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151. Emerging ideas about sarcoidosis pathophysiology

Author: Daniel A. Culver and Dominique Valeyre
Subjects: 030203 arthritis & rheumatology, Pulmonary and Respiratory Medicine, 03 medical and health sciences, medicine.medical_specialty, 0302 clinical medicine, 030228 respiratory system, business.industry, medicine, Sarcoidosis, Intensive care medicine, medicine.disease, business, Pathophysiology
Published: 2016

152. Sarcoidosis extra-pulmonary manifestations

Author: Hilario Nunes, Marc A. Judson, Florence Jeny, Matthieu Mahévas, and Dominique Valeyre
Subjects: medicine.medical_specialty, business.industry, medicine, Sarcoidosis, business, Extra pulmonary, medicine.disease, Dermatology
Published: 2017

153. Management and long-term outcomes of sarcoidosis-associated pulmonary hypertension

Author: Laurent Savale, Abdelatif Tazi, David Montani, Jason Weatherald, Olivier Sitbon, Vincent Cottin, Marc Humbert, Gérald Simonneau, Christophe Pison, Julie Traclet, Emmanuel Bergot, Dominique Valeyre, Xavier Jaïs, Martine Reynaud-Gaubert, Hilario Nunes, Catherine Viacroze, Athénaïs Boucly, Delphine Horeau-Langlard, Grégoire Prévot, Claire Dromer, Université Paris-Sud - Paris 11 - Faculté de médecine (UP11 UFR Médecine), Université Paris-Sud - Paris 11 (UP11), Department of Pneumology [Lyon], Hospices Civils de Lyon (HCL), Service des maladies respiratoires, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Groupe Hospitalier Avicenne-Jean Verdier-René Muret, Hypertension arterielle pulmonaire physiopathologie et innovation thérapeutique, Centre chirurgical Marie Lannelongue-Institut National de la Santé et de la Recherche Médicale (INSERM), Hôpital Saint-Louis, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7), Service de pneumologie [Toulouse], CHU Toulouse [Toulouse]-Hôpital Larrey [Toulouse], CHU Toulouse [Toulouse], Microbes évolution phylogénie et infections (MEPHI), Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS), Service de chirurgie thoracique, CHU Bordeaux [Bordeaux]-Hôpital Haut-Lévêque [CHU Bordeaux], CHU Bordeaux [Bordeaux], CHU Rouen, Normandie Université (NU), Centre hospitalier universitaire de Nantes (CHU Nantes), Laboratory of Fundamental and Applied Bioenergetics = Laboratoire de bioénergétique fondamentale et appliquée (LBFA), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Grenoble Alpes [2016-2019] (UGA [2016-2019]), Centre Hospitalier Universitaire [Grenoble] (CHU), CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN), Centre de Référence des Maladies Pulmonaires Rares [Hôpital Louis Pradel - HCL], Hôpital Louis Pradel [CHU - HCL], Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Hôpital Bicêtre, Hôpital Bicêtre-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris-Sud - Paris 11 (UP11), Service de pneumologie [Béclère], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-AP-HP - Hôpital Antoine Béclère [Clamart], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Université Paris-Sud 11 - Faculté de médecine (UP11 UFR Médecine), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Groupe Hospitalier Avicenne-Jean Verdier-René Muret, Université Paris Diderot - Paris 7 (UPD7)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), CHU Toulouse [Toulouse]-Hôpital Larrey, Laboratoire de bioénergétique fondamentale et appliquée (LBFA), Université Grenoble Alpes (UGA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Joseph Fourier - Grenoble 1 (UJF), Université Paris-Sud - Paris 11 (UP11)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Bicêtre, Université Paris-Sud - Paris 11 (UP11)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Antoine Béclère, Centre Chirurgical Marie Lannelongue (CCML)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), and Université Paris-Sud - Paris 11 (UP11)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Bicêtre
Subjects: Male, Pulmonary and Respiratory Medicine, medicine.medical_specialty, Sarcoidosis, Hypertension, Pulmonary, [SDV]Life Sciences [q-bio], Cardiac index, Hemodynamics, 030204 cardiovascular system & hematology, Time, 03 medical and health sciences, 0302 clinical medicine, medicine.artery, Internal medicine, medicine, Humans, Disease management (health), Lung, Aged, business.industry, Patient Acuity, Disease Management, Middle Aged, medicine.disease, Pulmonary hypertension, Respiratory Function Tests, 3. Good health, Radiography, Outcome and Process Assessment, Health Care, medicine.anatomical_structure, 030228 respiratory system, Pulmonary artery, Vascular resistance, Cardiology, Female, Vascular Resistance, France, business, Immunosuppressive Agents
Abstract: Studies reporting the effects of modern strategies with pulmonary arterial hypertension (PAH)-targeted therapies in sarcoidosis-associated pulmonary hypertension (S-APH) are limited.Clinical and haemodynamic data from newly diagnosed patients with severe S-APH (mean pulmonary artery pressure (mPAP) >35 mmHg or mPAP 25–35 mmHg with cardiac index −1·m−2) were collected from the French Pulmonary Hypertension Registry between 2004 and 2015.Data from 126 patients with severe S-APH were analysed (mean±sd age 57.5±11.6 years, 74% radiological stage IV). 97 patients (77%) received PAH-targeted therapy and immunosuppressive therapy was initiated or escalated in 33 patients at the time of pulmonary hypertension diagnosis. Four months after PAH-targeted therapy initiation, mean±sd pulmonary vascular resistance decreased from 9.7±4.4 to 6.9±3.0 Wood units (pPAH-targeted therapy improved short-term pulmonary haemodynamics in severe S-APH without change in exercise capacity. Immunosuppressive therapy improved haemodynamics in selected patients. Pulmonary hypertension in sarcoidosis remains associated with a poor prognosis.
Published: 2017

154. Transbronchial cryobiopsies for the diagnosis of diffuse parenchymal lung diseases: expert statement from the Cryobiopsy Working Group on safety and utility and a call for standardization of the procedure

Author: Simon L.F. Walsh, Claudia Ravaglia, Martin Hetzel, Venerino Poletti, Jürgen Hetzel, Pallav L. Shah, Thomas V. Colby, Lonny Yarmus, Jouke T. Annema, Marco Chilosi, Ganesh Krishna, Lars Hagmeyer, Nicola Sverzellati, Oren Fruchter, Jay H. Ryu, Sara Tomassetti, Kaid Darwiche, Sara Piciucchi, Dominique Valeyre, Athol U. Wells, Fabien Maldonado, Alessandra Dubini, Stefano Gasparini, Alfonso Torrego, Alberto Cavazza, Maik Haentschel, Felix J.F. Herth, Dimitri Leduc, Ralf Eberhardt, CCA - Imaging and biomarkers, and Pulmonology
Subjects: Pulmonary and Respiratory Medicine, Parenchymal lung disease, medicine.medical_specialty, Standardization, Statement (logic), Respiratory System, Medizin, Interstitial lung disease, Lung biopsy, Credentialing, SUPRAGLOTTIC AIRWAY DEVICE, Cryosurgery, 1102 Cardiovascular Medicine And Haematology, ACUTE EXACERBATIONS, 03 medical and health sciences, 0302 clinical medicine, Bronchoscopy, Transbronchial lung cryobiopsy, USUAL INTERSTITIAL PNEUMONIA, medicine, SINGLE INSTITUTION, Humans, Diffuse parenchymal lung disease, Safety, 030212 general & internal medicine, Intensive care medicine, Lung, Pulmonologists, COMPLICATIONS, Science & Technology, medicine.diagnostic_test, business.industry, medicine.disease, EFFICACY, YIELD, 030228 respiratory system, BIOPSY, EXPERIENCE, IDIOPATHIC PULMONARY-FIBROSIS, Lung Diseases, Interstitial, business, Life Sciences & Biomedicine
Abstract: Transbronchial cryobiopsies (TBCB) have recently been introduced as a promising and safer alternative to surgical lung biopsy in the diagnostic approach to diffuse parenchymal lung diseases (DPLD). Despite a substantial and expanding body of literature, the technique has not yet been standardized and its place in the diagnostic algorithm of DPLD remains to be defined. In part, this reflects concerns over the diagnostic yield and safety of the procedure, together with the rapid spread of the technique without competency and safety standards; furthermore, there is a substantial procedural variability among centers and interventional pulmonologists. We report this expert statement proposed during the third international conference on “Transbronchial Cryobiopsy in Diffuse Parenchymal Lung Disease” (Ravenna, October 27–28, 2016), which formulates evidence- and expert-based suggestions on the indications, contraindications, patient selection, and procedural aspects of the procedure. The following 5 domains were reviewed: (1) what is the role of TBCB in the diagnostic evaluation of DPLD: patient selection; (2) pathological considerations; (3) contraindications and safety considerations; (4) how should TBCB be performed and in what procedural environment; and (5) who should perform TBCB. Finally, the existence of white paper recommendations may also reassure local hospital credentialing committees tasked with endorsing an adoption of the technique.
Published: 2017

