Six-minute stepper test to assess effort intolerance in interstitial lung diseases.

The six-minute stepper test (6MST) is a new test for evaluating exercise tolerance. Unlike the six-minute walk test (6MWT) it can be carried out in a limited space. The aim of this study was to compare the 6MST and the 6MWT in patients with various diffuse interstitial lung disease (ILD). 6MWT and 6MST were performed the same day in 84 patients with various ILD. The covered distance during 6MWT was compared to the number of steps during the 6MST. We also compared heart rate, oxygen saturation, dyspnoea and leg tiredness on a Borg scale. All the patients successfully completed the tests, and tolerance was considered good. The number of steps completed in the 6MST was strongly correlated with the distance walked in the 6MWT (r2 = 0.5; p < 0.0001). Oxygen desaturation was less frequent and less severe (p < 0.0001), heart rate was higher (p < 0.0001) and dyspnoea and leg tiredness were more marked (p < 0.0001) in the 6MST than in the 6MWT. The 6MST is feasible for patients with ILD. It is a simple, safe, mobile test that is cheap and easy to carry out in all structures.

Hypersensitivity pneumonitis and metalworking fluids contaminated by mycobacteria.

Metalworking fluids (MWF) are responsible for hypersensitivity pneumonitis (HP). The aim of the present study was to identify the antigen (Ag) responsible for MWF-associated HP, and to optimise serological diagnosis by definition of a threshold allowing discrimination between HP patients and asymptomatic exposed workers. 13 patients, who were workers at a car engine manufacturing plant, were suspected of MWF-associated HP. Microbial analysis of 83 used MWFs was carried out. Sera from 13 MWF-associated HP patients, 12 asymptomatic exposed workers and 18 healthy unexposed controls were tested to determine their immunological responses to three Ags, including Mycobacterium immunogenum. M. immunogenum was identified in 40% of used fluids by culture and confirmed by DNA sequencing. The threshold for differentiating MWF-associated HP patients from asymptomatic exposed workers was five arcs of precipitation (sensitivity 77% and specificity 92%), as determined by electrosyneresis (ES). Using ELISA methods with protein extract from M. immunogenum, a threshold leading to 92% sensitivity and 100% specificity was established. The detection of specific antibodies against M. immunogenum Ag at high levels in case sera suggests that M. immunogenum-contaminated MWF is responsible for MWF-associated HP. To discriminate MWF-associated HP patients from asymptomatic exposed workers, we suggest a five-arc threshold for ES and a 1.6-AU threshold for ELISA methods.

[Physical activity and pulmonary rehabilitation in adults with asthma]. / Apports de l'activité physique et de la réadaptation respiratoire dans l'asthme de l'adulte.

Physical activity is reduced in people with asthma compared to the general population, especially in situations where patients have uncontrolled asthma symptoms, persistent airflow obstruction and other long-term medical problems, in particular obesity and anxiety. Exertional dyspnea, which is of multifactorial origin, is the main cause of reduced physical activity reduction and draws patients into a vicious circle further impairing quality of life and asthma control. Both the resumption of a regular physical activity, integrated into daily life, adapted to patients' needs and wishes as well as physical and environmental possibilities for mild to moderate asthmatics, and pulmonary rehabilitation (PR) for severe and/or uncontrolled asthmatics, improve control of asthma, dyspnea, exercise tolerance, quality of life, anxiety, depression and reduce exacerbations. A motivational interview to promote a regular programme of physical activity in mild to moderate asthma (steps 1 to 3) should be offered by all health professionals in the patient care pathway, within the more general framework of therapeutic education. The medical prescription of physical activities, listed in the Public Health Code for patients with long-term diseases, and pulmonary rehabilitation should be performed more often by specialists or the attending physician. Pulmonary rehabilitation addresses the needs of severe asthma patients (steps 4 and 5), and of any asthmatic patient with poorly controlled disease and/or requiring hospitalized for acute exacerbations, regardless of the level of airflow obstruction, and/or with associated comorbidities, and before prescribing biological therapies.

