0000000000122384

AUTHOR

Philippe Bonniaud

Efficacité du dupilumab dans l’asthme sévère non contrôlé : étude multicentrique de vraie vie en France

Introduction De septembre 2017 a janvier 2018, l’ouverture d’une autorisation temporaire d’utilisation (ATU) a permis de traiter par dupilumab (anticorps monoclonal anti-IL4/13R) des patients asthmatiques severes non controles en impasse therapeutique. L’objectif de ce travail est d’evaluer l’effet du traitement sur le controle de l’asthme. Methodes Une etude retrospective multicentrique a ete conduite dans les 13 hopitaux francais ayant fait une demande d’ATU nominative pour du dupilumab. Les caracteristiques demographiques, cliniques et fonctionnelles des patients ont ete recueillies a l’inclusion et a 12 mois de traitement. Les Resultats sont exprimes en tant que medianes avec ecarts int…

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Optimising experimental research in respiratory diseases: an ERS statement

Experimental models are critical for the understanding of lung health and disease and are indispensable for drug development. However, the pathogenetic and clinical relevance of the models is often unclear. Further, the use of animals in biomedical research is controversial from an ethical perspective.The objective of this task force was to issue a statement with research recommendations about lung disease models by facilitating in-depth discussions between respiratory scientists, and to provide an overview of the literature on the available models. Focus was put on their specific benefits and limitations. This will result in more efficient use of resources and greater reduction in the numb…

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Strategies targeting fibrosis in pulmonary disease

Pulmonary fibrosis is potentially a curable disease, but this potential is dependent on correct identification of disease targets at the research stage. While invaluable work has been done in animal models to identify factors involved in the initiation of fibrogenesis, we must now shift our focus towards identifying factors involved in the chronic progression of fibrosis and how to modulate the progressive nature of the disease towards repair or regeneration of non-functioning pulmonary tissue.

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Organisation des structures de soins transversales en allergologie en France en 2020

Resume Introduction Les allergies touchent 25 % de la population generale avec une augmentation des allergies severes. Le developpement en France de structures de soins transversales en allergologie pour une prise en charge pluridisciplinaire des patients repond a un besoin de sante publique. Notre travail a pour but de faire un etat des lieux de ces structures. Methodes Enquete descriptive par questionnaire envoye aux chefs de services de structures de soins soit avec une activite principale en allergologie, soit avec une activite variee en allergologie. L’enquete portait sur l’organisation administrative, les equipes medicales et paramedicales, l’offre de soins et les moyens techniques de…

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Interstitial Lung Disease during ANCA-Associated Vasculitis: A Poor-Prognosis Factor

IF 7.873; International audience

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VEGF, une puissante molécule dans l’air du temps

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Le site de pathologie iatrogène pneumotox : données métriques sur les tableaux et médicaments, intéressant potentiellement l’allergologue

Introduction En aout 2018 a ete installee une fonctionnalite de mesure de l’activite sur Pneumotox evaluant le taux de consultation des differents medicaments, tableaux cliniques, ainsi que l’imagerie et l’histologie, de meme que les pays utilisateurs et villes d’origine des utilisateurs, en conformite avec la RGPD. Resultats Au 8 janvier 2019, sur 5 mois, 35 720 connexions ont ete recensees, 9740 (33,6 %) en provenance des Etats-Unis, 3760 de France, 1830 d’Allemagne, 1412 du Canada, 1340 du Bresil, 1320 des Pays-Bas, 1160 du Royaume-Uni, 1090 d’Espagne, 821 de Suisse, 611 d’Italie, 576 de Turquie et 430 de Belgique. Ces chiffres sont a relativiser en fonction de la population du pays, du …

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Atteinte trachéobronchique isolée de la polychondrite atrophiante : intérêt du TEP-scanner

Resume Introduction La polychondrite atrophiante est une maladie rare et grave caracterisee par une atteinte inflammatoire et multifocale des cartilages. Le diagnostic bien qu’urgent est difficile lorsque l’atteinte tracheobronchique est isolee. Observation Nous rapportons le cas d’une femme de 55 ans aux antecedents recents d’episodes febriles non infectieux accompagnes d’un syndrome inflammatoire biologique et corticosensibles. L’auscultation, les explorations fonctionnelles respiratoires et le scanner thoracique evoquaient la presence d’une tracheobronchomalacie. Une polychondrite atrophiante etait suspectee sans pouvoir etre confirmee sur le plan histologique et biologique, ce d’autant …

