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INTRODUCTION: While pulmonary rehabilitation is registered in the COPD management guidelines with a high level of evidence, it is not adequately performed in accordance with patients' needs. Advanced nurse practitioners (IPA, in French) provide expertise enabling improved access and enhanced implementation of rehabilitation programs. Based on foreign experience, the present study has been designed to propose avenues for the development of more effective practices in France. METHODS: Using Google Scholar and Pubmed databases, a search algorithm was used to identify international publications from 2003 to 2022 dealing with the role of nurse practitioners (IP, in French) in respiratory rehabilitation for COPD patients. RESULTS: Fourteen articles, mainly from English-speaking countries, were analysed. The involvement of nurse practitioners in pulmonary rehabilitation is very broad-based, their main missions being initial disease assessment, therapeutic education, improved professional sensitivity and patient follow-up at all stages of a rehabilitation program. CONCLUSION: Nurse practitioners have a major role in the implementation of pulmonary rehabilitation programs for COPD patients. Their involvement at different levels can improve access and success of programs over time.

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INTRODUCTION: Cardiac MRI is increasingly used to assess and monitor pulmonary vascular disease. STATE OF THE ART: In pulmonary arterial hypertension, the role of cardiac MRI has become more clearly defined due to its proven capacity to assess the morphology and function not only of the heart, but also of pulmonary circulation. Complementarily to echocardiography, technological advances have rendered it possible for MRI to search and assess shunts. More generally, MRI provides prognostic information on the follow-up of patients undergoing treatment. In cases of chronic thromboembolic pulmonary hypertension, chest MRI locates lesions and assesses pulmonary microcirculation, thereby guiding therapeutic choice. It is also an important prognostic marker in diagnosis and follow-up of patients undergoing treatment. To ensure high-quality examination, it is essential that the patient having to carry out repeated apneas cooperate. PROSPECTS: Studies are ongoing in view of clarifying the role of cardiac MRI as compared to right cardiac catheterization in the follow-up of patients with pulmonary arterial hypertension. CONCLUSIONS: Cardiac MRI is the examination of choice in assessment of right ventricular morphology and function. It is a minimally invasive technique with good inter- and intra-operator reproducibility in the evaluation of patients with pulmonary arterial hypertension and chronic pulmonary thromboembolic hypertension.

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INTRODUCTION: Depression is a common comorbidity in COPD patients, worsening their quality of life and their current level of physical activity. Respiratory rehabilitation is therefore highly recommended for COPD patients but only few of them have access to that kind of program. In real life, exercise training is often the only therapeutic activity provided to patients. METHODS: The aim of this study was to evaluate the specific effect of exercise training on the level of depression (using Beck Depression inventory short form (BDI-SF)) and quality of life in COPD patients [using the Saint George's Respiratory Questionnaire (SGRQ)]. Five hundred and fifteen COPD patients were enrolled in home-based exercise training programs. 421 programs were completed and the data was available for 182 patients (SGRQ, BDI-SF et TE) at T0 and T12. RESULTS: Dyspnoea (mMRC), SGRQ, BDI-SF scores and TE were significantly improved by the exercise training: mMRC 2,7±0,9 vs. 2,3±1,2; SGRQ 45±15 vs 34±18; BDI-SF 4,2±5,1 vs. 2,7±4,3; TE 6,4±5,4 vs. 17,2±12,8 respectively at T0 and T12. The improvement of TE was significantly correlated to that of the SGRQ scores (r=0,4; P<0,001) and of the BDI-SF scores (r=-0,24; P=0,001). CONCLUSION: This home-based exercise training program improved dyspnoea, quality of life, depression and exercise capacity. The improvement of the TE and BDI-SF scores were correlated.

