Grandière L, Gille T, Brillet PY
… +7 more, Didier M, Freynet O, Vicaire H, Clero D, Martinod E, Mathian A, Uzunhan Y
Rev Mal Respir
· 2024 Jun · PMID 38762394
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Relapsing polychondritis is a systemic auto-immune disease that mainly affects cartilage structures, progressing through inflammatory flare-ups between phases of remission and ultimately leading to deformation of the car...Relapsing polychondritis is a systemic auto-immune disease that mainly affects cartilage structures, progressing through inflammatory flare-ups between phases of remission and ultimately leading to deformation of the cartilages involved. In addition to characteristic damage of auricular or nasal cartilage, tracheobronchial and cardiac involvement are particularly severe, and can seriously alter the prognosis. Tracheobronchial lesions are assessed by means of a multimodal approach, including dynamic thoracic imaging, measurement of pulmonary function (with recent emphasis on pulse oscillometry), and mapping of tracheal lesions through flexible bronchoscopy. Diagnosis can be difficult in the absence of specific diagnostic tools, especially as there may exist a large number of differential diagnoses, particularly as regards inflammatory diseases. The prognosis has improved, due largely to upgraded interventional bronchoscopy techniques and the development of immunosuppressant drugs and targeted therapies, offering patients a number of treatment options.
Alexandre F, Molinier V, Poulain M
… +7 more, Villiot-Danger E, Eichenauer B, Calvat A, Brandon L, Miffre C, Oliver N, Heraud N
Rev Mal Respir
· 2024 Jun · PMID 38762393
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INTRODUCTION: Over recent years, a growing number of studies have demonstrated the effectiveness of alternative models to centre-based pulmonary rehabilitation (PR) such as tele-PR or home-based unsupervised PR, offering...INTRODUCTION: Over recent years, a growing number of studies have demonstrated the effectiveness of alternative models to centre-based pulmonary rehabilitation (PR) such as tele-PR or home-based unsupervised PR, offering perspectives for improved accessibility and adherence. Other studies have demonstrated the relevance and long-term benefits of maintenance PR programs. However, they remain poorly implemented in real-life settings. In order to encourage patient adherence to new PR models and to guide future orientations, we conducted a survey assessing patients' views on PR models and maintenance programs. METHOD: The survey (37 questions) was circulated to COPD patients of the French national respiratory patient F.F.A.A.I.R network and in five specialised PR centres. RESULTS: Among the 298 respondents, 75% had previously taken part in a PR program, mainly in hospital settings (91%), with a high degree of satisfaction. The main barriers to PR were being physically separated from their loved ones (21%) and fears of having to share a double room (47%). Regarding maintenance PR programs, patients expressed diversified opinions, in terms of ideal duration and frequency of follow-up, format of follow-up (home-based, telephone, videoconference) and type of professional involved. CONCLUSIONS: Diversified PR settings offer perspectives to increase access and improve the effectiveness of current programs. Furthermore, comprehensive personalization (professionals involved, content, setting, duration) seems to be the key to success in concrete implementation and achievement of patient satisfaction.
Deflandre LA, Weber T, Ote M
… +1 more, Bourgeois P
Rev Mal Respir
· 2024 Jun · PMID 38760314
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INTRODUCTION: Cystic lung diseases are rare, with numerous differential diagnoses. Iconographic discovery consequently necessitates medical examinations in view of proposing an etiological orientation. CASE REPORT: A 57-...INTRODUCTION: Cystic lung diseases are rare, with numerous differential diagnoses. Iconographic discovery consequently necessitates medical examinations in view of proposing an etiological orientation. CASE REPORT: A 57-year-old woman consulted in pulmonology following fortuitous detection of a cystic lung disease on an abdominal CT scan. Complementary medical examinations did not allow orientation towards a particular diagnosis. During a follow-up consultation, the patient informed her pulmonologist of the recent detection of a monoallelic variant of a FAT4 gene in one of her daughters, who was suffering from edema of the lower limbs secondary to a disease of the lymphatic system. As our patient had a similar history, she likewise received a genetic analysis. A monoallelic variant not described in the genetic databases was observed, and considered as a probable pathogenic variant (class 4/5 on the pathogenicity scale of genetic variants). CONCLUSION: After analyzing the available literature data, we raise questions about a possible link between this variant of the FAT4 gene, chronic lymphedema and our patient's cystic lung disease.