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The small heat-shock protein α B-crystallin is essential for the nuclear localization of Smad4: impact on pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) is a devastating disease characterized by the proliferation of myofibroblasts and the accumulation of extracellular matrix (ECM) in the lungs. TGF-β1 is the major profibrotic cytokine involved in IPF and is responsible for myofibroblast proliferation and differentiation and ECM synthesis. αB-crystallin is constitutively expressed in the lungs and is inducible by stress, acts as a chaperone and is known to play a role in cell cytoskeleton architecture homeostasis. The role of αB-crystallin in fibrogenesis remains unknown. The principal signalling pathway involved in this process is the Smad-dependent pathway. We demonstrate here that αB-crystallin is stron…

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Pulmonary hypertension associated with ponatinib therapy

IF 8.332; International audience; A case of pulmonary hypertension associated with ponatinib therapy in a chronic myelogenous leukaemia patient.

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Pneumotox : données métriques sur les médicaments, tableaux, et pays utilisateurs. À la recherche de zones silencieuses

Introduction En aout 2018 a ete installee une mesure des medicaments, tableaux cliniques, d’imagerie ou d’histologie ainsi que les pays utilisateurs et villes d’origine, apres accord de l’utilisateur en conformite avec la RGPD. Resultats Au 5 septembre 2018, soit sur 26 jours, 4320 connexions ont ete recensees, 1645 (38,1 %) des Etats-Unis, 450 de France, 272 d’Allemagne, 242 du Canada, 201 des Pays-Bas, 201 du Bresil, 178 du Royaume-Uni, 148 d’Espagne, 120 de Suisse, 72 de Belgique et 59 d’Italie. Chiffres a interpreter au regard d’un taux de rebond de 18 %, et en fonction de la population, du nombre de medecins du pays, et de la periode estivale (Chiffres seront actualises pour le CPLF 20…

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Pleuroparenchymal fibroelastosis as a late complication of chemotherapy agents

To the Editor: We identified six patients with clinical, radiographic and physiological features typical of pleuroparenchymal fibroelastosis (PPFE). In the six cases, PPFE may have been causally related to prior alkylating drugs used to treat malignacy, namely cyclophosphamide in five of the cases and carmustine (BCNU (1,3-bis-(2-chloroethyl)-1-nitrosourea)) in one. Based on an extensive review of the literature, we suspect that similar cases may have already been reported in the past 4 decades but have not been recognised either as PPFE or as drug-induced in nature. In 2004, Frankel et al. [1] described a then-new clinicopathologic entity, which they termed “idiopathic PPFE”. The authors i…

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Le pneumocytome sclérosant : une tumeur rare et apparemment bénigne

International audience; Introduction: Sclerosing pneumocytoma is a benign and rare lung tumor affecting epithelial cells. In most cases, patients are asymptomatic and the diagnosis is made on an X ray or a CT scan performed for other enquiry. Sex ratio favors women. Epidemiological studies report that middle-aged Asian women are more frequently affected. Radiological investigations find a solitary nodule or a mass with peripheric localization. When performed, histological analysis shows a tumor composed of at least two of the four following architectures: papillary, sclerosing, hemangiomatous and solid, with two types of cells that can be round or cubic cells.Cases report: We report two cas…

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Fibroblast Growth Facor 19, a Downregulated Factor in Idiopathic Pulmonary Fibrosis, Inhibits Mice Lung Fibrosis

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Heat shock protein: a hot topic in idiopathic pulmonary fibrosis

HSP90 inhibition could be an exciting new treatment strategy for IPF http://ow.ly/HfKY306uvxw

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Une cause rare d’opacités alvéolaires : maladie de Waldenström à localisation pulmonaire

