per relapse averted and the incremental cost-utility ratio (ICUR) (sic) 37,782 per QALY gained. Besides the unit cost of rituximab, the major cost drivers were relapses and severe adverse events. Conclusion. Maintenance treatment by rituximab could be cost-effective for preventing relapses in patients with AAV.

The RESPIR survey aimed at analysing the current situation regarding feelings and expectations for the treatment of idiopathic pulmonary fibrosis with feedback from pulmonologists, patients and caregivers. A questionnaire designed by a scientific committee was sent to the 3,279 French pulmonologists. Two “mirror” questionnaires were given to patients and caregivers by their pulmonologists and analysed anonymously. From Nov 2017 to Oct 2018, 147 pulmonologists and 161 patients (75% on antifibrotic drugs) replied to the survey. The proportion of patients determined to fight the disease (86%) or keen to be involved in treatment decisions (81%) was high. The view of physicians was significantly different; they estimated these proportions to be 68% and 57% respectively. At antifibrotic drugs initiation, only 16% of patients preferred quality of life to lifespan, contrasting with pulmonologists who ranked it around 34%. This may likely explain why 31% of specialists declared that they generally did not start antifibrotic drugs in asymptomatic patients. For 76% of patients on antifibrotic drugs, starting treatment was associated with a positive feeling, gave hope, and provided relief. So, 66% expressed regret not having been prescribed this therapy earlier. Overall, patients had a more positive perception towards the treatment than the physicians had thought: they believed they had more benefits than disadvantages (73% vs 62% according to the physicians). The patients surveyed appear more enclined to an earlier initiation and a stronger involvement in medical care than pulmonologists thought. Shared decision-making could be strengthened.

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.

Introduction: Home-based pulmonary rehabilitation (PR) has demonstrated its effectiveness amongst patients with chronic obstructive pulmonary disease (COPD) but has never been investigated in severe asthma. Methods: In a retrospective study, we included 28 patients with severe asthma (61.5 +/- 16.2 years, FEV1: 51.4 +/- 17.3%) and 164 matched COPD patients (64.3 +/- 11.6 years, FEV1: 47.7 +/- 15.5%) who had completed a home-based PR program and pursued at least 12 months of follow-up. The number of steps performed during a 6-min stepper test (6MST), the Hospital Anxiety and Depression (HAD) scores, and the Visual Simplified Respiratory Questionnaire score (VSRQ) were compared between baseline, the post-PR period (post-PR) and after 12 months of follow-up (M12) within each group. The evolution of the 6MST, HAD and VSRQ values between baseline, post-PR and M12 was compared between severe asthma and COPD patients. Results: In the severe asthma group, the 6MST was higher post-PR (504 +/- 150, p = 0.043) and at M12 (538 +/- 163, p = 0.016) compared with baseline (450 +/- 148). The VSRQ score was higher at M12 (39.0 +/- 18.6, p = 0.049) but not post-PR (38.7 +/- 15.8, p = 0.119) in comparison with baseline (32.2 +/- 12.4). There was no difference in the HAD scores between baseline, post-PR and M12. PR outcome was not significantly different between severe asthma and COPD patients at short and long term (p > 0.05). Conclusion: In severe asthma, home-based PR is associated with improved exercise tolerance and quality of life on a long-term basis but does not modify anxiety and depression.

Non-coding RNAs have recently gained widespread attention given their broad implication in the initiation and progression of various complex diseases. Whereas previous studies on fibrotic lung disorders, especially the idiopathic form (IPF), have mainly focused on miRNAs, the precise contribution of other class of non-coding RNAs in lung fibrogenesis is unclear. Given the paucity of effective treatment in IPF, new insights into the deleterious mechanisms controlling lung fibroblast activation, the key cell type driving the fibrogenic process, are essential to develop new therapeutic strategies for this devastating disease. Here we identify a long non-coding RNA, termed DNM3OS, which tightly controls the various cellular and molecular phenotypic changes occurring during lung fibroblast activation in response to TGF-β. Notably, DNM3OS regulates these processes in trans by giving rise to 3 distinct profibrotic miRNAs (i.e. miR-199a-5p/3p and miR-214-3p), which repress distinct but functionally related targets within TGF-β signaling cascade. Finally, we provide in vivo evidence that interfering with DNM3OS function may be a promising strategy for the treatment of lethal fibrotic diseases such as IPF.