155. The Lung in Dysregulated States of Humoral Immunity

Author: Jean-François Bernaudin, Dominique Valeyre, Diane Bouvry, Yurdagul Uzunhan, Morgane Didier, Hilario Nunes, Marianne Kambouchner, and Florence Jeny
Subjects: Pulmonary and Respiratory Medicine, Lung Diseases, Pathology, medicine.medical_specialty, Lymphoproliferative disorders, Light chain deposition disease, 03 medical and health sciences, 0302 clinical medicine, medicine, Humans, Lung, Bronchiectasis, business.industry, Common variable immunodeficiency, Amyloidosis, Respiratory disease, medicine.disease, Immunity, Humoral, Pneumonia, medicine.anatomical_structure, Common Variable Immunodeficiency, 030228 respiratory system, 030220 oncology & carcinogenesis, Immunology, business
Abstract: In common variable immunodeficiency, lung manifestations are related to different mechanisms: recurrent pneumonias due to encapsulated bacteria responsible for diffuse bronchiectasis, diffuse infiltrative pneumonia with various patterns, and lymphomas, mostly B cell extranodal non-Hodgkin type. The diagnosis relies on significant serum Ig deficiency and the exclusion of any primary or secondary cause. Histopathology may be needed. Immunoglobulin (IgG) replacement is crucial to prevent infections and bronchiectasis. IgG4-related respiratory disease, often associated with extrapulmonary localizations, presents with solitary nodules or masses, diffuse interstitial lung diseases, bronchiolitis, lymphadenopathy, and pleural or pericardial involvement. Diagnosis relies on international criteria including serum IgG4 dosage and significantly increased IgG4/IgG plasma cells ratio in pathologically suggestive biopsy. Respiratory amyloidosis presents with tracheobronchial, nodular, and cystic or diffuse interstitial lung infiltration. Usually of AL (amyloid light chain) subtype, it may be localized or systemic, primary or secondary to a lymphoproliferative process. Very rare other diseases due to nonamyloid IgG deposits are described. Among the various lung manifestations of dysregulated states of humoral immunity, this article covers only those associated with the common variable immunodeficiency, IgG4-related disease, amyloidosis, and pulmonary light-chain deposition disease. Autoimmune connective-vascular tissue diseases or lymphoproliferative disorders are addressed in other chapters of this issue.
Published: 2017

156. Recommandations pratiques pour le diagnostic et la prise en charge de la fibrose pulmonaire idiopathique. Élaborées par le centre national de référence et les centres de compétence pour les maladies pulmonaires rares sous l’égide de la Société de pneumologie de langue française [French practical guidelines for the diagnosis and management of idiopathic pulmonary fibrosis. 2017 update. Full-length update]

Author: Emmanuel Bergot, Gilbert Ferretti, D. Bonnet, Sylvain Marchand-Adam, Jacques Cadranel, Philippe Carré, Dominique Israel-Biet, Claire Dromer, P. Terrioux, Dominique Valeyre, Charles-Hugo Marquette, Françoise Thivolet-Béjui, Vincent Cottin, G. Prévot, Bruno Crestani, J.-C. Dalphin, Jean-François Cordier, Martine Reynaud-Gaubert, Ronald C. Kessler, Claire Danel, Emmanuel Gomez, B. Trumbic, François Lebargy, Jean-Baptiste Faivre, Philippe Camus, Bernard Aguilaniu, B. Philippe, Stéphane Jouneau, N. Just, Benoit Wallaert, Cadieu, Muriel, Service de Pneumologie, Centre de Référence National des Maladies Pulmonaires Rares, Hospices Civils de Lyon (HCL), Centre de compétences pour les maladies pulmonaires rares, Laboratoire Chrono-environnement (UMR 6249) (LCE), Centre National de la Recherche Scientifique (CNRS)-Université de Franche-Comté (UFC), Université Bourgogne Franche-Comté [COMUE] (UBFC)-Université Bourgogne Franche-Comté [COMUE] (UBFC), Institut de recherche en santé, environnement et travail (Irset), Université d'Angers (UA)-Université de Rennes (UR)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Université d'Angers (UA)-Université de Rennes 1 (UR1), and Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique )
Subjects: Pulmonary and Respiratory Medicine, 03 medical and health sciences, 0302 clinical medicine, 030228 respiratory system, [SDV.MHEP.PSR] Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract, 030212 general & internal medicine, [SDV.MHEP.PSR]Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract, 3. Good health
Abstract: National audience; La fibrose pulmonaire idiopathique (FPI) est la forme la plus fréquente de pneumopathie interstitielle diffuse (PID) idiopathique chronique chez l’adulte. Il s’agit d’une maladie fibroproliférative, irréversible, de cause inconnue, dont l’évolution est habituellement progressive, survenant principalement à partir de 60 ans et limitée aux poumons. Qualifiée de maladie orpheline notamment en raison de l’absence de traitement ayant fait la preuve formelle de son efficacité jusqu’à une période très récente, la FPI est une maladie rare dont la prévalence a été évaluée aux États-Unis entre 14 et 28/100 000 personnes [1], ce qui correspondrait à un minimum de 9000 patients en France et une incidence entre 6,8 et 9/100 000 par an [1,2], soit un minimum de 4400 nouveaux patients par an en France.
Published: 2017

157. Predictive factors of idiopathic pulmonary fibrosis (IPF) in patients with a possible or inconsistent with usual interstitial pneumonia (UIP) pattern on high resolution computed tomography (HRCT)

Author: Olivia Freynet, Dominique Valeyre, Morgane Didier, Cécile Rotenberg, Florence Jeny, Lucile Sesé, Dean Kassam, Pierre-Yves Brillet, Marianne Kambouchner, Hilario Nunes, and Diane Pivert
Subjects: medicine.medical_specialty, High-resolution computed tomography, medicine.diagnostic_test, business.industry, Context (language use), Retrospective cohort study, respiratory system, medicine.disease, respiratory tract diseases, FEV1/FVC ratio, Idiopathic pulmonary fibrosis, Usual interstitial pneumonia, Internal medicine, Cohort, medicine, business, Idiopathic interstitial pneumonia
Abstract: Introduction: In patients with chronic fibrosing idiopathic interstitial pneumonias (IIPs) and a possible or inconsistent with UIP pattern on HRCT, surgical lung biopsy (SLB) is required to make a definitive diagnosis between IPF and nonspecific interstitial pneumonia (NSIP) after multidisciplinary discussion (MDD). The aim of the study was to determine the predictive factors of IPF in this context. Methods: This monocentric retrospective study was based on a cohort of 289 IIPs patients. We included cases with a possible or inconsistent with UIP pattern on HRCT and a final diagnosis of IPF or NSIP after SLB and MDD. A multivariate logistic regression was used to analyse the predictive factors of IPF diagnosis. Patients with unclassifiable IIPs, mainly due to the absence of SLB, served as a validation cohort. Results: Among patients with a possible or inconsistent with UIP pattern (IPF:n=31, PINS:n=36), age>65 years was the best threshold indicative of IPF by a ROC curve. After adjustment for tobacco and sex, age>65 years (OR=3.35, p=0.046) and FVC decline>5% at 6 months (OR=3.46, p=0.047) were the strongest independent predictors of IPF. The combination of age>65 years + FVC decline>5% had a specificity of 94% and positive predictive value of 75%. In patients with unclassifiable IIPs (n=101), those with age>65 years + FVC decline>5% had a worse survival compared to others (median survival: 3.1 vs 8.8 years, p Conclusion: The combination of age and functional decline may help differentiate IPF and NSIP and assist clinicians in therapeutic decisions for patients who cannot undergo SLB.
Published: 2017

158. Late Breaking Abstract - Pharmacokinetics (PK) of nintedanib with add-on pirfenidone in patients with idiopathic pulmonary fibrosis (IPF): results from INJOURNEY

Author: Wibke Stansen, Christopher J. Ryerson, Michael Kreuter, Carlo Vancheri, Luca Richeldi, Wim A. Wuyts, Dominique Valeyre, Sabrina Wiebe, Manuel Quaresma, Jan C. Grutters, and Susanne Stowasser
Subjects: medicine.medical_specialty, business.industry, Pirfenidone 267 MG, High variability, Pirfenidone, medicine.disease, Gastroenterology, chemistry.chemical_compound, Idiopathic pulmonary fibrosis, chemistry, Pharmacokinetics, Internal medicine, medicine, Combination group, Nintedanib, In patient, business, medicine.drug
Abstract: Introduction: In a small phase II trial in Japanese patients with IPF, there was a trend towards lower nintedanib exposure when nintedanib 150 mg bid was added to pirfenidone than when given alone Aim: To explore the PK of nintedanib with add-on pirfenidone. Methods: After a 4–5 week run-in with nintedanib 150 mg bid, patients with IPF were randomised to open-label nintedanib 150 mg bid with add-on pirfenidone or nintedanib 150 mg bid alone for 12 weeks. In the combination group, patients received pirfenidone 267 mg tid from randomisation to week 1, 534 mg tid from week 1 to week 2, 801 mg tid from week 2. Pre-dose plasma trough concentrations were measured at baseline (for nintedanib) and at weeks 2 and 4 (for nintedanib and pirfenidone). Results: Pre-dose pirfenidone gMean (gCV%) concentrations were 1120 (122) and 1220 (91) ng/mL at weeks 2 and 4, respectively. Pre-dose plasma trough concentrations of nintedanib were similar at each time point, irrespective of whether nintedanib 150 mg bid was administered alone or with add-on pirfenidone 534 mg or 801 mg tid (table). Moderate to high variability was observed in both groups. Conclusion: In patients with IPF, nintedanib plasma trough concentrations were similar when nintedanib was administered alone or with add-on pirfenidone; however, data from a dedicated drug–drug interaction study are needed to draw robust conclusions.
Published: 2017