Resume Introduction Les localisations pulmonaires de la maladie de Waldenstrom sont rares. Observation Nous rapportons le cas d’une femme de 71 ans presentant une toux chronique, associee a des opacites alveolaires disseminees persistantes apres plusieurs antibiotherapies. L’examen clinique etait normal. Le diagnostic de maladie de Waldenstrom etait suspecte devant un pic monoclonal de type IgM (immunoglobuline M) a l’electrophorese des proteines plasmatiques avec immunofixation et la presence d’une population B monoclonale a l’immunophenotypage du lavage broncho-alveolaire. Ce diagnostic etait confirme par le resultat de la biopsie osteomedullaire. La patiente etait traitee par chimiothera…

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Tonnerre de Brest ! Des Journées de Recherche Respiratoires 2020 pas comme les autres…

International audience; Tentons un peu d’oublier l’année 2020, mais pas complètement . . . C’est une année qui nousaura conduits à des envies et des visions différentes. Les rencontres conviviales et bien-veillantes qui ponctuent nos journées chaque année au mois d’octobre depuis 2005 n’ontpu se dérouler comme prévues, mais nous étions tous présents pour les 16esJournées deRecherche Respiratoire (J2R) le 16 octobre 2020, bien que devant nos écrans !Nous y avons cru jusqu’au bout et pensions pouvoir bénéficier de la fin de la fenêtreestivale, le programme était prêt et nos hôtes Brestois (nous ne remercierons jamais assezles équipes des Prs Francis Couturaud et Christophe Leroyer) avaient pe…

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Haemoptysis: a frequent diagnostic challenge

We appreciate and agree with the comments of M. Mondoni and colleagues, that the results of the ongoing prospective Italian multicentre trial ([www.ClinicalTrial.gov][1] identifier [NCT02045394][2]) will be of considerable interest for the management of haemoptysis, a frequent and severe symptom, especially since there is no clear consensus concerning aetiology and treatment. Interestingly, beyond determining the prevalence of diseases that may present with haemoptysis, their epidemiological results will be analysed according to the severity of the symptom. More importantly, their trial will hopefully make it possible to analyse the sensitivity and specificity of complementary tests, such a…

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Heat shock protein-90 toward theranostics: a breath of fresh air in idiopathic pulmonary fibrosis.

Heat shock proteins are potential biomarkers and therapeutic targets in idiopathic pulmonary fibrosis http://ow.ly/fHVP30hJUOl

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Le FGF-9 inhibe la fibrose pleurale induite par un adénovirus chez la souris

La fibrose pulmonaire idiopathique (FPI) est une pathologie incurable et mortelle. Elle debute et predomine dans les regions sous-pleurales. Elle serait la consequence d’une reactivation des voies impliquees dans le developpement pulmonaire et d’une anomalie de communication entre les differentes cellules pulmonaires. Le fibroblast growth factor-9 (FGF-9) est necessaire pour le developpement pulmonaire fœtal. Au cours de la FPI, le FGF-9 est reexprime par les cellules mesotheliales pleurales. L’objectif de ce travail est de determiner l’effet de la surexpression de FGF-9 par les cellules mesotheliales de la plevre au cours du processus fibrosant pulmonaire. Materiel et methodes De souris mâ…

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ARIA digital anamorphosis: Digital transformation of health and care in airway diseases from research to practice: Review

Submitted by (omml@ubi.pt) on 2021-07-05T10:37:14Z No. of bitstreams: 1 2021_Bousquet J_A_Anamorphosis.pdf: 1897974 bytes, checksum: 918eb581ab4e940b055ef8514f960e28 (MD5) Approved for entry into archive by Pessoa (pfep@ubi.pt) on 2021-07-05T11:16:44Z (GMT) No. of bitstreams: 1 2021_Bousquet J_A_Anamorphosis.pdf: 1897974 bytes, checksum: 918eb581ab4e940b055ef8514f960e28 (MD5) Approved for entry into archive by Pessoa (pfep@ubi.pt) on 2021-07-05T11:20:07Z (GMT) No. of bitstreams: 1 2021_Bousquet J_A_Anamorphosis.pdf: 1897974 bytes, checksum: 918eb581ab4e940b055ef8514f960e28 (MD5) Made available in DSpace on 2021-07-05T11:20:07Z (GMT). No. of bitstreams: 1 2021_Bousquet J_A_Anamorphosis.pdf: …

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Usefulness of transcutaneous PCO2to assess nocturnal hypoventilation in restrictive lung disorders