Idiopathic pulmonary fibrosis (IPF) is a fatal lung disease, with a median survival of 2–3 years and variable natural history, characterized by gradual and progressive deterioration. Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is a severe complication, associated with poor survival and a mortality > 50%. To date, no treatment has proven effective in AE-IPF, with cyclophosphamide (CYC) the only therapy suggested to be effective on survival, primarily based on retrospective series. Considering the high fatality rates of AE-IPF, evaluating the efficacy of immunosuppressive agents in a randomized controlled trial proves crucial, as the results could significantly impact treatment and prognosis of AE-IPF. The EXAFIP study is a French national multicenter double-blind placebo-controlled randomized trial. Its primary objective is to evaluate the efficacy of CYC compared to placebo on early survival in patients treated with corticosteroids. We hypothesize that adding CYC to high-dose corticosteroids would reduce 3-month mortality in AE-IPF patients. The primary outcome is all-cause mortality rate at Month 3; secondary objectives are to evaluate the efficacy of CYC compared to placebo on overall survival at Months 6 and 12, respiratory disease-specific mortality, respiratory morbidity, and chest high-resolution computed tomography features, and to determine prognostic factors in AE-IPF and compare the safety of the two treatment arms during 6 months’ follow-up. There is an urgent unmet clinical need for effective AE-IPF treatment. The EXAFIP study is the first large Phase III placebo-controlled randomized trial evaluating the efficacy and safety of CYC added to corticosteroids in treating AE-IPF. The results of this study could significantly impact treatment strategy and prognosis of AE-IPF. Clinical trials, NCT02460588 ; Date: June 2, 2015, prospectively; Issue date: 14/11/2017; Protocole Amendment Number: 03.

Background Few studies have examined the benefits of pulmonary rehabilitation in patients with fibrotic idiopathic pulmonary pneumonia (f-IIP). Here, we report the results of an observational study in routine clinical practice of home-based pulmonary rehabilitation for f-IIP patients. Methods A total of 112 consecutive patients (61 with idiopathic pulmonary fibrosis and 51 with fibrotic nonspecific interstitial pneumonitis) were enrolled, of whom 65 had mild-to-moderate disease (forced vital capacity (FVC) ≥50% predicted and diffusing capacity of the lung for carbon monoxide (DLCO) ≥30% predicted) and 47 had severe disease (FVC <50% predicted and/or DLCO <30% predicted). The 2-month pulmonary rehabilitation programme consisted of a once-weekly visit with retraining, therapeutic education and psychosocial support. Patients were provided with an individualised action plan and were followed-up bimonthly for 12 months. Exercise tolerance (6-min stepper test (6MST)), mood (Hospital Anxiety and Depression Scale (HADS)) and quality of life (QoL) (Visual Simplified Respiratory Questionnaire (VSRQ)) were assessed before (T0), immediately after (T2), 6 months after (T8) and 12 months after (T14) the end of the pulmonary rehabilitation programme. Results 6MST strokes, HADS Anxiety score and VSRQ score were each significantly improved at T2 (n=101), T8 (n=76) and T14 (n=62) compared with T0 values. The improvements in outcomes were not influenced by disease severity or subtype. Patients who completed the study had significantly better baseline FVC and DLCO values than those who did not. Conclusions Home-based pulmonary rehabilitation provides long-term benefits in exercise tolerance, anxiety and QoL for patients with f-IIP. Pulmonary rehabilitation should be prescribed systematically as part of the therapeutic arsenal for these patients.