Expectations about treatment of idiopathic pulmonary fibrosis: Comparative survey of patients, carers and physicians (the RESPIR French survey).

CONTEXT: Idiopathic pulmonary fibrosis (IPF) is a severe chronic disease during which anxiety and depression are frequent comorbidities. Better knowledge of patients' expectations is needed to inform an action plan to improve medical care. AIM: To describe feelings and expectations of patients suffering from IPF and of their carers about antifibrotic therapy and compare them to what is perceived by their pulmonologist. METHODS: National prospective study on practices and perceptions. Specific questionnaires were e-mailed to all 3276 pulmonologists in France who, in turn, invited patients and carers to participate in a survey. RESULTS: 147 pulmonologists, 161 patients and 144 carers participated in the survey. The role of the carer was evaluated as "important" or "very important" by more than 90% of participants, i.e. pulmonologists, patients or carers. Inconsistencies between how patients felt and how pulmonologists perceived them were identified: 88% of patients responded that they understood quite well what IPF is (vs. 75% of patients according to pulmonologists); 85.5% of patients said they were determined to fight the disease (vs. 68.0%); 61.7% of patients wanted to be kept informed of potential complications before they occurred (vs. 69.6%) and 81.2% wanted to be involved in therapeutic decisions (vs. 43.1%). Globally, patients had a more positive view of antifibrotic therapies than expected by pulmonologists: 41.5% evaluated their advantages superior to what they had expected (vs. 29.1% of patients according to pulmonologists) and 76.5% had a positive image of the benefits/disadvantages ratio (vs. 62.4%). Although pulmonologists had the impression that they were keeping their patients well-informed about exacerbations, hospital stays and the possible negative evolution of the disease despite antifibrotic therapies, 34.0%, 42.0% and 22.0% of patients respectively declared not being aware of these aspects. CONCLUSION: The feelings of patients suffering from IPF regarding their disease and treatment globally proved more positive compared with how pulmonologists perceived them. Taking into account the expectations and needs of patients may allow healthcare professionals to better address their needs and priorities.

Immunoreactivity for interleukin 3 and 5 and granulocyte/macrophage colony-stimulating factor of intestinal mucosa in bronchial asthma.

T lymphocytes and eosinophils are important components of the inflammatory cell infiltrate in bronchial mucosa in asthma. Because activated lymphocytes migrate through the thoracic duct and the general circulation to remote glandular and mucosal sites, we initiated this study to evaluate pathological abnormalities and immunoreactivity for interleukin (IL) 3, IL-5, and granulocyte/macrophage colony-stimulating factor (GM-CSF) of intestinal mucosa in bronchial asthma. 15 asthmatic patients, 8 nonasthmatic patients with chronic obstructive pulmonary disease, 6 atopic nonasthmatic healthy controls, and 6 nonatopic healthy controls were studied. Duodenal biopsies were performed by endoscopy. A significantly increased number of intraepithelial lymphocytes and eosinophils and a significant accumulation of mononuclear cells (lymphocytes and mast cells) and eosinophils in the lamina propria were detected in asthmatics and atopic controls. Immunostaining with antibodies directed against IL-3, IL-5, and GM-CSF was positive in asthmatics and atopic controls, whereas no staining was observed in nonatopic controls and chronic obstructive pulmonary disease. Combined ultrastructural study and immunogold labeling demonstrated that IL-3, IL-5, and GM-CSF were localized in eosinophils and mast cells. Although devoid of gastrointestinal symptoms, asthmatics and asymptomatic atopics had duodenal pathological abnormalities mimicking those observed in the bronchial mucosa in asthma, suggesting that the whole mucosal immune system is involved in bronchial asthma.

Long-term effects of pulmonary rehabilitation on daily life physical activity of patients with stage IV sarcoidosis: A randomized controlled trial.