Background and objectiveNocturnal hypoventilation is now an accepted indication for the initiation of non-invasive ventilation. Nocturnal hypoventilation may be an under diagnosed condition in chronic respiratory failure. The most appropriate strategy to identify sleep hypoventilation is not yet clearly defined. In clinical practice, it is indirectly assessed using nocturnal pulse oximetry (NPO) and morning arterial blood gases (mABG). Even though continuous transcutaneous carbon dioxide partial pressure (TcPCO2) monitoring is theoretically superior to NPO plus mABG, it is not routinely used. We aimed to prospectively compare NPO plus mABG with nocturnal TcPCO2 for the detection of alveolar…

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Activité du conseil médical en environnement intérieur (CMEI) en Bourgogne : évaluation auprès des médecins et des patients

Resume Depuis decembre 2009, les pneumologues et allergologues bourguignons peuvent faire appel a un conseiller medical en environnement interieur (CMEI). Les interventions, gratuites pour le patient, sont realisees sur recommandation medicale apres tests cutanes systematiques. Objectifs Decrire les indications et la repartition des prescriptions et mesurer l’impact de la visite a six mois sur les 100 premiers patients et a 18 mois aupres des medecins. Methode Entretien telephonique des 67 medecins (prescripteurs ou non) sur leurs motivations et/ou leurs attentes et des 100 premiers patients sur le suivi des recommandations. Resultats Soixante-dix pour cent des medecins ont repondu ( n  = 4…

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HSP27 : une nouvelle cible pour traiter la fibrose pulmonaire idiopathique ?

Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal disease without therapeutic options. The development of new therapeutic strategies for the disease is needed. IPF is characterized by myofibroblast accumulation and collagen overproduction. Transforming growth factor-β1 (TGF-β1) is a key cytokine activating these pathological processes. Heat shock proteins (HSPs) are crucial regulators and promote TGF-β1 activity. Among them, HSP27 is overexpressed in animal models and in the lung of patients with IPF. HSP27 activates pro-fibrotic mechanisms and therefore may represents a potential target. Strategies aiming to inhibit HSP27 might pave the way towards new treatment options for pa…

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Onset of effect and impact on health-related quality of life, exacerbation rate, lung function, and nasal polyposis symptoms for patients with severe eosinophilic asthma treated with benralizumab (ANDHI): a randomised, controlled, phase 3b trial

Background: ANDHI was done to assess the efficacy of benralizumab, including onset of effect and impact on health-related quality of life (HRQOL), exacerbation rate, lung function, and nasal polyposis symptoms. Methods: This phase 3b, randomised, double-blind, parallel-group, placebo-controlled ANDHI study was completed in adults (aged 18–75 years) with severe eosinophilic asthma with at least 2 exacerbations in the previous year, despite high-dose inhaled corticosteroid plus additional controllers, screening blood eosinophil counts of at least 150 cells per μL, and an Asthma Control Questionnaire 6 (ACQ-6) score of 1·5 or more. Patients who met eligibility criteria were randomly assigned (…

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Dual regulation of SPI1/PU.1 transcription factor by heat shock factor 1 (HSF1) during macrophage differentiation of monocytes

International audience; : In addition to their cytoprotective role in stressful conditions, heat shock proteins (HSPs) are involved in specific differentiation pathways, e.g. we have identified a role for HSP90 in macrophage differentiation of human peripheral blood monocytes exposed to Macrophage Colony-Stimulating Factor (M-CSF). Here, we show that deletion of the main transcription factor involved in heat shock gene regulation, heat shock factor 1 (HSF1), affects M-CSF-driven differentiation of mouse bone marrow cells. HSF1 transiently accumulates in the nucleus of human monocytes undergoing macrophage differentiation, including M-CSF-treated peripheral blood monocytes and phorbol ester-…

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Heat Shock Proteins: Cell Protection through Protein Triage

Heat shock proteins (HSPs) are chaperones that catalyze the proper folding of nascent proteins and the refolding of denatured proteins. The ubiquitin-proteasome system is an error-checking system that directs improperly folded proteins for destruction. A coordinated interaction between the HSPs (renaturation) and the proteasome (degradation) must exist to assure protein quality control mechanisms. Although it still remains unknown how the decision of folding vs. degradation is taken, many pieces of evidence demonstrate that HSPs interact directly or indirectly with the proteasome, assuring quite selectively the proteasomal degradation of certain proteins under stress conditions. In this rev…