Background and objectives: Little is known about the consequences of chronic sarcoidosis on daily life physical activity (DLPA). The aim of this prospective study was to measure DLPA in patients with chronic sarcoidosis and to determine its relationship to clinical and functional parameters. Methods: Fifty-three patients with chronic sarcoidosis and 28 healthy control subjects were enrolled in this multicenter prospective study. Two markers of DLPA (number of steps walked per day [SPD]) and total daily energy expenditure (TEE) were assessed for five consecutive days with a physical activity monitor. Pulmonary function, aerobic capacity (maximal oxygen uptake [VO(2)max]), exercise capacity (6-min walk test [6MWT]), and quality of life (self-reported questionnaires) were also evaluated. Comparisons of DLPA parameters between the two groups were performed using an analysis of covariance adjusted for age, sex, and body mass index (BMI). Relationships between DLPA parameters and patient characteristics were assessed in multivariable linear regression models. Results: Patients with sarcoidosis walked significantly fewer SPD than did the control subjects (6395 +/- 4119 and 11 817 +/- 3600, respectively; P < 0.001 after adjustment for age, BMI, and sex). TEE was not significantly different between patients with sarcoidosis and healthy controls (median [interquartile range]: 2369 [2004-2827] and 2387 [2319-2876] kcal/day, respectively, P = 0.054 adjusted for age, BMI, and sex). SPD showed significant positive correlations with 6MWT distance (Pearson's correlation, r = 0.32, 95% confidence intervals [95%CI] = 0.06, 0.55; P = 0.019), VO(2)max (r = 0.44, 95%CI = 0.17, 0.65; P = 0.002), and Visual Simplified Respiratory Questionnaire score (r = 0.44, 95%CI = 0.19, 0.64; P = 0.001), and a significant negative correlation with modified Medical Research Council questionnaire score (r = -0.38, 95%CI = -0.60, -0.10; P = 0.009). TEE was significantly correlated with BMI (r = 0.38, 95%Cl = 0.13, 0.59; P = 0.004), forced expiratory volume in 1 second 0.55, 95%CI = 0.33, 0.71; P < 0.001), total lung capacity (r = 0.44, 95%CI = 0.18, 0.64; P = 0.001), and forced vital capacity (r = 0.56, 95%CI = 0.34, 0.72; P < 0.001). In multivariable analysis, SPD remained associated only with VO(2)max. Conclusion: Patients with chronic sarcoidosis appear to have reduced DLPA mainly because of compromised VO(2)max.

We previously reported a new form of light chain deposition disease (LCDD) presenting as diffuse cystic lung disorder that differs from the usual systemic form with respect to patient age, the male/female ratio, the involved organs, and the hematologic characteristics. We also demonstrated that the light chains were produced by an intrapulmonary B-cell clone and that this clone shared a stereotyped antigen receptor IGHV4-34/IGKV1. However, we only analyzed 3 patients. We conducted a retrospective study including lung tissue samples from 24 patients with pulmonary LCDD (pLCDD) matched with samples from 13 patients with pulmonary κ light chain amyloidosis (pAL amyloidosis) used as controls. Mass spectrometry–based proteomics identified immunoglobulin κ peptides as the main protein component of the tissue deposits in all patients. Interestingly, in pLCDD, IGKV1 was the most common κ family detected (86.4%), and IGKV1-8 was overrepresented compared with pAL amyloidosis (75% vs 11.1%, P = .0033). Furthermore, IGKV1-8 was predominantly associated with a diffuse cystic pattern (94%) in pLCDD. In conclusion, the high frequency of IGKV1-8 usage in cystic pLCDD constitutes an additional feature arguing for a specific entity distinct from the systemic form that preferentially uses IGKV4-1.