INTRODUCTION: Pulmonary rehabilitation (PR) is known to improve exercise tolerance, mood, and quality of life in patients with chronic respiratory diseases. The aim of this work was to determine whether PR provides long-term benefits in increasing daily life physical activity in patients with chronic sarcoidosis. METHODS: This randomized prospective study (registered ClinicalTrials.gov NCT02044939) of 38 patients with stage IV chronic sarcoidosis was performed between 2012 and 2016. Patients were assigned to participate in a 2-month PR program (n=20) or receive counseling (n=18). Assessments were performed at baseline, 2 months (end of the PR program), 6months, and 12months, and included daily life physical activity parameters (measured for 5 consecutive days), exercise tolerance, dyspnea, anxiety, depression, fatigue, and quality of life. The primary outcome was the 12-month change in time spent in activities above an estimated energy expenditure of 2.5metabolic equivalents (METs). Secondary daily life physical activity outcomes included number of steps per day, total daily energy expenditure, and total energy expenditure above 2.5METs. RESULTS: The primary outcome did not differ between the two groups; mean between-group differences were -13.2min (95% confidence interval [CI]: -76.3 to 49.8) at 6 months and -18.1min (95% CI: -55.7 to 19.4) at 12months. Although PR had no effect on secondary daily life physical activity outcomes, it did significantly increase exercise tolerance at 6 and 12 months and decrease the dyspnea score at 6 months and the fatigue score at 12months. CONCLUSION: This trial failed to demonstrate a beneficial effect of PR on daily life physical activity in sarcoidosis patients, suggesting that long-term behavioral programs may be necessary to complement PR.

Influence of DISC behavioral profile on the short- and long-term outcomes of home-based pulmonary rehabilitation in patients with chronic obstructive pulmonary disease.

INTRODUCTION: Pulmonary rehabilitation (PR) programs are commonly prescribed for patients with severe respiratory disorders, but little is known about how the patient's personality traits influence PR outcomes. We analyzed the response of patients with chronic obstructive pulmonary disease (COPD) to a home-based PR program according to their predominant behavioral profiles using the Dominance - Influence - Steadiness - Conscientiousness (DISC) tool. METHODS: This was a retrospective observational study of 335 COPD patients referred by their pulmonologists between January 2010 and December 2015. The DISC behavioral profile was determined at the beginning of the program. Patients received individual supervised sessions at home once a week for 8 weeks, which consisted of exercise training and psychosocial, motivational, and educational support, all tailored to the participant's DISC profile. Exercise tolerance (6-minute stepper test, 6MST), anxiety and depression (Hospital anxiety and depression scale, HADS), and quality of life (Visual simplified respiratory questionnaire, VSRQ) were evaluated immediately before and after the PR program (T0 and T2, respectively) and then 6 and 12 months later (T8 and T14, respectively). Responders were defined as patients who exhibited at least minimal clinically important differences (improvements) from baseline. RESULTS: Of the 335 COPD patients, 102 (30.4%), 98 (29.3%), 82 (24.5%), and 53 (15.8%) were classified as having predominant D, I, S, and C behavioral traits, respectively. All four patient groups showed significantly (P<0.01) improved performance in the 6MST, HADS, and VSRQ evaluations at T2 (n=300), T8 (n=262), and T14 (n=231) compared with T0, and the proportion of responders in all groups at T8 and T14 was high (∼60%). The percentage of responders differed significantly between groups only at T2, when the S group contained fewer responders on the HADS anxiety subscale. Most patients who did not complete the study were classified as D type (42/102, 41.2%), followed by I (28/98, 28.6%), S (22/82, 26.8%), and C (12/53, 22.6%) types. CONCLUSION: The personality profile of COPD patients influenced their adherence to, but not their benefit from, a home-based PR program. The high proportion of patients in all personality groups showing significant improvements in outcomes supports a personalized approach to the design of PR programs.

Lung function in Birt-Hogg-Dubé syndrome: a retrospective analysis of 96 patients.