Dargentolle G, Georges M, Beltramo G
… +2 more, Poisson C, Bonniaud P
Rev Mal Respir
· 2024 May · PMID 38653607
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INTRODUCTION: Asthma is a pathology that remains severe and is inadequately controlled in 4% of patients. Identification of multiple pathophysiological mechanisms has led to the development of biomedicines, of which ther...INTRODUCTION: Asthma is a pathology that remains severe and is inadequately controlled in 4% of patients. Identification of multiple pathophysiological mechanisms has led to the development of biomedicines, of which there are currently five available in France, with a safety profile that appears favorable but remains uncertain due to a lack of real-life experience with these new molecules. STATE OF KNOWLEDGE: Although relatively benign, the adverse effects of biologics are diverse. Headache, joint pain, skin reactions at the injection site, fever and asthenia are commonly observed during the different treatments. Ophthalmological complications seem restricted to dupilumab, with numerous cases of keratitis and conjunctivitis in patients with atopic dermatitis. Several respiratory complications have also been observed, essentially consisting in pharyngitis and other upper respiratory infections. Hypereosinophilia may occur, mainly with dupilumab, requiring investigation of systemic repercussions or vasculitis. Allergic reactions are uncommon but require careful monitoring during initial injections. CONCLUSION: Biologics for severe asthma are recent drugs with a favorable safety profile, but with little real-life experience, justifying increased vigilance by prescribing physicians.
Ancel J, Chen E, Pavot A
… +10 more, Regard L, Le Rouzic O, Guecamburu M, Zysman M, Rapin A, Martin C, Soumagne T, Patout M, Roche N, Deslee G
Rev Mal Respir
· 2024 May · PMID 38609767
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INTRODUCTION: The second COPD Biennial organized by the COPD working group of the French Society of Respiratory Diseases took place in Paris (Cochin) on 13th December 2023. STATE OF THE ART: Major trends in 2023 were dis...INTRODUCTION: The second COPD Biennial organized by the COPD working group of the French Society of Respiratory Diseases took place in Paris (Cochin) on 13th December 2023. STATE OF THE ART: Major trends in 2023 were discussed; they encompassed concepts, definitions, biologics, care pathways, pulmonary rehabilitation and complex situations entailed by respiratory infections, cardiovascular comorbidities and pulmonary hypertension, and modalities of oxygen therapy and ventilation. PERSPECTIVES: The different talks underlined major changes in COPD including the concepts of pre-COPD, etiotypes, health trajectories and new definitions of exacerbation. Recent results in biologics for COPD open the door to new pharmacological options. Assessment of current care pathways in France highlighted some causes for concern. For example, pulmonary rehabilitation is a key but insufficiently practiced element. Respiratory infections require careful assessment and treatments. Diagnosis and treatment of cardiovascular comorbidities and pulmonary hypertension are of paramount importance. As of late, oxygen therapy and ventilation modalities have evolved, and are beginning to afford more personalized options. CONCLUSIONS: As regards COPD, a personalized approach is crucial, placing the patient at the center of the care pathway and facilitating coordination between healthcare providers.
Mayaux J, Decavele M, Dres M
… +2 more, Lecronier M, Demoule A
Rev Mal Respir
· 2024 May · PMID 38609766
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Acute respiratory failure (ARF) is a leading cause, along with sepsis, of admission to the intensive care unit (ICU) of patients with active cancer. Presenting variable clinical severity, ARF in onco-hematological patien...Acute respiratory failure (ARF) is a leading cause, along with sepsis, of admission to the intensive care unit (ICU) of patients with active cancer. Presenting variable clinical severity, ARF in onco-hematological patients has differing etiologies, primarily represented by possibly opportunistic acute infectious pneumonia (de novo hypoxemic ARF), and decompensation in chronic cardiac or respiratory diseases (e.g., acute pulmonary edema or exacerbated chronic obstructive pulmonary disease). In these patients, orotracheal intubation is associated with a doubled risk of in-hospital mortality. Consequently, over the last three decades, numerous researchers have attempted to demonstrate and pinpoint the precise role of non-invasive ventilation (NIV) in the specific context of ARF in onco-hematological patients. While the benefits of NIV in the management of acute pulmonary edema or alveolar hypoventilation (hypercapnic ARF) are well-demonstrated, its positioning in de novo hypoxemic ARF is debatable, and has recently been called into question. In the early 2000s, based on randomized controlled trials, NIV was recommended as first-line treatment, one reason being that it allowed significantly reduced use of orotracheal intubation. In the latest randomized studies, however, the benefits of NIV in terms of survival orotracheal intubation have not been observed; as a result, it is no longer recommended in the management of de novo hypoxemic ARF in onco-haematological patients.