159. Prevalence and clinical characteristics of unclassifiable idiopathic interstitial pneumonias (IIPs)

Author: Lucile Sesé, Diane Piver, Olivia Freynet, Marianne Kambouchner, Hilario Nunes, Pierre Yves Brillet, Aurélie Hervé, Dominique Valeyre, and Cécile Rotenberg
Subjects: medicine.medical_specialty, business.industry, medicine, medicine.disease, business, Dermatology, Idiopathic interstitial pneumonia
Published: 2017

160. Obstructive sleep apnoea and related comorbidities in incident idiopathic pulmonary fibrosis

Author: Loris Moya, Danielle Sadoun-Danino, Frédéric Gagnadoux, Thomas Gille, Dominique Israel-Biet, Marie-Pia d'Ortho, Carole Planès, Morgane Didier, Marouane Boubaya, Fanny Baran-Marszak, Hilario Nunes, Vincent Cottin, Angela Sutton, Zohra Carton, Pierre-Yves Brillet, Bruno Crestani, Dominique Valeyre, Hypoxie et Poumon : pneumopathologies fibrosantes, modulations ventilatoires et circulatoires (H&P), Université Paris 13 (UP13)-UFR SMBH, Unité de recherche clinique [Avicenne], Hôpital Avicenne [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Hémostase, bio-ingénierie et remodelage cardiovasculaires (LBPC), Université Paris Diderot - Paris 7 (UPD7)-Université Paris 13 (UP13)-Université Sorbonne Paris Cité (USPC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut Galilée, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Service de Pneumologie, Paris, Department of Pneumology [Lyon], Hospices Civils de Lyon (HCL), Stress Oxydant et Pathologies Métaboliques (SOPAM), Université d'Angers (UA)-Institut National de la Santé et de la Recherche Médicale (INSERM), Physiopathologie et Epidemiologie de l'Insuffisance Respiratoire, Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM), Physiopathologie et Thérapeutiques Respiratoires, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Henri Mondor-Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR10, Réponses Cellulaires et Fonctionnelles à l'Hypoxie (LRPH), Université Paris 13 (UP13)-Université Sorbonne Paris Cité (USPC)-UFR SMBH, and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Avicenne [AP-HP]
Subjects: Male, Pathology, [SDV]Life Sciences [q-bio], Disease, Polysomnography, Comorbidity, Idiopathic pulmonary fibrosis, 0302 clinical medicine, Prevalence, 030212 general & internal medicine, Prospective Studies, Prospective cohort study, ComputingMilieux_MISCELLANEOUS, Sleep Apnea, Obstructive, medicine.diagnostic_test, [SDV.BA]Life Sciences [q-bio]/Animal biology, Sleep apnea, Calcinosis, Middle Aged, [SDV.SP]Life Sciences [q-bio]/Pharmaceutical sciences, Coronary Vessels, 3. Good health, medicine.anatomical_structure, 8-Hydroxy-2'-Deoxyguanosine, Cardiovascular Diseases, Cardiology, Female, France, Artery, Pulmonary and Respiratory Medicine, Adult, Risk, medicine.medical_specialty, macromolecular substances, [SDV.BC]Life Sciences [q-bio]/Cellular Biology, 03 medical and health sciences, stomatognathic system, Internal medicine, medicine, Humans, Medical history, Aged, business.industry, Deoxyguanosine, medicine.disease, Idiopathic Pulmonary Fibrosis, nervous system diseases, respiratory tract diseases, Oxidative Stress, 030228 respiratory system, business, Tomography, X-Ray Computed, [SDV.AEN]Life Sciences [q-bio]/Food and Nutrition, Biomarkers
Abstract: The objectives of this prospective study were: 1) to determine the prevalence and determinants of obstructive sleep apnoea (OSA) in patients with newly diagnosed idiopathic pulmonary fibrosis (IPF); 2) to determine whether OSA was associated with cardiovascular disease (CVD) as well as increased oxidative stress and levels of IPF biomarkers in the blood.A group of 45 patients with newly diagnosed IPF attended polysomnography. The prevalence of CVD and the severity of coronary artery calcification were investigated by high-resolution computed tomography. The levels of 8-hydroxydeoxyguanosine (8-OH-DG) and various IPF biomarkers in the blood were compared between patients with no or mild OSA (apnoea–hypopnoea index (AHI) −1), with moderate OSA (15 ≤AHI −1) and with severe OSA (AHI ≥30 events·h−1).The prevalence of moderate-to-severe OSA and severe OSA was 62% and 40%, respectively. AHI did not correlate with demographic or physiological data. All patients with severe OSA had a medical history of CVD,versus41.2% and 40% of those with no or mild OSA, or with moderate OSA, respectively (pModerate-to-severe OSA is highly prevalent in incident IPF and severe OSA is strongly associated with the presence of CVD, particularly IHD.
Published: 2017

161. AB0007 Shared genetic predisposition in rheumatoid arthritis–interstitial lung disease and familial pulmonary fibrosis

Author: Serge Amselem, Marie-Christophe Boissier, Catherine Boileau, Benoit Wallaert, Aurélien Justet, Gabriel Thabut, Baptiste Coustet, A Clément, Bruno Crestani, Martin Soubrier, Yannick Allanore, Vincent Cottin, S. Ottaviani, Nadia Nathan, Christelle Ménard, Olivier Sand, Steven Gazal, Hilario Nunes, Lidwine Wemeau-Stervinou, H. Lioté, Isabelle Callebaut, Philippe Dieudé, Pierre-Antoine Juge, Marie-Pierre Debray, P. Richette, R.M. Flipo, Patrick Revy, Dominique Valeyre, Dlm Florence, S. Marchand-Adam, J. Sibilia, T. Schaeverbeke, Aline Frazier, Christophe Richez, Caroline Kannengiesser, Raphael Borie, Claire Dromer, and N. Saidenberg
Subjects: 0301 basic medicine, Oncology, medicine.medical_specialty, Pathology, Mutation, business.industry, Interstitial lung disease, Odds ratio, respiratory system, medicine.disease, medicine.disease_cause, respiratory tract diseases, Pathogenesis, 03 medical and health sciences, 030104 developmental biology, 0302 clinical medicine, Usual interstitial pneumonia, 030220 oncology & carcinogenesis, Rheumatoid arthritis, Internal medicine, Pulmonary fibrosis, medicine, Genetic predisposition, business
Abstract: Background Despite its high prevalence and mortality, little is known about the pathogenesis of RA–associated interstitial lung disease (RA-ILD). Given that familial pulmonary fibrosis (FPF) and RA–ILD frequently share the usual interstitial pneumonia pattern and common environmental risk factors, we hypothesized that the two diseases may share additional risk factors including FPF-linked genes. Objectives Our aim was to identify coding mutations of FPF-risk genes associated with RA-ILD. Methods We used whole-exome sequencing (WES) followed by restricted analysis of a discrete number of FPF-linked genes and performed a Burden test to assess the excess number of mutations in RA–ILD patients compared to controls. Results Among the 101 RA–ILD patients included, 12 (11.9%) had 13 WESidentified heterozygous mutations in the TERT, RTEL1, PARN or SFTPC coding regions. The burden test, based on 81 RA–ILD patients and 1010 controls of European ancestry, revealed an excess of TERT, RTEL1, PARN or SFTPC mutations for RA–ILD patients (p=9.45’10-4, odds ratio [OR] 3.17 95% CI 1.53 – 6.12). Telomeres were shorter for RA-ILD patients with a TERT, RTEL1 or PARN mutation than controls (p=2.87x10-2). Conclusions Our results support the contribution of FPF-linked genes to RA–ILD susceptibility. Disclosure of Interest None declared
Published: 2017