BACKGROUND: Birt-Hogg-Dubé syndrome (BHD) is a rare autosomal dominant disorder caused by mutations in the FLCN gene coding for folliculin. Its clinical expression includes cutaneous fibrofolliculomas, renal tumors, multiple pulmonary cysts, and recurrent spontaneous pneumothoraces. Data on lung function in BHD are scarce and it is not known whether lung function declines over time. We retrospectively assessed lung function at baseline and during follow-up in 96 patients with BHD. RESULTS: Ninety-five percent of BHD patients had multiple pulmonary cysts on computed tomography and 59% had experienced at least one pneumothorax. Mean values of forced expiratory volume in 1 second (FEV1), forced vital capacity (FVC), FEV1/FVC ratio, and total lung capacity were normal at baseline. Mean (standard deviation) residual volume (RV) was moderately increased to 116 (36) %pred at baseline, and RV was elevated > 120%pred in 41% of cases. Mean (standard deviation) carbon monoxide transfer factor (DLco) was moderately decreased to 85 (18) %pred at baseline, and DLco was decreased < 80%pred in 33% of cases. When adjusted for age, gender, smoking and history of pleurodesis, lung function parameters did not significantly decline over a follow-up period of 6 years. CONCLUSIONS: Cystic lung disease in BHD does not affect respiratory function at baseline except for slightly increased RV and reduced DLco. No significant deterioration of lung function occurs in BHD over a follow-up period of 6 years.

[Allergic asthma due to sausage mould]. / Asthme allergique à la "fleur de saucisson"

INTRODUCTION: Chronic cough is a common reason for consultation in respiratory medicine. The three most frequent causes of chronic cough are asthma, postnasal drip syndrome or rhinosinusitis, and gastro-oesophageal reflux disease. CASE REPORT: We describe a case of chronic cough related to occupational asthma with sensitivity to dry sausage mould (Penicillium nalgiovensis) in a worker in a semi-industrial pork butchers. The diagnosis was based on the history of symptoms, positive skin prick-tests, spirometry, a normal pulmonary CT-scan, and a favourable outcome after avoidance of the allergen. It was notified as a case of occupational disease. CONCLUSION: A diagnosis of asthma should be considered in a worker exposed to dry sausage mould presenting with respiratory symptoms like chronic cough without evidence of hypersensitivity pneumonitis. Skin prick-tests and allergen avoidance are useful in the diagnosis.

[Home-based pulmonary rehabilitation in idiopathic pulmonary fibrosis]. / Réhabilitation respiratoire à domicile au cours de la fibrose pulmonaire idiopathique.

INTRODUCTION: Idiopathic pulmonary fibrosis (IPF) is a severe chronic lung disease. Pulmonary rehabilitation could improve the quality of life of patients with this condition. METHODS: We prospectively evaluated the impact of an 8 week home-based pulmonary rehabilitation program over 10 months in stable patients suffering from IPF. Exercise capacity, pulmonary function, dyspnea and quality of life were analyzed before and after the rehabilitation program. RESULTS: 17 patients were included and 13 completed the study. Mean FVC was 2.15+/-0.79 L and mean DLCO was 7.81+/-3.99 ml/min/mmHg. Six patients were treated with low dose oral steroids ($20 mg/day of prednisone) with or without immunosuppressive treatments; 6 were taking part in therapeutic trials. Mean endurance time (7.4+/-9.1 min vs 14.1+/-12.1 min; p<0,01), number of beats per minute on a stepper (322+/-97 vs 456+/-163; p=0.026), 6 min walking distance to heart rate ratio (11+/-6 vs 17+/-12; p=0.006), exercise dyspnea (p=0.026), sensation of physical limitation at the SF-36 (25%+/-26 vs 49%+/-38; p=0.047) and 4 out of 7 visual analog scales were significantly improved after rehabilitation. In contrast, no significant difference was observed in resting pulmonary function or in other items of quality of life questionnaires CONCLUSION: A home-based program of pulmonary rehabilitation is feasible in IPF patients. It significantly improves endurance parameters and physical limitation in this patient group without changing pulmonary function.