Rev Mal Respir
· 2024 May · PMID 38594123
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INTRODUCTION: Pesticides are used worldwide, mainly in agriculture as a means of controlling pests and protecting crops. That said, the entire world population is ultimately subject to pesticide exposure (consumption of...INTRODUCTION: Pesticides are used worldwide, mainly in agriculture as a means of controlling pests and protecting crops. That said, the entire world population is ultimately subject to pesticide exposure (consumption of fruits and vegetables, living near treated fields…), with varying degrees of toxicity involved. STATE OF THE ART: In recent decades, epidemiological studies have contributed to the identification of chemical pesticide families with detrimental effects on human health: cognitive disorders, Parkinson's disease, prostate cancer… and impairment in respiratory functioning. Current scientific evidence points to the implication of the active substances in insecticides, herbicides and fungicides in chronic respiratory diseases, two examples being chronic obstructive pulmonary disease (COPD) in exposed workers, and asthmatic wheezing in children during prenatal or postnatal exposure. PERSPECTIVES: The safety of individuals exposed to pesticides is of key importance in public health. Further epidemiological investigations are needed to identify the chemical families affecting certain populations. CONCLUSIONS: The scientific literature suggests strong links between pesticide exposure and respiratory health. Whether it be environmental or occupational, pesticide exposure can lead to respiratory disorders and symptoms of varying severity.
Lachkar S, Guisier F, Dantoing E
… +2 more, Thiberville L, Salaün M
Rev Mal Respir
· 2024 May · PMID 38580585
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The management of peripheral lung nodules is challenging, requiring specialized skills and sophisticated technologies. The diagnosis now appears accessible to advanced endoscopy (see Part 1), which can also guide treatme...The management of peripheral lung nodules is challenging, requiring specialized skills and sophisticated technologies. The diagnosis now appears accessible to advanced endoscopy (see Part 1), which can also guide treatment of these nodules; this second part provides an overview of endoscopy techniques that can enhance surgical treatment through preoperative marking, and stereotactic radiotherapy treatment through fiduciary marker placement. Finally, we will discuss how, in the near future, these advanced endoscopic techniques will help to implement ablation strategy.
Chronic obstructive pulmonary disease (COPD) is a chronic respiratory disease characterized by a non-reversible limitation of expiratory airflow. In patients with COPD, distal airways are the major site of obstruction; e...Chronic obstructive pulmonary disease (COPD) is a chronic respiratory disease characterized by a non-reversible limitation of expiratory airflow. In patients with COPD, distal airways are the major site of obstruction; early in the course of the disease, they show signs of being remodeled, inflamed, and/or obliterated. Recent technological advances, particularly in imaging and transcriptomics, have provided new information on this key area of the lung. The objective of this review is to provide an updated overall vision of knowledge on distal airways and how they are damaged in COPD.
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive and fatal lung disease of which the origin and development mechanisms remain unknown. The few available pharmacological treatments can only slow the progressi...Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive and fatal lung disease of which the origin and development mechanisms remain unknown. The few available pharmacological treatments can only slow the progression of the disease. The development of curative treatments is hampered by the absence of experimental models that can mimic the specific pathophysiological mechanisms of IPF. The aim of this mini-review is to provide an overview of the most commonly used experimental animal models in the study of IPF and to underline the urgent need to seek out new, more satisfactory models.
Down syndrome (DS), or trisomy 21, is a genetic disorder caused by the presence of an extra copy of chromosome 21, leading to various characteristic physical features as well as developmental and cognitive delays. Obstru...Down syndrome (DS), or trisomy 21, is a genetic disorder caused by the presence of an extra copy of chromosome 21, leading to various characteristic physical features as well as developmental and cognitive delays. Obstructive sleep apnea syndrome (OSAS) is a common disorder in both adult and pediatric patients with DS. Several characteristics of DS may contribute to the development or worsening of OSAS. Numerous murine models of DS exist. A number of studies have explored apneas and the risk of upper airway obstruction in these models, but up until now, only in adulthood.