162. Efficacy and Tolerance of Anti-Tumor Necrosis Factor α Agents in Cutaneous Sarcoidosis: A French Study of 46 Cases

Author: Alicia Marquet, Olivier Chosidow, Laurence Bouillet, Didier Bessis, Pascal Joly, Françoise Sarrot-Reynauld, Matthieu Mahévas, Emmanuel Delaporte, Marc Ruivard, Jean-Benoît Monfort, Anne-Bénédicte Duval-Modeste, Olivier Fain, Nicolas Noel, Thierry Passeron, Saskia Ingen-Housz-Oro, Catherine Chapelon-Abric, Diane Bouvry, Sylvain Marchand-Adam, Laurence Fardet, Sébastien Debarbieux, Denis Verrot, Valentine Heidelberger, Pascal Sève, Frédéric Caux, and Dominique Valeyre
Subjects: Adult, Male, medicine.medical_specialty, Adolescent, Databases, Factual, Sarcoidosis, Cutaneous Sarcoidosis, Dermatology, Skin Diseases, 030207 dermatology & venereal diseases, 03 medical and health sciences, Young Adult, 0302 clinical medicine, Recurrence, Internal medicine, Epidemiology, Medicine, Humans, Adverse effect, Aged, 030203 arthritis & rheumatology, business.industry, Tumor Necrosis Factor-alpha, Lupus pernio, Correction, Middle Aged, medicine.disease, Infliximab, 3. Good health, Surgery, Discontinuation, Treatment Outcome, Concomitant, Female, Dermatologic Agents, France, business, Immunosuppressive Agents, medicine.drug, Follow-Up Studies
Abstract: Importance Evidence for the long-term efficacy and safety of anti–tumor necrosis factor α agents (anti-TNF) in treating cutaneous sarcoidosis is lacking. Objective To determine the efficacy and safety of anti-TNF in treating cutaneous sarcoidosis in a large observational study. Design, Setting, and Participants STAT (Sarcoidosis Treated with Anti-TNF) is a French retrospective and prospective multicenter observational database that receives data from teaching hospitals and referral centers, as well as several pneumology, dermatology, and internal medicine departments. Included patients had histologically proven sarcoidosis and received anti-TNF between January 2004 and January 2016. We extracted data for patients with skin involvement at anti-TNF initiation. Main Outcomes and Measures Response to treatment was evaluated for skin and visceral involvement using the ePOST (extra-pulmonary Physician Organ Severity Tool) severity score (from 0 [not affected] to 6 [very severe involvement]). Epidemiological and cutaneous features at baseline, efficacy, steroid-sparing, safety, and relapses were recorded. The overall cutaneous response rate (OCRR) was defined as complete (final cutaneous ePOST score of 0 or 1) or partial response (ePOST drop ≥2 points from baseline but >1 at last follow-up). Results Among 140 patients in the STAT database, 46 had skin involvement. The most frequent lesions were lupus pernio (n = 21 [46%]) and nodules (n = 20 [43%]). The median cutaneous severity score was 5 and/or 6 at baseline. Twenty-one patients were treated for skin involvement and 25 patients for visceral involvement. Reasons for initiating anti-TNF were failure or adverse effects of previous therapy in 42 patients (93%). Most patients received infliximab (n = 40 [87%]), with systemic steroids in 28 cases (61%) and immunosuppressants in 32 cases (69.5%). The median (range) follow-up was 45 (3-103) months. Of the 46 patients with sarcoidosis and skin involvement who were treated with anti-TNF were included, median (range) age was 50 (14-78) years, and 33 patients (72%) were women. The OCRR was 24% after 3 months, 46% after 6 months, and 79% after 12 months. Steroid sparing was significant. Treatment was discontinued because of adverse events in 11 patients (24%), and 21 infectious events occurred in 14 patients (30%). Infections were more frequent in patients treated for visceral involvement than in those treated for skin involvement (n = 12 of 25 [48%] vs n = 2 of 21 [9.5%], respectively; P = .02). The relapse rate was 44% 18 months after discontinuation of treatment. Relapses during treatment occurred in 35% of cases, mostly during anti-TNF or concomitant treatment tapering. Conclusions and Relevance Anti-TNF agents are effective but suspensive in cutaneous sarcoidosis. The risk of infectious events must be considered.
Published: 2017

163. Inborn Errors of Metabolism: The Achilles' Heel of the Respiratory System

Author: Nadia Nathan, Jean-François Bernaudin, Dominique Valeyre, Hôpital Avicenne [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Université Sorbonne Paris Cité (USPC), Hypoxie et Poumon : pneumopathologies fibrosantes, modulations ventilatoires et circulatoires (H&P), UFR SMBH-Université Sorbonne Paris Nord, Service de Pneumologie pédiatrique [CHU Trousseau], CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Centre de référence national pour les maladies respiratoires rares de l’enfant RespiRare [CHU Trousseau], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-CHU Trousseau [APHP], Physiopathologie des maladies génétiques d'expression pédiatrique, Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Université Pierre et Marie Curie - Paris 6 - UFR de Médecine Pierre et Marie Curie (UPMC), Université Pierre et Marie Curie - Paris 6 (UPMC), Couvet, Sandrine, and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)
Subjects: Pulmonary and Respiratory Medicine, Heel, business.industry, Respiratory System, 030204 cardiovascular system & hematology, [SDV.MHEP.PSR]Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract, 03 medical and health sciences, 0302 clinical medicine, medicine.anatomical_structure, 030228 respiratory system, Anesthesia, medicine, [SDV.MHEP.PSR] Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract, Humans, Respiratory system, business, Metabolism, Inborn Errors
Abstract: International audience
Published: 2017

164. In situ analysis by SEM-EDX spectroscopy of 10 sarcoidosis cases from MINASARC study

Author: Catherine Cavalin, Nathalie Freymond, Olivia Freynet, Michel Vincent, C. Chemarin, Paul-André Rosental, F. Arbib, Ana-Maria Sfarghiu, Marianne Kambouchner, Christophe Pison, Jean-François Mornex, Mickaël Catinon, Françoise Thivolet, Jean-François Bernaudin, Dominique Valeyre, Laboratoire d'Ecologie Alpine (LECA ), Université Savoie Mont Blanc (USMB [Université de Savoie] [Université de Chambéry])-Centre National de la Recherche Scientifique (CNRS)-Université Grenoble Alpes [2016-2019] (UGA [2016-2019]), Service d’Anatomie et de Cytologie Pathologiques [Louis Pradel - CHU Lyon], Hôpital Louis Pradel [CHU - HCL], Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Service d’anatomie pathologique, Hôpital Avicenne [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Physiopathologie des maladies génétiques d'expression pédiatrique (UMRS_933), Sorbonne Université (SU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Institut de Recherche Interdisciplinaire en Sciences Sociales (IRISSO), Institut National de la Recherche Agronomique (INRA)-Université Paris Dauphine-PSL, Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL), Laboratoire de Mécanique des Contacts et des Structures [Villeurbanne] (LaMCoS), Institut National des Sciences Appliquées de Lyon (INSA Lyon), Institut National des Sciences Appliquées (INSA)-Université de Lyon-Institut National des Sciences Appliquées (INSA)-Université de Lyon-Centre National de la Recherche Scientifique (CNRS), Clinique de pneumologie, CHU Grenoble, Physiopathologie et Epidémiologie des Maladies Respiratoires (PHERE (UMR_S_1152 / U1152)), Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre Hospitalier Lyon Sud [CHU - HCL] (CHLS), Hospices Civils de Lyon (HCL), Rétrovirus et Pathologie Comparée (RPC), Institut National de la Recherche Agronomique (INRA)-École pratique des hautes études (EPHE), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Ecole Nationale Vétérinaire de Lyon (ENVL), Études sociales et politiques des populations de la protection sociale et de la santé, Centre d'études européennes et de politique comparée (CEE), Sciences Po (Sciences Po)-Centre National de la Recherche Scientifique (CNRS)-Sciences Po (Sciences Po)-Centre National de la Recherche Scientifique (CNRS), Centre de Mise en Forme des Matériaux (CEMEF), MINES ParisTech - École nationale supérieure des mines de Paris, Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Centre National de la Recherche Scientifique (CNRS), Service des maladies respiratoires, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Groupe Hospitalier Avicenne-Jean Verdier-René Muret, Laboratory of Fundamental and Applied Bioenergetics = Laboratoire de bioénergétique fondamentale et appliquée (LBFA), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Grenoble Alpes [2016-2019] (UGA [2016-2019]), Laboratoire d'Ecologie Alpine (LECA), Centre National de la Recherche Scientifique (CNRS)-Université Savoie Mont Blanc (USMB [Université de Savoie] [Université de Chambéry])-Université Joseph Fourier - Grenoble 1 (UJF)-Université Grenoble Alpes (UGA), Hôpital avicenne, Université Paris 13 (UP13)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Avicenne-Université Paris 13 (UP13)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Avicenne, Physiopathologie des maladies génétiques d'expression pédiatrique, Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE)-Université Paris Dauphine-PSL, Université de Lyon-Institut National des Sciences Appliquées (INSA)-Université de Lyon-Institut National des Sciences Appliquées (INSA)-Centre National de la Recherche Scientifique (CNRS), Institut National de la Recherche Agronomique (INRA)-École pratique des hautes études (EPHE)-Université Claude Bernard Lyon 1 (UCBL), Centre National de la Recherche Scientifique (CNRS)-Sciences Po (Sciences Po)-Centre National de la Recherche Scientifique (CNRS)-Sciences Po (Sciences Po), Centre National de la Recherche Scientifique (CNRS)-PSL Research University (PSL)-MINES ParisTech - École nationale supérieure des mines de Paris, Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Groupe Hospitalier Avicenne-Jean Verdier-René Muret, Laboratoire de bioénergétique fondamentale et appliquée (LBFA), Université Joseph Fourier - Grenoble 1 (UJF)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Grenoble Alpes (UGA), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Université Paris 13 (UP13)-Hôpital Avicenne-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Université Paris 13 (UP13)-Hôpital Avicenne, Université Paris Dauphine-PSL-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE), and Université Grenoble Alpes (UGA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Joseph Fourier - Grenoble 1 (UJF)
Subjects: medicine.diagnostic_test, Scanning electron microscope, business.industry, Energy-dispersive X-ray spectroscopy, inorganic exposome, [SDV.CAN]Life Sciences [q-bio]/Cancer, medicine.disease, [SPI]Engineering Sciences [physics], Tissue sections, medicine.anatomical_structure, In situ analysis, Biopsy, medicine, Sarcoidosis, Lymph, sarcoidosis, Nuclear medicine, business, MINARSAC, Lymph node, ComputingMilieux_MISCELLANEOUS
Abstract: Introduction: MINASARC is a case-control study designed to assess the inorganic “exposome” by a specific questionnaire concomitantly with the mineralogical analysis (MA) of Broncho-Alveolar Lavage (BAL) by transmission electron microscopy (MA-TEM) and paraffin embedded biopsy by scanning electron microscopy (MA-SEM). We report the results obtained by MA-SEM from 10 sarcoidosis cases. Objectives: To compare the results of MA-SEM and MA-TEM. Methods: 5 mediastinal lymph nodes obtained by endobronchial ultrasound trans-bronchial needle aspiration (EBUS/TBNA) and 5 bronchial biopsies (BB) were studied by SEM-EDX. Results: * + to ++++: level of particles load Discussion and conclusion: MA-SEM on tissue sections is feasible and may give potential interesting results. In one manicurist (n°4), it identified silica particles in the lymph node and a high level in BAL. In a fire safety worker (n°10), it identified steel particles associated to a steel overload in the BAL. However, we have to be cautious for interpretation of EBUS/TBNA collected samples, as we identified FeCrNi and TiNi particles compatible with metal particles release from needles used for TBNA, as already reported [Gounant et al., Chest 2010]. MA-SEM on paraffin embedded biopsies allows to determine the chemical composition of particles observed in the preserved structures of tissues. It offers additional information on the patient9s exposure to inorganic dusts.
Published: 2017