[The DISC tool improves communication and results in pulmonary rehabilitation]. / L'outil DISC améliore la communication et les résultats de la réhabilitation respiratoire : une approche comportementale adaptée.

INTRODUCTION: Competence in personal relationships is essential for a caregiver, especially in pulmonary rehabilitation (PR). Considering the behavioral profile of patients might help to optimize their management and the results of PR. METHODS: We evaluated eight hundred and thirty-two consecutive patients with chronic respiratory disease who received eight weeks of home-based PR. Their exercise tolerance (six-minute stepper test, 6MST), mood (HAD), and quality of life (VSRQ, MRF28) were evaluated at the beginning and end of PR. For six hundred and ninety patients, a behavioral approach was implemented at the beginning of PR by using the DISC tool to identify four behavioral profiles: dominance, influence, steadiness, conscientiousness. The remaining 142 patients served as the control group. RESULTS: Subjectively, the therapeutic alliance was more easily established with the behavioral approach. Compared with the control group, patients with the "steadiness" profile were younger (60.7±12 years) and mostly female (52.8%), whereas patients with the "conscientiousness" profile were older (67.5±10.6 years) and mostly male (85.5%). The four behaviorally profiled groups showed no differences in exercise tolerance, mood, or quality of life scores at baseline. Globally, all patients improved their exercise tolerance, mood and quality of life. The percentage of responders to 6MST and VSRQ (>MCID) was 7.5% and 5.3% higher with the behavioral approach. For non-responders to 6MST and VSRQ (

[Cardiopulmonary exercise testing for the diagnosis of unexplained dyspnea: a review of 194 cases]. / Épreuve fonctionnelle à l'exercice et dyspnée inexpliquée : à propos de 194 cas.

INTRODUCTION: Chronic dyspnoea that remains unexplained after resting pulmonary function and cardiovascular testing is a common problem in clinical practice. The aim of this study was to determine the utility of cardiopulmonary exercise testing (CPET) in the diagnosis of unexplained dyspnoea. METHODS: This retrospective single-centre study included consecutive patients with dyspnoea who had normal resting cardiopulmonary examinations (including chest X-ray, electrocardiography, pulmonary function tests [PFTs], and cardiac ultrasound). CPET was performed using a cycle ergometer with analysis of blood gases. The results were interpreted as being most likely due to one of the six pathophysiological mechanisms shown below. Consensus required agreement between at least three of the authors. RESULTS: Of the 194 patients included (median age 53 years, sex-ratio (M:F) 0.83, mean body mass index 27.3±5.36kg/m2), 32% of the test profiles were compatible with deconditioning, 20% with inappropriate hyperventilation (without gas exchange abnormalities), 18% with disorders of gas exchange, 13% with sub-maximal CPET, 9% with cardiovascular anomalies, and 8% with normal CPET. Of the patients with gas exchange abnormalities, the most common causes were bronchiectasis (6), emphysema (6), recent pneumonia (2), and diffuse interstitial pneumonitis (2). Ten of the patients with cardiovascular abnormalities had chronotropic insufficiencies, 5 had excessive tension responses, and 3 had disorders of rhythm or repolarisation. CONCLUSIONS: CPET may greatly facilitate the diagnosis of unexplained dyspnoea. More than 50% of the dyspnoea cases examined here were due to deconditioning or hyperventilation syndrome and would benefit from a simple pulmonary rehabilitation program.

Improvement in body composition following a supervised exercise-training program of adult patients with cystic fibrosis.