Lipofibroblasts form a sub-population of fibroblasts located in the mesenchymal alveolar stem cell niche. They show close proximity with alveolar epithelial type 2 cells and play a key role in alveolar development and lu...Lipofibroblasts form a sub-population of fibroblasts located in the mesenchymal alveolar stem cell niche. They show close proximity with alveolar epithelial type 2 cells and play a key role in alveolar development and lung homeostasis. Their role in various diseases such as acute respiratory distress syndrome, pulmonary fibrosis and emphysema is progressively better understood. Through the activation of signaling pathways such as PPARg lipofibroblasts may help to induce endogenous alveolar regeneration.
Ahmed E, Assou S, Foisset F
… +10 more, Bourdais C, Vanheerswynghels M, Petit A, Gamez AS, Gras D, Chanez P, de Vos J, Hammad H, Bourdin A, Lambrecht B
Severe asthma patients with persistent airflow obstruction are characterized by functional obstruction due to mucus plugs containing mucins, fibrin, and eosinophil derived Charcot- Leyden crystals. The molecular mechanis...Severe asthma patients with persistent airflow obstruction are characterized by functional obstruction due to mucus plugs containing mucins, fibrin, and eosinophil derived Charcot- Leyden crystals. The molecular mechanisms underlying this endotype are not clearly understood. Developing new models is crucial to respiratory research insofar as critical differences exist between human and rodent airway epithelium. We (and other teams) have shown that it is possible to reconstitute in vitro a complex and functional airway epithelium displaying all the features described in vivo from human-induced pluripotent stem cells (hiPSC). Our aim is to establish a human in vitro model of severe asthma that will recapitulate airway epithelium remodeling and mucus plugs.
Primary thoracic cancers affect a large number of patients, mainly those with lung cancer and to a lesser extent those with pleural mesothelioma and thymic tumours. Given their frequency and associated comorbidities, in...Primary thoracic cancers affect a large number of patients, mainly those with lung cancer and to a lesser extent those with pleural mesothelioma and thymic tumours. Given their frequency and associated comorbidities, in patients whose mean age is high, these diseases are associated with multiple complications. This article, the last of a series dedicated to emergencies in onco-haematological patients, aims to present a clinical picture of the severe complications (side effects, immune-related adverse events) associated with systemic treatments, excluding infections and respiratory emergencies, with which general practitioners and specialists can be confronted. New toxicities are to be expected with the implementation of innovative therapeutic approaches, such as CAR-T cells, along with immunomodulators and antibody-drug conjugates.
Lung cancer is the first cancer-related cause of death worldwide. This is in partially due to therapeutic resistance, which occurs in around 70% of patients, especially those receiving platinum salts, the gold-standard c...Lung cancer is the first cancer-related cause of death worldwide. This is in partially due to therapeutic resistance, which occurs in around 70% of patients, especially those receiving platinum salts, the gold-standard chemotherapy. The massive deregulation of alternative transcript splicing processes observed in many cancers has led to the development of a new class of pharmacological agents aimed at inhibiting the activity of the splicing machinery (spliceosome). The molecular mechanisms by which these inhibitors act remain largely unknown, as do the benefits of using them in combination with other therapies. In this context, our work is focused on an inhibitor of the SRPK1 kinase, a major regulator of the spliceosome.
Pulmonary hypertension (PH) is the main pathology in lung circulation, characterized by increased pressure in pulmonary arteries and ultimately resulting in right heart failure with potentially fatal outcomes. Given the...Pulmonary hypertension (PH) is the main pathology in lung circulation, characterized by increased pressure in pulmonary arteries and ultimately resulting in right heart failure with potentially fatal outcomes. Given the current lack of available curative treatments, it is of paramount importance to identify novel therapeutic targets. Due to its involvement in pulmonary arterial remodeling, hyperreactivity, and inflammation, our explorations have focused on the nerve growth factor (NGF), offering promising avenues for innovative therapeutic approaches.