165. Role of atmospheric pollution on the natural history of idiopathic pulmonary fibrosis

Author: Annelyse Nardi, Dominique Valeyre, Violaine Giraud, Marie Wislez, Karine Juvin, Abdellatif Tazi, Isabella Annesi-Maesano, Morgane Didier, Sandra Dury, Shreosi Sanyal, Lucile Sesé, Raphael Borie, Bruno Crestani, Sylvain Marchand-Adam, Stephanie Guillot-Dudoret, Vincent Cottin, Zohra Carton, Anne Gondouin, Hilario Nunes, Grégoire Prévot, Bernard Maitre, Benoit Wallaert, Dominique Israel-Biet, and Jacques Cadranel
Subjects: Pulmonary and Respiratory Medicine, Male, medicine.medical_specialty, Pathology, Time Factors, Nitrogen Dioxide, Air pollution, medicine.disease_cause, 03 medical and health sciences, Idiopathic pulmonary fibrosis, chemistry.chemical_compound, 0302 clinical medicine, Ozone, Risk Factors, Internal medicine, Air Pollution, Medicine, Humans, Nitrogen dioxide, 030212 general & internal medicine, Longitudinal Studies, Prospective cohort study, Aged, Proportional Hazards Models, Air Pollutants, business.industry, Proportional hazards model, Environmental exposure, Environmental Exposure, Middle Aged, medicine.disease, Idiopathic Pulmonary Fibrosis, Natural history, 030228 respiratory system, chemistry, Cohort, Female, Particulate Matter, France, business
Abstract: IntroductionIdiopathic pulmonary fibrosis (IPF) has an unpredictable course corresponding to various profiles: stability, physiological disease progression and rapid decline. A minority of patients experience acute exacerbations (AEs). A recent study suggested that ozone and nitrogen dioxide might contribute to the occurrence of AE. We hypothesised that outdoor air pollution might influence the natural history of IPF.MethodsPatients were selected from the French cohort COhorte FIbrose (COFI), a national multicentre longitudinal prospective cohort of IPF (n=192). Air pollutant levels were assigned to each patient from the air quality monitoring station closest to the patient’s geocoded residence. Cox proportional hazards model was used to evaluate the impact of air pollution on AE, disease progression and death.ResultsOnset of AEs was significantly associated with an increased mean level of ozone in the six preceding weeks, with an HR of 1.47 (95% CI 1.13 to 1.92) per 10 µg/m3 (p=0.005). Cumulative levels of exposure to particulate matter PM10 and PM2.5 were above WHO recommendations in 34% and 100% of patients, respectively. Mortality was significantly associated with increased levels of exposure to PM10 (HR=2.01, 95% CI 1.07 to 3.77) per 10 µg/m3 (p=0.03), and PM2.5 (HR=7.93, 95% CI 2.93 to 21.33) per 10 µg/m3 (pConclusionThis study suggests that air pollution has a negative impact on IPF outcomes, corroborating the role of ozone on AEs and establishing, for the first time, the potential role of long-term exposure to PM10 and PM2.5 on overall mortality.
Published: 2017

166. Pathology of Vascular Changes in Interstitial Lung Diseases

Author: Hilario Nunes, Marianne Kambouchner, Peter Dorfmüller, Jean-François Bernaudin, Dominique Valeyre, and Yurdagul Uzunhan
Subjects: Pathology, medicine.medical_specialty, Lung, business.industry, Connective tissue, respiratory system, medicine.disease, behavioral disciplines and activities, respiratory tract diseases, body regions, Idiopathic pulmonary fibrosis, medicine.anatomical_structure, Lymphangioleiomyomatosis, Medicine, Sarcoidosis, business, Vasculitis, Idiopathic interstitial pneumonia, Hypersensitivity pneumonitis
Abstract: Interstitial lung diseases (ILDs) embrace a heterogeneous group of disorders with diverse clinical outcomes. ILDs are classically separated into four categories: (i) ILDs of known cause, such as those in relation with occupational or environmental exposures, drugs, connective tissue diseases (CTDs), or vasculitis; (ii) idiopathic interstitial pneumonias (IIPs); (iii) sarcoidosis; and (iv) particular forms of ILDs, such as pulmonary Langerhans cell histiocytosis (PLCH), lymphangioleiomyomatosis (LAM), chronic idiopathic eosinophilic pneumonia [1]. More frequent ILDs are sarcoidosis, CTD-associated ILDs, hypersensitivity pneumonitis, and the chronic fibrosing IIPs, including idiopathic pulmonary fibrosis (IPF) and nonspecific interstitial pneumonia (NSIP).
Published: 2017

167. French practical guidelines for the diagnosis and management of idiopathic pulmonary fibrosis: 2017 update. Summary

Author: G. Prévot, Philippe Carré, Charles-Hugo Marquette, Gilbert Ferretti, Claire Dromer, Emmanuel Bergot, Sylvain Marchand-Adam, P. Terrioux, Jacques Cadranel, Philippe Camus, Jean-Baptiste Faivre, D. Bonnet, Bruno Crestani, B. Trumbic, Dominique Valeyre, Dominique Israel-Biet, Jean-François Cordier, B. Philippe, François Lebargy, Ronald C. Kessler, Stéphane Jouneau, Bernard Aguilaniu, Benoit Wallaert, N. Just, Vincent Cottin, J.-C. Dalphin, Claire Danel, Françoise Thivolet-Béjui, Emmanuel Gomez, Martine Reynaud-Gaubert, Centre de Référence des Maladies Pulmonaires Rares [Hôpital Louis Pradel - HCL], Hôpital Louis Pradel [CHU - HCL], Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Department of Pneumology [Lyon], Hospices Civils de Lyon (HCL), Physiopathologie et Epidemiologie de l'Insuffisance Respiratoire, Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Pneumologie A, AP-HP - Hôpital Bichat - Claude Bernard [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Centre de compétence des maladies pulmonaires rares, Département Hospitalo-universtaire FIRE (Fibrosis, Inflammation and Remodeling), LabEx Inflamex, Service de pneumologie A, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7)-Hôpital Bichat, Service de Pneumologie - Oncologie Thoracique - Maladies Pulmonaires Rares [CHU Tenon], CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Theranoscan, Université Pierre et Marie Curie - Paris 6 (UPMC), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon, Pathologies Respiratoires : Protéolyse et Aérosolthérapie, Université de Tours (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Toulouse [Toulouse], Service de Pneumologie et Immuno-Allergologie [CHU LIlle], Pole Cardio-vasculaire et pulmonaire [CHU Lille], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN), Service de Pneumologie Soins Intensifs, Appareillage Respiratoire [CHU de Dijon], Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), CHU Bordeaux [Bordeaux], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Service de Pneumologie, Paris, Institut de recherche en santé, environnement et travail (Irset), Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique )-Institut National de la Santé et de la Recherche Médicale (INSERM)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Université d'Angers (UA), Nouvel Hôpital Civil Strasbourg, Centre Hospitalier Universitaire de Nice (CHU Nice), Unité de Recherche sur les Maladies Infectieuses et Tropicales Emergentes (URMITE), Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR48, INSB-INSB-Centre National de la Recherche Scientifique (CNRS), Université Grenoble Alpes [2016-2019] (UGA [2016-2019]), Le CHCB, Centre Hospitalier de la Côte Basque, Centre Hospitalier de Carcassonne, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Centre Hospitalier de Roubaix, Plasticité de l'épithélium respiratoire dans les conditions normales et pathologiques - UMR-S 903 (PERPMP), Université de Reims Champagne-Ardenne (URCA)-Centre Hospitalier Universitaire de Reims (CHU Reims)-Institut National de la Santé et de la Recherche Médicale (INSERM)-SFR CAP Santé (Champagne-Ardenne Picardie Santé), Université de Reims Champagne-Ardenne (URCA)-Université de Picardie Jules Verne (UPJV)-Université de Reims Champagne-Ardenne (URCA)-Université de Picardie Jules Verne (UPJV), Centre Hospitalier René Dubos [Pontoise], Centre Hospitalier de Meaux, Groupe Hospitalier Est, Centre de Pathologie Est, Service des maladies respiratoires, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Groupe Hospitalier Avicenne-Jean Verdier-René Muret, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Université d'Angers (UA)-Université de Rennes (UR)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Institut des sciences biologiques (INSB-CNRS)-Institut des sciences biologiques (INSB-CNRS)-Centre National de la Recherche Scientifique (CNRS), Centre Hospitalier de la Côte Basque (CHCB), Université de Reims Champagne-Ardenne (URCA)-Université de Reims Champagne-Ardenne (URCA), Université de Tours-Institut National de la Santé et de la Recherche Médicale (INSERM), SFR CAP Santé (Champagne-Ardenne Picardie Santé), and Université de Reims Champagne-Ardenne (URCA)-Université de Picardie Jules Verne (UPJV)-Université de Reims Champagne-Ardenne (URCA)-Université de Picardie Jules Verne (UPJV)-Centre Hospitalier Universitaire de Reims (CHU Reims)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Reims Champagne-Ardenne (URCA)
Subjects: Pulmonary and Respiratory Medicine, Pediatrics, medicine.medical_specialty, Biopsy, MEDLINE, Lung pathology, 030218 nuclear medicine & medical imaging, Diagnosis, Differential, 03 medical and health sciences, Idiopathic pulmonary fibrosis, 0302 clinical medicine, X ray computed, Pulmonary Medicine, Humans, Medicine, Lung, ComputingMilieux_MISCELLANEOUS, [SDV.EE.SANT]Life Sciences [q-bio]/Ecology, environment/Health, business.industry, medicine.disease, Idiopathic Pulmonary Fibrosis, 3. Good health, 030228 respiratory system, Evidence-Based Practice, Radiography, Thoracic, France, Tomography, X-Ray Computed, business, Algorithms
Abstract: International audience; no abstract
Published: 2017