OBJECTIVES: Maintenance of optimal nutritional status is a crucial issue for cystic fibrosis (CF) patients. Here, we evaluate the effects of an 8-week exercise training (ET) program on body composition in CF patients. METHODS: This prospective pilot observational study was conducted in adult CF subjects in stable condition following their annual check-up. The ET program consisted of three sessions per week and included aerobic training (≥30min), muscle strengthening, circuit training, and relaxation. Exercise tolerance (6-minute walk test, 6MWT), pulmonary function, quadriceps isometric strength, and body composition (bioelectrical impedance analysis of fat-free mass [FFM], fat mass, and body cell mass) were analyzed before and immediately after the ET program. A control group of CF patients who preferred not to participate in the ET program received the same evaluations. RESULTS: A total of 43 CF patients were enrolled and offered the ET program; 28 accepted (aged 28±5 years, forced expiratory volume in 1s [FEV1] 48.8±19% predicted) and 15 declined the ET program but agreed to be part of the control group (matched for age and CF severity: 30.8±9 years, FEV1 51.8±16.5%). Pulmonary function was unchanged at the end of the ET program, but significant improvements were observed in 6MWT distance (from 520±96m to 562±105m, P<0.001) and muscle strength (331±141N to 379±168N, P<0.001). Although mean body mass index did not change, the ET group showed significantly increased FFM (43.85±8kg to 44.5±9.2kg, P=0.03) and a trend towards increased body cell mass (21.4±6 to 22.1±6.6kg, P=0.06). All other parameters were unchanged by ET. There were no significant correlations between the increase in FFM and the improvements in either 6MWT distance or muscle strength. The CF control group exhibited no significant changes in any parameters between evaluations. CONCLUSIONS: ET significantly improved FFM, but not body mass index, in CF patients. The results illustrate the superiority of bioimpedancemetry for assessing changes in body composition and reveal the importance of ET for improving not only exercise tolerance but also nutritional status in these patients.

Interstitial lung disease and anti-Jo-1 antibodies: difference between acute and gradual onset.

AIM: A multicentre retrospective study was undertaken to examine patients with interstitial lung disease (ILD) with the initial clinical manifestation of an anti-synthetase syndrome (anti-Jo-1 antibodies), and to analyse the characteristics and long-term outcome of these patients according to their clinical presentation (acute or gradual onset), treatment and adverse events related to treatment. METHODS: 32 patients, 15 (47%) presenting with acute onset and associated respiratory insufficiency (group A) and 17 (53%) with gradual onset (group G) were examined. Myositis was diagnosed at admission in only 31% of cases and was observed during follow-up in 56% of cases, but the prevalence did not differ between the two groups. RESULTS: Fever and radiological patterns including diffuse patchy ground-glass opacities, basal irregular lines and consolidation on high-resolution CT scan were more frequent in group A than in group G. More patients in group G had neutrophils in the bronchoalveolar lavage fluid and autoantibodies other than anti-Jo-1 (rheumatoid factor, anti SSa/SSb) than in group A. The percentage of patients in whom the ILD improved at 3 months was significantly higher in group A than in group G (13/15 vs 9/17; p = 0.006). In contrast, after 12 months, most patients with ILD progression were in group A and were treated with corticosteroids alone. A combination of corticosteroids and an immunosuppressive drug was required in most cases (84%) at the end of the follow-up period. Severe adverse effects of treatment were observed and varicella zoster virus infection was frequent. CONCLUSIONS: Early testing for anti-synthetase antibodies, particularly anti-Jo-1, and creatine kinase determination are useful procedures in patients presenting with ILD. Treatment with corticosteroids and immunosuppressive drugs is required in most patients. At the end of the study, around two-thirds of patients had stable ILD while the other third had disease progression with respiratory insufficiency.

Airway remodeling is correlated with obstruction in children with severe asthma.