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BACKGROUND: The intrathoracic manifestations of IgG4-related disease include a range of conditions and severity, and can on occasion cause acute respiratory failure as reported in the case described here. OBSERVATION: A 69-year-old male former smoker, was admitted to our hospital with dyspnea, fever, cough, fatigue, and a 3-month history of weight loss. He received high flow oxygen therapy and non-invasive ventilation for severe respiratory failure. Chest computed tomography revealed multifocal condensations and ground glass opacities, accompanied by thickening of the perilymphatic interstitium, mediastinal lymphadenopathy and bilateral pleural effusion. Elevated serum concentrations of IgG4 suggested an IgG4-Related Disease. He developed renal failure and underwent a renal biopsy. Histopathological analysis of which supported the diagnosis by showing dense lymphocytic infiltrate with a count of IgG4+ cells/hpf higher than 60, and storiform fibrosis - a swirling, "cartwheel" pattern of fibrosis which may have a patchy distribution. The patient responded well to steroid therapy. CONCLUSION: Although respiratory symptoms are usually mild in IgG4-relatd disease, thoracic features can evolve into acute respiratory failure with few extra thoracic manifestations.

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Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension. Heritable and sporadic forms have been distinguished. Hypoxemia, profound reduction in the diffusion of carbon monoxide and haemodynamic confirmation of pre-capillary pulmonary hypertension are the major diagnostic criteria. Thoracic CT scanning and a response to pharmaceutical therapy provide additional information to confirm the diagnosis. A 52-year-old patient, three of whose siblings had pulmonary hypertension, was admitted with dyspnoea, malaise and palpitations. Right heart catheterisation confirmed pre-capillary pulmonary hypertension. A search for an EIF2AK4 mutation was carried out, and this showed a composite biallelic heterozygous mutation compatible with the diagnosis of familial PVOD, identical to that showed in one of his brothers. Given the signs of severity of the disease and the diagnosis of PVOD, whose response to pharmaceutical therapy is often poor, the patient was placed on a waiting list for lung transplantation. Despite a similar diagnosis in 3 brothers and follow-up proposed 11 years before the diagnosis, pulmonary hypertension appeared within a few weeks and led immediately to a severe clinical situation. Annual clinical and echocardiographic monitoring had been strongly advised to the patient, but had not allowed diagnosis at a mild or moderate stage of the disease. This clinical case shows that the identification of factors predicting the development of heritable PVOD at a pre-symptomatic stage is an important issue for clinical research.

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INTRODUCTION: Eosinophilic pneumonias are characterized by an increase in lung eosinophils. These disorders can be induced by drug reactions. CASE REPORT: A 57-year-old woman suffering from bipolar disorder and treated by sodium divalproate for more than 2 years was hospitalised in the department of respiratory medicine for dyspnoea and cough. The investigations showed severe hypoxaemia, airflow limitation, multiple ground-glass opacities and crazy paving on the chest CT-scan and a blood eosinophilia. A significant alveolar eosinophilia was found in the broncho-alveolar lavage. A complete assessment of possible causes was made. Finally, we made the diagnosis of eosinophilic pneumonia secondary to sodium divalproate. The treatment was stopped and systemic corticosteroid therapy was not introduced. The patient showed an improvement of her dyspnoea in a few days. Lung function and the CT-scan were normal within a few months. CONCLUSIONS: Sodium divalproate, frequently used in the treatment of bipolar disorder, is a rare cause of eosinophilic lung disease, even years after its introduction. Rapid diagnosis and withdrawal of treatment led to complete resolution in the reported case.

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INTRODUCTION: Pulmonary hypertension (PH) is a life-threatening haemodynamic condition associated with many disorders. In pulmonary arterial hypertension (PAH) and chronic thrombo-embolic pulmonary hypertension, several treatments acting against pulmonary endothelial dysfunction have been shown to be effective. Exposure to cigarette smoke leads to endothelial dysfunction which is comparable to that observed in patients with PAH. The main objective was to investigate the difference in the haemodynamic changes during exercise in patients under specific treatment, from diagnosis (T0) to the first reassessment visit with a right heart catheterisation (T1), between those exposed or not exposed to cigarette smoke. MATERIALS AND METHODS: Between January 2002 and December 2018, all patients under specific treatment for PAH or chronic thromboembolic PH who underwent supine submaximal exercise assessment at T0 and T1 in our institution were asked specific questions about tobacco smoking. RESULTS: Overall (n=109), difference in mean isoflow mPAP was 19.0±15.4mmHg in patients exposed to tobacco smoke versus 21.3±16.6mmHg in unexposed patients (P=0.59). Median survival in exposed group was 83 months while in the unexposed group, median survival was 100 months (P=0.099). DISCUSSION: Our study did not show any significant difference in terms of haemodynamic changes during exercise between a group of patients under specific PH treatment exposed to cigarette smoke and a group unexposed. Endothelial dysfunction induced by smoke exposure does not appear to have a major impact on the response to the specific PH treatment.