168. French practical guidelines for the diagnosis and management of idiopathic pulmonary fibrosis - 2017 update. Full-length version

Author: Françoise Thivolet-Béjui, Gilbert Ferretti, D. Bonnet, G. Prévot, N. Just, Vincent Cottin, Jean-Charles Dalphin, Philippe Camus, P. Terrioux, Jean-Baptiste Faivre, Dominique Israel-Biet, Bruno Crestani, Sylvain Marchand-Adam, B. Trumbic, Bernard Aguilaniu, Charles-Hugo Marquette, Jean-François Cordier, Ronald C. Kessler, Martine Reynaud-Gaubert, Jacques Cadranel, Philippe Carré, Emmanuel Bergot, François Lebargy, Emmanuel Gomez, Dominique Valeyre, B. Philippe, Stéphane Jouneau, Benoit Wallaert, Claire Danel, Claire Dromer, Centre de Référence des Maladies Pulmonaires Rares [Hôpital Louis Pradel - HCL], Hôpital Louis Pradel [CHU - HCL], Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Department of Pneumology [Lyon], Hospices Civils de Lyon (HCL), Service de Pneumologie A, AP-HP - Hôpital Bichat - Claude Bernard [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Centre de compétence des maladies pulmonaires rares, Service de Pneumologie - Oncologie Thoracique - Maladies Pulmonaires Rares [CHU Tenon], CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Pathologies Respiratoires : Protéolyse et Aérosolthérapie, Université de Tours-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Toulouse [Toulouse], Service de Pneumologie et Immuno-Allergologie [CHU LIlle], Pole Cardio-vasculaire et pulmonaire [CHU Lille], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN), Service de Pneumologie Soins Intensifs, Appareillage Respiratoire [CHU de Dijon], Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), CHU Bordeaux [Bordeaux], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Service de Pneumologie, Paris, Institut de recherche en santé, environnement et travail (Irset), Université d'Angers (UA)-Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Nouvel Hôpital Civil Strasbourg, Centre Hospitalier Universitaire de Nice (CHU Nice), Unité de Recherche sur les Maladies Infectieuses et Tropicales Emergentes (URMITE), Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR48, INSB-INSB-Centre National de la Recherche Scientifique (CNRS), Université Grenoble Alpes [2016-2019] (UGA [2016-2019]), Le CHCB, Centre Hospitalier de la Côte Basque, Centre Hospitalier de Carcassonne, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Centre Hospitalier de Roubaix, Plasticité de l'épithélium respiratoire dans les conditions normales et pathologiques - UMR-S 903 (PERPMP), SFR CAP Santé (Champagne-Ardenne Picardie Santé), Université de Reims Champagne-Ardenne (URCA)-Université de Picardie Jules Verne (UPJV)-Université de Reims Champagne-Ardenne (URCA)-Université de Picardie Jules Verne (UPJV)-Centre Hospitalier Universitaire de Reims (CHU Reims)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Reims Champagne-Ardenne (URCA), Centre Hospitalier René Dubos [Pontoise], Centre Hospitalier de Meaux, Groupe Hospitalier Est, Centre de Pathologie Est, Service des maladies respiratoires, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Groupe Hospitalier Avicenne-Jean Verdier-René Muret, Université Claude Bernard Lyon 1 (UCBL), Université de Lyon, Université Paris Diderot - Paris 7 (UPD7), Université Pierre et Marie Curie - Paris 6 (UPMC), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Hôpital Albert Calmette, Université de Lille, Droit et Santé-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Hôpital JeanMinjoz, Laboratoire Chrono-environnement (UMR 6249) (LCE), Centre National de la Recherche Scientifique (CNRS)-Université de Franche-Comté (UFC), Université Bourgogne Franche-Comté [COMUE] (UBFC)-Université Bourgogne Franche-Comté [COMUE] (UBFC), Hôpital Haut-Lévêque [CHU Bordeaux], Université d'Angers (UA)-Université de Rennes (UR)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), CHU Pontchaillou [Rennes], Institut des sciences biologiques (INSB-CNRS)-Institut des sciences biologiques (INSB-CNRS)-Centre National de la Recherche Scientifique (CNRS), Hôpital Nord [CHU - APHM], Institut Hospitalier Universitaire Méditerranée Infection (IHU Marseille), Centre Hospitalier de la Côte Basque (CHCB), CHU Grenoble, Centre Hospitalier Victor Provo, Hôpital Maison Blanche, Centre Hospitalier Universitaire de Reims (CHU Reims), Hôpital Avicenne [AP-HP], Université de Tours (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM), Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique )-Institut National de la Santé et de la Recherche Médicale (INSERM)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Université d'Angers (UA), Université de Reims Champagne-Ardenne (URCA)-Centre Hospitalier Universitaire de Reims (CHU Reims)-Institut National de la Santé et de la Recherche Médicale (INSERM)-SFR CAP Santé (Champagne-Ardenne Picardie Santé), and Université de Reims Champagne-Ardenne (URCA)-Université de Picardie Jules Verne (UPJV)-Université de Reims Champagne-Ardenne (URCA)-Université de Picardie Jules Verne (UPJV)
Subjects: Pulmonary and Respiratory Medicine, Pediatrics, medicine.medical_specialty, Biopsy, MEDLINE, Short length, Lung pathology, Bronchoalveolar Lavage, [SDV.MHEP.PSR]Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract, Diagnosis, Differential, 03 medical and health sciences, Idiopathic pulmonary fibrosis, 0302 clinical medicine, X ray computed, medicine, Pulmonary Medicine, Humans, 030212 general & internal medicine, Lung, ComputingMilieux_MISCELLANEOUS, [SDV.EE.SANT]Life Sciences [q-bio]/Ecology, environment/Health, business.industry, medicine.disease, Idiopathic Pulmonary Fibrosis, 3. Good health, 030228 respiratory system, Evidence-Based Practice, Radiography, Thoracic, France, business, Tomography, X-Ray Computed, Algorithms
Abstract: International audience; no abstract
Published: 2017