BACKGROUND: Severe asthma may involve an irreversible obstructive pattern, and structural changes in bronchial airways are believed to play a key role in this context. The aim of the present study was to compare airway remodeling in severe asthmatic children with or without obstructive pattern. METHODS: Two groups of children with severe asthma and persistent symptoms, 5-14 years old were included, 15 with persistent obstructive pattern (group O) and 10 without obstructive pattern (group N). Persistent obstructive pattern was defined as a forced expiratory volume in 1 s (FEV(1)) less than 80% of the predicted value after a course of systemic corticosteroids and no significant improvement after bronchodilator. We examined bronchial biopsies by pathological and immunochemical methods and quantified airway smooth muscle (ASM) and mucus gland areas, reticular basement membrane (RBM) thickening, distance between ASM and RBM, muscle light chain kinase (MLCK) expression and number of vessels (CD31 expression). RESULTS: Surface area of ASM (P = 0.009), MLCK expression (P = 0.03) and number of vessels (P = 0.0008) were increased in group O compared with group N. Distance of RBM-ASM was shorter in group O (P = 0.007). FEV(1) negatively correlated with ASM area (r = -0.6; P = 0.002), MLCK expression (r = -0.45; P = 0.02) and CD31 expression (r = -0.7; P = 0.0003), and positively correlated with the distance of RBM-ASM (r = 0.5; P = 0.007). CONCLUSIONS: Structural abnormalities of airway remodeling are present in children with severe asthma. Only an increase in surface area of ASM and the density of the vascular network are more pronounced in children with persistent obstructive pattern, while RBM thickening is similar. These results are concordant with longitudinal studies which emphasize the precocity of bronchial obstruction.

Effects of oxygen on exercise-induced increase of pulmonary arterial pressure in idiopathic pulmonary fibrosis.

INTRODUCTION: Idiopathic pulmonary fibrosis (IPF) is a severe disease with no known effective therapy. Patients with IPF may develop severe increase of pulmonary arterial pressure (PAP) on exercise, the mechanisms of which is not clearly identified. OBJECTIVES: To determine whether oxygen may correct the increase of PAP developed during exercise in patients with IPF. PATIENTS AND METHODS: We performed a prospective study on patients with IPF and no hypoxaemia at rest. The absence of pulmonary hypertension (PH) at rest was confirmed by echocardiography (systolic PAP <35 mmHg). Eight patients underwent echocardiography during exercise in air and with oxygen (to maintain saturation of at least 94%). Right ventricle-right atrium gradient and cardiac output were measured at rest, after each increment and at peak. We then compared the echocardiographic results obtained for air and oxygen. RESULTS: All patients developed significant increase of SPAP on exercise (73 +/- 14 mmHg in air). Oxygen did not significantly improve SPAP on exercise (SPAP: 76 +/- 15 mmHg). Echocardiographic characteristics were similar between air and oxygen except for exercise tolerance in term of workload (p=0.045) and endurance (p=0.017). Resting pulmonary function tests did not predict the occurrence of increase of PAP on exercise. CONCLUSION: Our results demonstrate that oxygen does not improve exercise-induced increase of PAP in patients with IPF and support the hypothesis that hypoxic vaso-constriction is not the main mechanism of acute increase of PAP during exercise.

[Inspiratory muscle myopathy and antisynthetase syndrome]. / Myopathie des muscles inspiratoires au cours du syndrome des antisynthétases.

Respiratory muscle involvement is a rarely reported manifestation of inflammatory myopathies. We report the case of a 38-year-old woman with antisynthetase syndrome who presented with inflammatory myopathy and acute pulmonary interstitial involvement that initially improved with corticosteroids and immunoglobulins. A few months later dyspnoea resumed despite the absence of evidence of interstitial deterioration or other common diagnosis including vascular or infectious disorders. Isolated respiratory muscular involvement was evidenced by functional testing. Disease course was favourable with corticosteroids and immunoglobulins. Inflammatory myopathy of the antisynthetases syndrome could be limited or mainly expressed to respiratory muscles. Unexplained dyspnoea should call in mind a muscular respiratory myositis.

[Functional exercise testing in idiopathic inflammatory myopathies with pulmonary involvement]. / Intérêt de l'épreuve fonctionnelle d'exercice au cours des myopathies inflammatoires avec atteinte pulmonaire.