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BACKGROUND: Breathlessness is the prominent symptom of chronic obstructive pulmonary disease (COPD). Despite optimal therapeutic management including pharmacological and non-pharmacological interventions, many COPD patients exhibit significant breathlessness. Chronic breathlessness is defined as breathlessness that persists despite optimal treatment of the underlying disease. Because of the major disability related to chronic breathlessness, symptomatic treatments including opioids have been recommended by several authors. The prevalence of chronic breathlessness in COPD and its management in routine clinical practice have been poorly investigated. Our aim was to examine prevalence, associated characteristics and management of chronic breathlessness in patients with COPD recruited in a real-life tertiary hospital-based cohort. METHODS: A prospective study was conducted among 120 consecutive COPD patients recruited, in stable condition, at Nancy University Hospital, France. In parallel, 88 pulmonologists of the same geographical region were asked to respond to an on-line questionnaire on breathlessness management. RESULTS: Sixty four (53%) patients had severe breathlessness (modified Medical Research Council scale≥3), despite optimal inhaled medications for 94% of them; 40% had undergone pulmonary rehabilitation within the past 2 years. The severity of breathlessness increased with increasing airflow limitation. Breathlessness was associated with increased symptoms of anxiety, depression and with osteoporosis. No relation was found with other symptoms, exacerbation rate, or cardiovascular comorbidities. Among the patients with chronic breathlessness and Hospitalized Anxiety and/or Depression score > 10, only 25% were treated with antidepressant or anxiolytic. Among the pulmonologists 46 (52%) answered to the questionnaire and expressed a high willingness to prescribe opioids forchronic breathlessness, which contrasted with the finding that none of these patients received such treatments against breathlessness. CONCLUSION: Treatment approaches to breathlessness and associated psychological distress are insufficient in COPD. This study highlights underuse of pulmonary rehabilitation and symptomatic treatment for breathlessness.

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INTRODUCTION: The natural history of orphan lung diseases is often unclear. We report the long-term follow-up of a case of bronchiectasis due to pulmonary non amyloid light chain deposition disease (LCDD). CASE REPORT: A 50-year-old woman who was a smoker, was diagnosed with diffuse thin walled bronchiectasis of uncertain origin after presenting with a respiratory tract infection. Ten years later, the combination of bronchiectasis, the appearance of pulmonary cysts and the identification of increased kappa free light chains evoked the diagnosis of pulmonary LCDD. The diagnosis was confirmed by lung biopsy. No immunoproliferative disorder was identified. During the 12 years follow-up, dyspnea worsened progressively and bronchiectasis and lung cysts extended leading to multicystic lung disease. Pulmonary function tests did not show any ventilatory defect but a small decrease in carbon monoxide transfer factor occurred. CONCLUSION: We describe the evolution of a rare presentation of isolated pulmonary LCDD, characterized by cystic diffuse atypical bronchiectasis with thin walls, associated with progressive cystic destruction of the lung parenchyma. The possibility of pulmonary LCDD should be considered in cases of atypical bronchiectasis of unknown etiology.

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INTRODUCTION: The anti programmed death-1 (PD-1) and the programmed death ligand 1 (PD-L1) antibodies are used as immunotherapies in the treatment of many solid tumours. Cases of interstitial pneumonitis induced by anti PD-1 have been widely described, but there are fewer data with anti PD-L1. Avelumab is a new immunotherapy of the anti PD-L1 class. CASE REPORT: A 66-year-old woman, ex-smoker, had been treated with avelumab and axitinib since November 2016 for renal cell cancer. Interstitial pneumonitis was discovered accidentally 4 months after the beginning of the treatment, with ground glass opacities, intra-lobular crosslinking and adenopathy of the 4R zone on the CT scan. An exhaustive assessment did not reveal any respiratory function defect or an infectious or immunological cause. The radiological abnormalities regressed spontaneously after cessation of treatment confirming the diagnosis of drug-induced pneumonitis. CONCLUSION: Avelumab can induce interstitial lung disease. The mechanism is uncertain and requires further studies. Monitoring of respiratory function and CT scanning are necessary for its early management.