169. Shared genetic predisposition in rheumatoid arthritis-interstitial lung disease and familial pulmonary fibrosis

Author: Steven Gazal, Hilario Nunes, Jean Sibilia, Christelle Ménard, Aurélien Justet, Philippe Dieudé, Isabelle Callebaut, Amélie Bonnefond, Martin Soubrier, Patrick Revy, Catherine Boileau, Pierre-Antoine Juge, Thierry Schaeverbeke, Aline Frazier, Nathalie Saidenberg, Sébastien Ottaviani, Nadia Nathan, Bruno Crestani, Christophe Béroud, Baptiste Coustet, Vincent Cottin, Lidwine Wemeau-Stervinou, Jean-Pierre Desvignes, Marie-Christophe Boissier, Florence Dastot-Le Moal, Serge Amselem, Philippe Froguel, Yannick Allanore, Annick Clement, Olivier Sand, Gabriel Thabut, Marie-Pierre Debray, Pascal Richette, Caroline Kannengiesser, Benoit Wallaert, Christophe Richez, Dominique Valeyre, Sylvain Marchand-Adam, Huguette Lioté, Nicolas Leulliot, René-Marc Flipo, Raphael Borie, Claire Dromer, David Salgado, Service de Rhumathologie, Hôpital Bichat - Claude Bernard, Assistance Publique - Hôpitaux de Paris (AP-HP), Université Paris Diderot - Paris 7 (UPD7), Université Sorbonne Paris Cité (USPC), Service de Pneumologie, Centre Hospitalier Lyon Sud [CHU - HCL] (CHLS), Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), UMR 1152, Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Génétique, Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E), UMR 1149, Centre de Recherches sur l'Inflammation, Ecologie et Ecophysiologie Forestières [devient SILVA en 2018] (EEF), Institut National de la Recherche Agronomique (INRA)-Université de Lorraine (UL), Plateforme de génomique constitutionnelle, Imagine - Institut des maladies génétiques (IMAGINE - U1163), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de Pneumologie et Immuno-Allergologie [CHU LIlle], Pole Cardio-vasculaire et pulmonaire [CHU Lille], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Université de Lille, Service de Radiologie, Hospices Civils de Lyon (HCL), UMR 1100, Université Francois Rabelais [Tours], Physiopathologie des maladies génétiques d'expression pédiatrique, Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Université Pierre et Marie Curie - Paris 6 (UPMC), CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Service de Rhumatologie, CH Belfort-Montbéliard, UMR 5164, Immuno ConcEpT Lab, Centre National de la Recherche Scientifique (CNRS), Université de Bordeaux (UB), Sorbonne Université (SU), Institut de minéralogie, de physique des matériaux et de cosmochimie (IMPMC), Muséum national d'Histoire naturelle (MNHN)-Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut de recherche pour le développement [IRD] : UR206-Centre National de la Recherche Scientifique (CNRS), Institut de Recherche pour le Développement (IRD [France-Ouest]), Muséum national d'Histoire naturelle (MNHN), Laboratoire de cristallographie et RMN biologiques (LCRB - UMR 8015), Université Paris Descartes - Paris 5 (UPD5)-Institut de Chimie du CNRS (INC)-Centre National de la Recherche Scientifique (CNRS), Université Paris Descartes - Paris 5 (UPD5), Université de Lille, Droit et Santé, Institut Européen de Génomique du Diabète - European Genomic Institute for Diabetes - FR 3508 (EGID), Institut Pasteur de Lille, Réseau International des Instituts Pasteur (RIIP)-Réseau International des Instituts Pasteur (RIIP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre National de la Recherche Scientifique (CNRS), Génétique Médicale et Génomique Fonctionnelle (GMGF), Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)- Hôpital de la Timone [CHU - APHM] (TIMONE)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), UMR 1132, Service de Rhumatologie, Hôpital Lariboisière-Fernand-Widal [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Metabolic functional (epi)genomics and molecular mechanisms involved in type 2 diabetes and related diseases - UMR 8199 - UMR 1283 (EGENODIA (GI3M)), Department of Genomics of Common Diseases, Imperial College London, Service Rhumatologie A, Hôpital Cochin [AP-HP], Institut Cochin (IC UM3 (UMR 8104 / U1016)), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Service Imagerie, service de Rhumatologie, Centre Hospitalier Universitaire de Lille (CHU de Lille), Laboratoire de génétique des maladies rares. Pathologie moleculaire, etudes fonctionnelles et banque de données génétiques (LGMR), Université Montpellier 1 (UM1)-IFR3, Université Montpellier 1 (UM1)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM), Service Rhumatologie, Hôpital La Rabta [Tunis], FMTS, CRHI, Laboratoire Immunologie Rhumatologie Moléculaire, Université de Strasbourg (UNISTRA), Fédération Hospitalo-Universitaire OMICARE, Rétrovirus et Pathologie Comparée (RPC), Institut National de la Recherche Agronomique (INRA)-École Pratique des Hautes Études (EPHE), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Ecole Nationale Vétérinaire de Lyon (ENVL), Maladie Pulmonaires Rares, Hôpital Louis Pradel [CHU - HCL], UMR U1125 Service rhumatologie, service de rhumatologie, CHU Clermont-Ferrand, Unité de Nutrition Humaine (UNH), Institut National de la Recherche Agronomique (INRA)-Université Clermont Auvergne [2017-2020] (UCA [2017-2020]), Centre de Recherche en Nutrition Humaine d'Auvergne (CRNH d'Auvergne), Hôpital Avicenne [AP-HP], service de génétique, Societe Francaise de Rhumatologie, Club Rhumatismes Inflammation, la Chancellerie des Universites de Paris (legs Poix), Sorbonne Paris Cite (FPI-SPC Program), Agence Nationale de la Recherche ANR-10-LABX-46 ANR-10-EQPX-07-01 ANR-14-CE10-0006 ANR-10-INBS-09, France Genomique National Infrastructure, Pfizer, Chugai, Centre de Resources Biologiques Hopital Bichat Paris France, ANR-14-CE10-0006,GENEXGERTEL,Rôles génomiques et extragénomiques de RTEL1(2014), Hôpital Bichat - Claude Bernard, Assistance Publique - Hôpitaux de Paris ( AP-HP ), Université Paris Diderot (Paris 7), Université Sorbonne Paris Cité ( USPC ), Centre Hospitalier Lyon Sud [CHU - HCL] ( CHLS ), Hospices Civils de Lyon ( HCL ) -Hospices Civils de Lyon ( HCL ), Institut National de la Santé et de la Recherche Médicale ( INSERM ), CHU Saint-Etienne, UMR 1137, Fac Sci, Imagine - Institut des maladies génétiques ( IMAGINE - U1163 ), Université Paris Descartes - Paris 5 ( UPD5 ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Centre National de la Recherche Scientifique ( CNRS ), Service de Pneumologie et Immuno-Allergologie, Centre Hospitalier Régional Universitaire [Lille] ( CHRU Lille ), Hospices Civils de Lyon ( HCL ), Service de Pneumologie Pédiatrique, Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Trousseau [APHP], Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Université Pierre et Marie Curie (Paris 6), Centre National de la Recherche Scientifique ( CNRS ), Université de Bordeaux ( UB ), Sorbonne Universités, Institut de minéralogie, de physique des matériaux et de cosmochimie ( IMPMC ), Muséum National d'Histoire Naturelle ( MNHN ) -Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Institut de recherche pour le développement [IRD] : UR206-Centre National de la Recherche Scientifique ( CNRS ), Institut de Recherche pour le Développement ( IRD [France-Ouest] ), Muséum National d’Histoire Naturelle ( MNHN ), Laboratoire de cristallographie et RMN biologiques ( LCRB - UMR 8015 ), Université Paris Descartes - Paris 5 ( UPD5 ) -Centre National de la Recherche Scientifique ( CNRS ), Université Paris Descartes - Paris 5 ( UPD5 ), Université Droit et Santé (Lille 2) ( UDSL ), European Genomic Institute for Diabetes ( EGID ), Génétique Médicale et Génomique Fonctionnelle ( GMGF ), Aix Marseille Université ( AMU ) -Assistance Publique - Hôpitaux de Marseille ( APHM ) - Hôpital de la Timone [CHU - APHM] ( TIMONE ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Centre National de la Recherche Scientifique ( CNRS ), Hôpital Lariboisière, Génomique Intégrative et Modélisation des Maladies Métaboliques ( EGID ), Université de Lille-Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Institut Pasteur de Lille, Réseau International des Instituts Pasteur ( RIIP ) -Réseau International des Instituts Pasteur ( RIIP ) -Centre National de la Recherche Scientifique ( CNRS ) -Centre Hospitalier Régional Universitaire [Lille] ( CHRU Lille ), CHU Cochin [AP-HP], Institut Cochin ( UM3 (UMR 8104 / U1016) ), Centre Hospitalier Universitaire de Lille ( CHU de Lille ), Laboratoire de génétique des maladies rares. Pathologie moleculaire, etudes fonctionnelles et banque de données génétiques, Université Montpellier 1 ( UM1 ) -IFR3-Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Université de Montpellier ( UM ), Hôpital La Rabta [Tunis), Université de Strasbourg ( UNISTRA ), Rétrovirus et Pathologie Comparée ( RPC ), Institut National de la Recherche Agronomique ( INRA ) -École pratique des hautes études ( EPHE ) -Université Claude Bernard Lyon 1 ( UCBL ), Université de Lyon-Université de Lyon-Ecole Nationale Vétérinaire de Lyon ( ENVL ), Département Génétique, Institut de l'Elevage, Centre Reference Maladies Respiratoires Rares, Hôpital Armand Trousseau, Assistance Publique - Hôpitaux de Paris ( AP-HP ), Centre Hospitalier Universitaire de Clermont-Ferrand, Unité de Nutrition Humaine - Clermont Auvergne ( UNH ), Université Clermont Auvergne ( UCA ) -Institut national de la recherche agronomique [Auvergne/Rhône-Alpes] ( INRA Auvergne/Rhône-Alpes ), Centre de Recherche en Nutrition Humaine d'Auvergne ( CRNH d'Auvergne ), Hôpital Avicenne, Assistance Publique - Hôpitaux de Paris ( AP-HP ), European Genomic Institute for Diabetes - FR 3508 (EGID), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre National de la Recherche Scientifique (CNRS), Institut National de la Santé et de la Recherche Médicale (INSERM)-Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)- Hôpital de la Timone [CHU - APHM] (TIMONE)-Centre National de la Recherche Scientifique (CNRS), Metabolic functional (epi)genomics and molecular mechanisms involved in type 2 diabetes and related diseases - UMR 8199 - UMR 1283 (GI3M), Centre National de la Recherche Scientifique (CNRS)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), Institut National de la Recherche Agronomique (INRA)-École pratique des hautes études (EPHE), Institut de l'élevage (IDELE), ProdInra, Archive Ouverte, Centre de référence national pour les maladies respiratoires rares de l’enfant RespiRare [CHU Trousseau], Service de Pneumologie pédiatrique [CHU Trousseau], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Service de génétique et embryologie médicales [CHU Trousseau], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Trousseau [APHP], Institut de recherche pour le développement [IRD] : UR206-Centre National de la Recherche Scientifique (CNRS)-Muséum national d'Histoire naturelle (MNHN)-Université Pierre et Marie Curie - Paris 6 (UPMC), Centre National de la Recherche Scientifique (CNRS)-Université Paris Descartes - Paris 5 (UPD5), European Genomic Institute for Diabetes [Lille] (EGID), Génomique Intégrative et Modélisation des Maladies Métaboliques (EGID), Université de Lille-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut Pasteur de Lille, Réseau International des Instituts Pasteur (RIIP)-Réseau International des Instituts Pasteur (RIIP)-Centre National de la Recherche Scientifique (CNRS)-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Institut National de la Recherche Agronomique (INRA)-École pratique des hautes études (EPHE)-Université Claude Bernard Lyon 1 (UCBL), Hôpital Armand Trousseau, Assistance Publique - Hôpitaux de Paris (AP-HP), Unité de Nutrition Humaine - Clermont Auvergne (UNH), Institut National de la Recherche Agronomique (INRA)-Université Clermont Auvergne (UCA), Hôpital Avicenne, Assistance Publique - Hôpitaux de Paris (AP-HP), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut de recherche pour le développement [IRD] : UR206-Muséum national d'Histoire naturelle (MNHN)-Centre National de la Recherche Scientifique (CNRS), Centre National de la Recherche Scientifique (CNRS)-Institut de Chimie du CNRS (INC)-Université Paris Descartes - Paris 5 (UPD5), Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille, IFR3, Université Montpellier 1 (UM1)-Université Montpellier 1 (UM1)-Université de Montpellier (UM)-Institut National de la Santé et de la Recherche Médicale (INSERM), Université Paris Descartes - Paris 5 (UPD5)-Centre National de la Recherche Scientifique (CNRS), and Réseau International des Instituts Pasteur (RIIP)-Réseau International des Instituts Pasteur (RIIP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)-Centre National de la Recherche Scientifique (CNRS)
Subjects: Male, Pathology, medicine.disease_cause, Arthritis, Rheumatoid, 0302 clinical medicine, Risk Factors, Pulmonary fibrosis, Exome, Telomerase, Exome sequencing, Mutation, [SDV.MHEP] Life Sciences [q-bio]/Human health and pathology, Interstitial lung disease, Middle Aged, 3. Good health, Europe, Polyarthrite rhumatoïde, maladie pulmonaire, Phenotype, Rheumatoid arthritis, Female, Pulmonary and Respiratory Medicine, Adult, medicine.medical_specialty, Heterozygote, fibrose pulmonaire, 03 medical and health sciences, [ SDV.MHEP ] Life Sciences [q-bio]/Human health and pathology, medicine, Genetic predisposition, Humans, Genetic Predisposition to Disease, respiratory system diseases, Genetic Association Studies, Aged, 030203 arthritis & rheumatology, pulmonary fibrosis, business.industry, Case-control study, DNA Helicases, Sequence Analysis, DNA, medicine.disease, 030228 respiratory system, Case-Control Studies, Immunology, business, Lung Diseases, Interstitial, Software, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
Abstract: Despite its high prevalence and mortality, little is known about the pathogenesis of rheumatoid arthritis-associated interstitial lung disease (RA-ILD). Given that familial pulmonary fibrosis (FPF) and RA-ILD frequently share the usual pattern of interstitial pneumonia and common environmental risk factors, we hypothesised that the two diseases might share additional risk factors, including FPF-linked genes. Our aim was to identify coding mutations of FPF-risk genes associated with RA-ILD.We used whole exome sequencing (WES), followed by restricted analysis of a discrete number of FPF-linked genes and performed a burden test to assess the excess number of mutations in RA-ILD patients compared to controls.Among the 101 RA-ILD patients included, 12 (11.9%) had 13 WES-identified heterozygous mutations in the TERT, RTEL1, PARN or SFTPC coding regions. The burden test, based on 81 RA-ILD patients and 1010 controls of European ancestry, revealed an excess of TERT, RTEL1, PARN or SFTPC mutations in RA-ILD patients (OR 3.17, 95% CI 1.53–6.12; p=9.45×10−4). Telomeres were shorter in RA-ILD patients with a TERT, RTEL1 or PARN mutation than in controls (p=2.87×10−2).Our results support the contribution of FPF-linked genes to RA-ILD susceptibility.
Published: 2017