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Outcomes in Patients Receiving Nintedanib or Pirfenidone for Idiopathic Pulmonary Fibrosis

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094 Transition épithélio-mésenchymateuse (TEM) lors de la fibrose pleurale induite par Transforming Growth Factor (TGF)-1

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017 Fibrose pleurale progressive induite par le transfert du gène de Transforming Growth Factor (TGF)-β1 à la plèvre

Introduction De nombreux agents ou toxines (medicaments, radiation, amiante, maladies infectieuses) sont responsables de fibrose pleurale, pathologie invalidante entrainant un syndrome restrictif parfois severe. Les mecanismes conduisant a cette fibrose pleurale ont ete jusqu’ici peu explores. TGF-β est un facteur cle de l’initiation et de la progression de la fibrose dans de nombreux organes. Le transfert transitoire du gene de TGF-β1 actif (AdTGF-β1) aux poumons de rat, a l’aide d’adenovirus induit une fibrose pulmonaire severe et progressive ( J Clin Invest 1997; 100: 768-76). Nous avons developpe un modele de fibrose pleurale par transfert du gene de TGF-β1 au mesothelium pleural afin d…

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Interstitial lung disease induced by drugs and radiation.

An ever-increasing number of drugs can reproduce variegated patterns of naturally occurring interstitial lung disease (ILD), including most forms of interstitial pneumonias, alveolar involvement and, rarely, vasculitis. Drugs in one therapeutic class may collectively produce the same pattern of involvement. A few drugs can produce more than one pattern of ILD. The diagnosis of drug-induced ILD (DI-ILD) essentially rests on the temporal association between exposure to the drug and the development of pulmonary infiltrates. The histopathological features of DI-ILD are generally consistent, rather than suggestive or specific to the drug etiology. Thus, the diagnosis of DI-ILD is mainly made by …

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La fumée de cannabis a des effets toxiques immédiats sur les cellules épithéliales bronchiques in vivo

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Haemoptysis in adults: a 5-year study using the French nationwide hospital administrative database.

Haemoptysis is a serious symptom with various aetiologies. Our aim was to define the aetiologies, outcomes and associations with lung cancer in the entire population of a high-income country.This retrospective multicentre study was based on the French nationwide hospital medical information database collected over 5 years (2008–2012). We analysed haemoptysis incidence, aetiologies, geographical and seasonal distribution and mortality. We studied recurrence, association with lung cancer and mortality in a 3-year follow-up analysis.Each year, ∼15 000 adult patients (mean age 62 years, male/female ratio 2/1) were admitted for haemoptysis or had haemoptysis as a complication of their hospital s…

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Progressive pulmonary fibrosis is mediated by TGF-β isoform 1 but not TGF-β3

Tissue repair is a well-orchestrated biological process involving numerous soluble mediators, and an imbalance between these factors may result in impaired repair and fibrosis. Transforming growth factor (TGF)-beta is a key profibrotic element in this process and it is thought that its three isoforms act in a similar way. Here, we report that TGF-beta3 administered to rat lungs using transient overexpression initiates profibrotic effects similar to those elicited by TGF-beta1, but causes less severe and progressive changes. The data suggest that TGF-beta3 does not lead to inhibition of matrix degradation in the same way as TGF-beta1, resulting in non-fibrotic tissue repair. Further, TGF-bet…

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Updated guidelines (2015) for management and monitoring of adult and adolescent asthmatic patients (from 12 years and older) of the Societe de Pneumologie de Langue Francaise (SPLF) (Full length text)

Revue des Maladies Respiratoires - In Press.Proof corrected by the author Available online since samedi 16 avril 2016

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Heat shock proteins in fibrosis and wound healing: Good or evil?