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Haemodynamic follow up in pulmonary arterial hypertension (PAH) is currently based on right heart catheterisation (RHC). The primary objective of the EVITA study is to compare the use of cardiac magnetic resonance imaging (cMRI) with RHC in the identification of an unfavourable hemodynamic status. The secondary objectives are to determine the role of cMRI in the follow up process. Patients will undergo at diagnosis and at follow up visits both RHC and cMRI. Patients will be followed and treated according to the current guidelines. The primary endpoint will be an unfavourable haemodynamic status defined by cardiac index<2.5L/min/m2 or a right atrial pressure≥8mm Hg measured with RHC compared with a cardiac index<2.5L/min/m2 or right ventricle ejection fraction<35% or an absolute decrease of 10% from the previous measurement with cMRI. Exact values of sensitivity, specificity and 95% confidence intervals will be computed. A population of 180 subjects will have a power of 90% with an α risk of 5%. Univariate and multivariate Cox analysis will allow answering to the secondary objectives. We expect to demonstrate that cMRI could be partly used instead of RHC in the follow up of patients with PAH.

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INTRODUCTION: Pulmonary arterial hypertension is a pulmonary vascular disease with a poor prognosis. Continuous intravenous treatment with prostacyclin analogues requires the placement of a tunnelled catheter. The occurrence of catheter-related infections in this context is unusual due to the alkaline pH of the prostacyclin analogue solutions. OBSERVATION: A 50-year-old patient with inherited pulmonary artery hypertension, treated with bosentan, sildenafil and epoprostenol, experienced generalized malaise associated with a weight loss of 9kg over a 12-month period without evidence of a source of infection or malignancy. There was no evidence of hemodynamic disturbance. The diagnosis was made after 1 year of follow-up, when the patient presented with a 38° fever and a biological inflammatory syndrome. Repeated peripheral blood cultures were positive for Dietzia, an alkalophilic coryneform bacillus. The patient's condition responded favourably to antibiotic therapy. CONCLUSION: Infection of a tunneled intravenous catheter should be considered in the case of non-specific symptoms or where there is evidence of sepsis, in patients treated with intravenous prostacyclin analogues administered intravenously. In this context, the laboratory should be warned to search for slow-growing organisms.

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OBJECTIVE: Obstructive sleep apnea-hypopnea syndrome (OSAHS) seems to be underdiagnosed. The aim of this study was to assess awareness of OSAHS among the general population of the Lorraine Region of France. METHODS: A descriptive epidemiological study was carried out from July to November 2015 in the Lorraine Region, using an anonymous questionnaire that assessed knowledge of OSAHS-related symptoms and complications. The survey was also circulated on the Internet via social media. Exclusion criteria comprised age under 18 years, refusal to fill out the questionnaire and linguistic barrier. RESULTS: 1307 subjects filled out the survey: 1020 on paper format and 287 via the Internet. About two-thirds of the population recognized a majority of symptoms. However, there was a significant lack of knowledge of complications, especially cardiological and neurological. Suffering from OSAHS, having had higher education, and being under 40 years of age, were factors linked to better awareness of the syndrome. Internet respondents also showed better awareness. CONCLUSION: Despite encouraging results regarding OSAHS symptoms, the general population showed limited awareness of its complications. Innovative educational campaigns must be organized to inform practitioners and the general public about the disease and raise awareness of its complications.