Jean-Marie Grosbois Justine Heluain-Robiquet François Machuron Gaelle Terce Cécile Chenivesse Benoit Wallaert 3,5,6 Olivier Le Rouzic 3,5 1FormAction Santé, Pérenchies F-59840, France; 2CH Béthune, Service de Pneumologie et Réhabilitation Respiratoire, Béthune F-62400, France; 3CHU Lille, Service de Pneumologie et Immuno-Allergologie, Centre de Référence Constitutif des Maladies Pulmonaires Rares, Department of Heart and Lung Diseases, Lille F-59000, France; 4CHU Lille, Department of Biostatistics, University Lille, EA 2694 Santé Publique: Epidémiologie Et Qualité Des Soins, Lille F-59000, France; 5University Lille, Lille F-59000, France; 6Inserm, CNRS, Institut Pasteur de Lille, U1019 UMR 8204 CIIL Center for Infection and Immunity of Lille, Lille F-59000, France Background: Pulmonary rehabilitation (PR) improves exercise tolerance and quality of life in patients with chronic obstructive pulmonary disease (COPD), regardless of disease severity. Socioeconomic deprivation has been linked to the incidence of COPD; however, little is known about its impact on PR outcomes. Methods: In this retrospective observational study, 459 COPD patients were enrolled and dichotomized into socially deprived (n=276) and non-socially deprived (n=183) groups based on a cut-off of 30.17 in the EPICES questionnaire (Evaluation of Deprivation and Inequalities in Health Centers), which evaluates socioeconomic disadvantage. The PR program consisted of once-weekly home sessions for 8 weeks, and consisted of an individualized plan of retraining exercises, physical activities, therapeutic education, and psychosocial and motivational support. Exercise tolerance, anxiety and depression, and quality of life were assessed using the 6 min stepper test (6MST), Hospital Anxiety and Depression Scale (HADS), and Visual Simplified Respiratory Questionnaire (VSRQ). Assessments were made before the PR program (baseline) and then at 2 (T2), 8 (T8), and 14 (T14) months after baseline. Results: Compared with the non-socially deprived group, socially deprived patients were younger, more frequently women, active smokers, and living alone, and belonged to lower socioprofessional categories. At baseline, 6MST, VSRQ, and HADS measures were lower for the socially deprived than the non-socially deprived group. At T2, T8, and T14, there were no significant between-group differences in any outcome, and the percentage of patients showing clinically important improvements was the same in both groups. Conclusion: Home-based PR is effective for COPD patients in the short and long term, regardless of socioeconomic status.

Background Pulmonary rehabilitation (PR) improves exercise tolerance and quality of life in patients with chronic obstructive pulmonary disease (COPD), regardless of disease severity. Socioeconomic deprivation has been linked to the incidence of COPD; however, little is known about its impact on PR outcomes. Methods In this retrospective observational study, 459 COPD patients were enrolled and dichotomized into socially deprived (n=276) and non-socially deprived (n=183) groups based on a cut-off of 30.17 in the EPICES questionnaire (Evaluation of Deprivation and Inequalities in Health Centers), which evaluates socioeconomic disadvantage. The PR program consisted of once-weekly home sessions for 8 weeks, and consisted of an individualized plan of retraining exercises, physical activities, therapeutic education, and psychosocial and motivational support. Exercise tolerance, anxiety and depression, and quality of life were assessed using the 6 min stepper test (6MST), Hospital Anxiety and Depression Scale (HADS), and Visual Simplified Respiratory Questionnaire (VSRQ). Assessments were made before the PR program (baseline) and then at 2 (T2), 8 (T8), and 14 (T14) months after baseline. Results Compared with the non-socially deprived group, socially deprived patients were younger, more frequently women, active smokers, and living alone, and belonged to lower socioprofessional categories. At baseline, 6MST, VSRQ, and HADS measures were lower for the socially deprived than the non-socially deprived group. At T2, T8, and T14, there were no significant between-group differences in any outcome, and the percentage of patients showing clinically important improvements was the same in both groups. Conclusion Home-based PR is effective for COPD patients in the short and long term, regardless of socioeconomic status.