Heat shock proteins (HSPs) are key regulators of cell homeostasis, and their cytoprotective role has been largely investigated in the last few decades. However, an increasing amount of evidence highlights their deleterious effects on several human pathologies, including cancer, in which they promote tumor cell survival, proliferation and drug resistance. Therefore, HSPs have recently been suggested as therapeutic targets for improving human disease outcomes. Fibrotic diseases and cancer share several properties; both pathologies are characterized by genetic alterations, uncontrolled cell proliferation, altered cell interactions and communication and tissue invasion. The discovery of new HSP…

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FGF-9 overexpression prevents pleural fibrosis induced by intra-pleural adenovirus injection in mice

Introduction: Lung fibrosis is associated with the reactivation of molecular pathways involved in lung development. Mesothelial cells are involved in the fibrotic lung process but their exact role is debated. Fibroblast Growth Factor-9 (FGF-9) is expressed by epithelial cells and visceral pleura during embryonic lung development. Mice homozygous for a targeted disruption of FGF-9 exhibit lung hypoplasia with reduced mesenchyme and early postnatal death. The aim of this study was to evaluate the role of FGF-9 expression by mesothelial cells in the adult lung. We used adenovirus-mediated (FGF-9) gene transfer in mesothelial cells in vivo. Material and Methods: AdFGF9 or a control adenovirus (…

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Identification of periplakin as a major regulator of lung injury and repair in mice

IF 12.784 (2016); International audience; Periplakin is a component of the desmosomes that acts as a cytolinker between intermediate filament scaffolding and the desmosomal plaque. Periplakin is strongly expressed by epithelial cells in the lung and is a target antigen for autoimmunity in idiopathic pulmonary fibrosis. The aim of this study was to determine the role of periplakin during lung injury and remodeling in a mouse model of lung fibrosis induced by bleomycin. We found that periplakin expression was downregulated in the whole lung and in alveolar epithelial cells following bleomycin-induced injury. Deletion of the Ppl gene in mice improved survival and reduced lung fibrosis developm…

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Inhibition of HSP27 blocks fibrosis development and EMT features by promoting Snail degradation

Idiopathic pulmonary fibrosis (IPF) is a devastating disease characterized by myofibroblast proliferation. Transition of epithelial/mesothelial cells into myofibroblasts [epithelial-to-mesenchymal transition (EMT)] occurs under the influence of transforming growth factor (TGF)-β1, with Snail being a major transcription factor. We study here the role of the heat-shock protein HSP27 in fibrogenesis and EMT. In vitro, we have up- and down-modulated HSP27 expression in mesothelial and epithelial cell lines and studied the expression of different EMT markers induced by TGF-β1. In vivo, we inhibited HSP27 with the antisense oligonucleotide OGX-427 (in phase II clinical trials as anticancer agent)…

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Halo Sign and Eosinophilia: What Is Your Diagnosis?

Blood eosinophilia associated with transient and migrating nodules with a halo sign on chest computed tomography scan should suggest larva migrans related to toxocariasis or <i>ascaris suum</i>.

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Respiratory manifestations of eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

The respiratory manifestations of eosinophilic granulomatosis with polyangiitis (EGPA) have not been studied in detail.In this retrospective multicentre study, EGPA was defined by asthma, eosinophilia and at least one new onset extra-bronchopulmonary organ manifestation of disease.The study population included 157 patients (mean±sd age 49.4±14.1 years), with a mean±sd blood eosinophil count of 7.4±6.4×109 L−1 at diagnosis. There was a mean±sd of 11.8±18.2 years from the onset of asthma to the diagnosis of EGPA, of 1.4±8.4 years from the first onset of peripheral eosinophilia to the diagnosis of EGPA, and of 7.4±6.4 years from EGPA diagnosis to the final visit. Despite inhaled and oral corti…

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[Choosing a quality-of-life questionnaire].

International audience; Define the objective of the questionnaire: Discrimination: do you want a questionnaire to enable you to describe the quality of life of patients or to compare the quality of life between groups of patients, for example, to determine who has improved and who has gotten worse? Assessment: do you want a questionnaire to help you measure changes over time (improvement or aggravation) in your patients? Determine the properties of instruments necessary for this objective: If the objective is discrimination, analyze: construct validity, reliability. If the objective is assessment, analyze construct validity, sensitivity to changes. Choose the general category of questionnai…

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Drug-induced and iatrogenic infiltrative lung disease.

