[Nurse practitioners as key actors in pulmonary rehabilitation for chronic obstructive pulmonary disease]. / L'infirmier en pratique avancée, un acteur-clé dans le développement de la réadaptation respiratoire chez le patient atteint de BPCO.

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.

[The role of cardiac magnetic resonance imaging in pulmonary arterial hypertension and chronic thrombo-embolic pulmonary hypertension]. / Place de l'IRM cardiaque dans l'hypertension artérielle pulmonaire et l'hypertension pulmonaire thrombo-embolique chronique.

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.

[Specific effect of exercise training on depression in COPD patients]. / Impact spécifique du réentraînement à l'exercice sur les symptômes de dépression dans la broncho-pneumopathie chronique obstructive : expérience du Réseau insuffisance respiratoire de Lorraine.

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.

[Familial pulmonary veno-occlusive disease with a composite biallelic heterozygous EIF2AK4 mutation]. / Une maladie veino-occlusive pulmonaire familiale avec mutation biallélique hétérozygote composite d'EIF2AK4.

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.

[The impact of exposure to cigarette smoke on the haemodynamic response during exercise of patients under specific treatment for pulmonary arterial hypertension]. / Impact de l'exposition à la fumée de cigarette sur la réponse hémodynamique à l'exercice de patients sous traitement spécifique de l'HTAP.

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.

Prevalence and management of chronic breathlessness in COPD in a tertiary care center.

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.

[A case of bronchiectasis due to light chain deposition disease]. / Histoire naturelle d'une forme bronchectasiante de la maladie à dépôts de chaînes légères.

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.

[Evaluation of cardiac MRI in the follow up assessment of patients with pulmonary arterial hypertension]. / Évaluation de l'IRM cardiaque dans le suivi des patients ayant une hypertension artérielle pulmonaire (EVITA). IRM cardiaque dans le suivi de l'hypertension artérielle pulmonaire.

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.

[Tunnelled central venous catheter infection during treatment with epoprostenol]. / Infection de cathéter veineux central tunnelisé survenue sous traitement par époprosténol.

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.

Occupational rhinitis and asthma to latex.

Occupational allergic rhinitis to proteins is increasing in importance. Two cases of latex-induced rhinitis are reported in a nurse's aid and a laboratory technician. A crossed food allergy is quoted. Positive prick-tests and specific IgE to latex have been demonstrated in both cases. A double-blind nasal challenge test has been performed with the rinse fluid from a brand of latex gloves. Clinical manifestations with endoscopic modifications of the nasal mucosa have been observed. A rise in nasal secretory eosinophilia has been demonstrated. The authors wish to draw attention to this new aetiology of occupational rhinitis insofar as it precedes the onset of work-related asthma as shown in one case. The eviction of wearing gloves is not sufficient as latex allergens are airborne. The eviction of ordinary latex gloves must be extended to all other workers in the same place and the use of powder-free gloves is advisable.