INTRODUCTION: Dyspnoea occurring during inflammatory myopathy associated with interstitial lung disease (ILD) can be ascribed to several physiopathological pathways. The purpose of this study was to analyse cardiopulmonary exercise testing (CPET) abnormalities in these patients in order to precise mechanisms responsible for decreased aerobic capacity. METHODS: Ten patients (52 +/- 12 years) were diagnosed as having inflammatory myopathy with interstitial lung disease. Exercise dyspnoea was found in 9 patients. All of them had a restrictive pattern associated to and impairment of the transfer factor for carbon monoxide. CPET was performed with a bicycle ergometer using a standard protocol of incremental increasing work load. RESULTS: Two patients had a normal exercise capacity while eight patients had abnormal VO2 associated with hyperventilation, abnormal gas exchange, as well as and abnormal dead space. Five patients also exhibited decreased oxygen pulse (<80% expected value) at peak exercise; 4 out of 5 had persistence of ventilatory reserve. In addition DeltaFC/DeltaVO2 was increased (>50) in 7 cases. CONCLUSION: CPET provides useful information in the understanding of mechanisms of dyspnoea and might be an effective tool in treatment decision making: in patients with inflammatory myopathy and associated ILD abnormal oxygen pulse may reflect cardiac disorder or muscular abnormalities (inflammatory or steroid myopathy).

[Theoretical and practical assessment of Lille general practice and pharmacy students' knowledge about use of inhaler devices for asthma control]. / Évaluation théorique et pratique de la connaissance d'utilisation des dispositifs inhalés dans l'asthme chez les internes en médecine générale et les étudiants en pharmacie de Lille.

INTRODUCTION: Asthma is a potentially serious chronic respiratory disease impacting patients quality of life. Satisfactory control requires proper use of inhaled devices. This study assesses general medical residents and pharmacy students knowledge about proper use of inhaled asthma devices. MATERIALS AND METHODS: We evaluated knowledge of 43 general practice students and 43 pharmacy students in Lille for three inhaler devices (metered-dose inhaler, Turbuhaler® and Diskus®) during individual interviews. Students were assessed on 8 proper use criterias for each device. RESULTS: General practice and pharmacy students are unfamiliar with proper use of inhaler devices. However, pharmacy students get better average scores than general practice students for all devices included in this study: 6.3/8 respected criterias against 5/8 for metered-dose inhaler; 5.3/8 against 3.2/8 for Turbuhaler®; and 6/8 against 4.3/8 for Diskus®. Pharmacy students more frequently perform a demonstration of proper use to patients when a device is first prescribed or when a prescription is renewed; general practice students more frequently ask patients themselves to perform a demonstration of proper use. CONCLUSION: Introducing trainings workshops for inhaler devices to pharmacy and general practice students appears appropriate in order to promote therapeutic patient education, to increase asthma control and better patients life quality.

[Alveolar hemorrhage and cocaine use]. / Hémorragies intra-alvéolaires et consommation de cocaïne.

In France, cocaine is the second most commonly illicit drug used after cannabis. Cocaine, mainly smoked in the form of crack, can be responsible for a wide range of respiratory disorders. The aim of this systematic literature review was to clarify what is known about the link between cocaine use and alveolar hemorrhage. We performed a Medline search covering the period 1980-2016 and collected data from 84 articles. The number of acute forms described in the literature is small but postmortem studies show that hidden forms are frequent. The diagnosis is based on the association of hemoptysis, anemia and diffuse alveolar opacity. Bronchoalveolar lavage shows hemosiderin-laden macrophages. Of the 13 cases identified, 12 patients had hemoptysis, anemia and diffuse alveolar infiltration on pulmonary or chest CT. In 9 cases, the presence of hemosiderin-laden macrophages was identified in bronchoalveolar lavage or pulmonary biopsy. Except for 2 deaths, the outcome was favorable when cocaine use was stopped. Systematic interventions to help people stopping using this psychoactive substance will protect lung health.