At present more than 350 drugs are known to cause injury of the lung parenchyma,upper and lower airways, pulmonary circulation, pleura, mediastinum, lymph nodes,and neuromuscular system. Infiltrative lung disease (ILD) is the most common pattern of drug-induced injury. This article, which is clinically oriented rather than drug oriented, reviews the patterns of ILD produced by therapeutic drugs and radiation therapy.

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Transfer of the active form of transforming growth factor-beta 1 gene to newborn rat lung induces changes consistent with bronchopulmonary dysplasia.

Bronchopulmonary dysplasia is a chronic lung disease of premature human infancy that shows pathological features comprising varying sized areas of interstitial fibrosis in association with distorted large alveolar spaces. We have previously shown that transfer of active transforming growth factor (TGF)-beta 1 (AdTGF beta 1(223/225)) genes by adenovirus vector to embryonic lungs results in inhibition of branching morphogenesis and primitive peripheral lung development, whereas transfer to adult lungs results in progressive interstitial fibrosis. Herein we show that transfer of TGF-beta1 to newborn rat pups results in patchy areas of interstitial fibrosis developing throughout a period of 28 …

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Extracellular Heat Shock Proteins as Therapeutic Targets and Biomarkers in Fibrosing Interstitial Lung Diseases.

Interstitial lung diseases (ILDs) include a large number of diseases and causes with variable outcomes often associated with progressive fibrosis. Although each of the individual fibrosing ILDs are rare, collectively, they affect a considerable number of patients, representing a significant burden of disease. Idiopathic pulmonary fibrosis (IPF) is the typical chronic fibrosing ILD associated with progressive decline in lung. Other fibrosing ILDs are often associated with connective tissues diseases, including rheumatoid arthritis-ILD (RA-ILD) and systemic sclerosis-associated ILD (SSc-ILD), or environmental/drug exposure. Given the vast number of progressive fibrosing ILDs and the dispariti…

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Daptomycin-induced eosinophilic pneumonia: Are there any risk factors?

Abstract Objectives Daptomycin is a widely used antibiotic. Rhabdomyolysis related to daptomycin is one of the adverse effects of treatment, justifying the need for regular monitoring of muscle enzymes throughout treatment. Daptomycin may also lead to eosinophilic pneumonia. However, risk factors for this adverse reaction have not been identified and do not permit targeting of at-risk populations who could benefit from appropriate monitoring. Patients and methods Literature was reviewed for cases of daptomycin-induced eosinophilic pneumonia (DIEP), which that were compared to cases of patients without this adverse effect. Results Fifty patients with DIEP and 54 controls were identified. Age…

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Pneumopathies non infectieuses induites par les biothérapies ciblées

Resume Les biotherapies des rhumatismes inflammatoires chroniques ont revolutionne leur prise en charge. Ces nouveaux medicaments sont susceptibles d’induire des effets secondaires multiples, parfois severes et ceux-ci peuvent atteindre l’appareil respiratoire. Les presentations cliniques et radiologiques sont multiples et l’apparition de symptomes respiratoires chez un patient sous biotherapie doit faire suspecter un effet iatrogenique avec au premier plan la survenue de pneumopathies interstitielles diffuses parfois tres severes, de granumolatoses et d’anomalies auto-immunes. La complication infectieuse doit toujours etre eliminee. La demarche est complexe et doit etre rigoureuse ce d’aut…

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Le FGF-9 inhibe la fibrose pleurale viro-induite chez la souris

La fibrose pulmonaire idiopathique (FPI) est une pathologie mortelle, predominante en zone sous-pleurale. Elle serait en partie due a une reactivation des voies impliquees dans le developpement pulmonaire. Le Fibroblast Growth Factor-9 (FGF-9) est reexprime par les cellules mesotheliales lors de la FPI. Nous avons determine l’effet de la surexpression de FGF-9 par les cellules mesotheliales chez la souris. Des souris C56/Bl6 ont recu une injection intrapleurale unique de serum physiologique (groupe Phy) ou un adenovirus recombine exprimant le FGF-9 humain (groupe AdFGF-9) ou un adenovirus controle (groupe Adcont), a la concentration de 1 × 108 pfu/mL. Les souris ont ete sacrifiees a j3, j5,…

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