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INTRODUCTION: The pneumonic form is very characteristic of lepidic pattern adenocarcinoma of the lung. However, the diagnosis and treatment of the disease when it presents in this way may be delayed by atypical radiological findings and severity of hypoxemia. CASE REPORT: A 48-year-old female, non-smoker, asthmatic since the age of 20 years, was hospitalized for a diffuse infiltrative pneumonia complicated by severe respiratory failure. The history included chronic cough, gradually increasing dyspnoea on exertion lasting for 14 months and the onset of haemoptysis of low abundance associated with arthralgia in the previous month. She had no professional or domestic exposure to any risk factors. Chest CT scan revealed bilateral alveolar condensations, ground glass areas with thickened septa creating a crazy paving pattern, and numerous large cysts of various sizes and locations, often with irregular thin walls. Microbiological and immunological tests were negative. She required early invasive ventilation and then venovenous ECMO together with broad-spectrum empiric antibiotic therapy, but died after 39 days in intensive care. The autopsy revealed lesions consistent with mucinous lepedic adenocarcinoma with no EGFR mutation and KRAS mutation. There was also associated pulmonary suppuration. CONCLUSION: Mucinous lepidic adenocarcinoma is an alternative diagnosis for pneumonic consolidation associated with multiple cysts.

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INTRODUCTION: Alpha-1 antitrypsin deficiency is a hereditary disease defined at the biological level by a serum alpha-1 antitrypsin level below 11µM/L. The null variants are characterized by undetectable circulating alpha-1 antitrypsin levels. Suspicion of a null variant requires the use of appropriate diagnostic techniques. CASE REPORT: We report the case of a 33-year old patient presenting with dyspnea on exertion, associated with a moderate airflow obstruction, incompletely reversible. His tobacco use was less than 3pack-years. The thoracic CT-scan showed emphysema. The serum alpha-1 antitrypsin level was collapsed. Phenotyping by isoelectrofocusing on agarose gels did not show any band. The study of the SERPINA1 gene, by PCR-sequence of the II, III, IV and V exons and the flanking intronic sequences, allowed identification of the NullQ0ourém allele in homozygous state. This mutation was found in heterozygous state in both parents of the index case and in one of his brothers. The index case showed a rapid aggravation of the airflow obstruction. CONCLUSION: In the case of a serum alpha-1 antitrypsin deficiency, the analysis of the phenotype of the protein by isoelectrofocusing must be performed as a first-line investigation. The detection of an atypical profile may suggest the presence of deficient alleles other than the PI S and PI Z alleles that can only be characterized by sequencing of the whole SERPINA1 gene. The patients carrying a null mutation have a high risk of severe chronic obstructive pulmonary disease.

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INTRODUCTION: Bronchiolo-alveolar carcinoma is a primary pulmonary adenocarcinoma developing in the terminal respiratory unit. CASE REPORT: An 84-year-old non-smoker woman with a history of untreated acute myeloid leukaemia was referred to the intensive care unit for pneumonia and acute respiratory failure. The patient reported dyspnoea and a productive cough for 3 months, treated by antibiotics and steroids without improvement. Thoracic CT-scan showed alveolar condensations and multiple nodular lesions. All microbiological samples were negative and the evolution was fatal within 72 hours despite empirical antibiotic therapy and noninvasive ventilation. Post-mortem lung biopsies gave a diagnosis of mucinous and non-mucinous bronchiolo-alveolar carcinoma with typical lepidic growth pattern of tumor cells and discrete septal thickening but no fibrosis, inflammation or local invasion. CONCLUSION: Bronchiolo-alveolar carcinoma is an alternative diagnosis in alveolar condensations associated with pulmonary nodules even in a patient with immunosupression. Early diagnosis allows effective treatment in some cases.

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INTRODUCTION: The syndrome of combined basal pulmonary fibrosis and apical emphysema (CPFE) is characterised by severe dyspnoea not fully explained by pulmonary function tests that show subnormal lung volumes and expiratory flows while CT imaging reveals varying degrees of emphysema and interstitial fibrosis. CASE REPORT: A man presented with chronic obstructive pulmonary disease associated with severe emphysema. Airflow obstruction was associated with early over-inflation. Five years later he developed interstitial fibrosis with CT appearances compatible with non-specific interstitial pneumonitis. Simultaneously, the decreased expiratory flows due to emphysema had become normal and the pulmonary distension had resolved. CONCLUSION: When CPFE develops, the reduced expiratory flows and thoracic distension due to emphysema can resolve. These changes may be explained by the late development of interstitial changes, which balance the functional consequences of emphysema by an increase in elastic recoil and reduction in volume. This is the first case report that describes the evolutionary profile in a single patient. It reinforces the pathophysiological hypothesis that explains the normal pulmonary volumes and expiratory flows in CPFE.

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