170. Effect of pirfenidone on mortality: pooled analyses and meta-analyses of clinical trials in idiopathic pulmonary fibrosis

Author: Lisa Lancaster, Steven D. Nathan, Ian Glaspole, Paul W. Noble, David J. Lederer, Carlo Albera, Klaus Uwe Kirchgaessler, Monica Daigl, Dominique Valeyre, Jeffrey J. Swigris, Carlos Alberto de Castro Pereira, Ulrich Costabel, Marilyn K. Glassberg, David Kardatzke, and Williamson Z. Bradford
Subjects: Pulmonary and Respiratory Medicine, medicine.medical_specialty, Pyridones, Population, Vital Capacity, Medizin, Placebo, 03 medical and health sciences, Idiopathic pulmonary fibrosis, 0302 clinical medicine, Internal medicine, Cause of Death, medicine, Humans, 030212 general & internal medicine, education, Randomized Controlled Trials as Topic, education.field_of_study, business.industry, Hazard ratio, Anti-Inflammatory Agents, Non-Steroidal, Pirfenidone, medicine.disease, Idiopathic Pulmonary Fibrosis, Surgery, Clinical trial, Treatment Outcome, 030228 respiratory system, Clinical Trials, Phase III as Topic, Relative risk, Meta-analysis, business, medicine.drug
Abstract: Summary Background In clinical trials of idiopathic pulmonary fibrosis, rates of all-cause mortality are low. Thus prospective mortality trials are logistically very challenging, justifying the use of pooled analyses or meta-analyses. We did pooled analyses and meta-analyses of clinical trials of pirfenidone versus placebo to determine the effect of pirfenidone on mortality outcomes over 120 weeks. Methods We did a pooled analysis of the combined patient populations of the three global randomised phase 3 trials of pirfenidone versus placebo—Clinical Studies Assessing Pirfenidone in Idiopathic Pulmonary Fibrosis: Research of Efficacy and Safety Outcomes (CAPACITY 004 and 006; trial durations 72–120 weeks) and Assessment of Pirfenidone to Confirm Efficacy and Safety in Idiopathic Pulmonary Fibrosis (ASCEND 016; 52 weeks)—for all-cause mortality, treatment-emergent all-cause mortality, idiopathic-pulmonary-fibrosis-related mortality, and treatment-emergent idiopathic-pulmonary-fibrosis-related mortality at weeks 52, 72, and 120. We also did meta-analyses of these data and data from two Japanese trials of pirfenidone versus placebo—Shionogi Phase 2 (SP2) and Shionogi Phase 3 (SP3; trial durations 36–52 weeks). Findings At week 52, the relative risk of death for all four mortality outcomes was significantly lower in the pirfenidone group than in the placebo group in the pooled population (all-cause mortality hazard ratio [HR] 0·52 [95% CI 0·31–0·87; p=0·0107]; treatment-emergent all-cause mortality 0·45 [0·24–0·83; 0·0094]; idiopathic-pulmonary-fibrosis-related mortality 0·35 [0·17–0·72; 0·0029]; treatment-emergent idiopathic-pulmonary-fibrosis-related mortality 0·32 [0·14–0·76; 0·0061]). Consistent with the pooled analysis, meta-analyses for all-cause mortality at week 52 also showed a clinically relevant and significant risk reduction in the pirfenidone group compared with the placebo group. Over 120 weeks, we noted significant differences in the pooled analysis favouring pirfenidone therapy compared with placebo for treatment-emergent all-cause mortality (p=0·0420), idiopathic-pulmonary-fibrosis-related mortality (0·0237), and treatment-emergent idiopathic-pulmonary-fibrosis-related (0·0132) mortality; similar results were shown by meta-analyses. Interpretation Several analytic approaches demonstrated that pirfenidone therapy is associated with a reduction in the relative risk of mortality compared with placebo over 120 weeks. Funding F Hoffmann-La Roche/Genentech.
Published: 2017

171. Impact des facteurs démographiques sur l’étiologie des PID

Author: Bruno Crestani, Jacques Cadranel, B. Duchemann, Dominique Valeyre, Isabella Annesi-Maesano, C. Jacobe De Naurois, Paul-André Rosental, Hilario Nunes, Service de pneumologie [Avicenne], Hôpital Avicenne [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Chimie, Structures et Propriétés de Biomatériaux et d'Agents Thérapeutiques (CSPBAT), Université Paris 13 (UP13)-Institut Galilée-Université Sorbonne Paris Cité (USPC)-Institut de Chimie du CNRS (INC)-Centre National de la Recherche Scientifique (CNRS), Service d'Oncologie Médicale [AP-HP Hôpital Avicenne], Université Paris 13 (UP13)-Hôpital Avicenne [AP-HP], Institut des Sciences du Mouvement Etienne Jules Marey (ISM), Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS), PSA Peugeot Citroën (PSA), Centre d'histoire de Sciences Po (Sciences Po) (CHSP), Sciences Po (Sciences Po), European Project: 295817,EC:FP7:ERC,ERC-2011-ADG_20110406,SILICOSIS(2012), Institut de Chimie du CNRS (INC)-Centre National de la Recherche Scientifique (CNRS)-Université Paris 13 (UP13)-Institut Galilée-Université Sorbonne Paris Cité (USPC), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris 13 (UP13), Centre National de la Recherche Scientifique (CNRS)-Aix Marseille Université (AMU), and Centre d'histoire de Sciences Po (CHSP)
Subjects: Pulmonary and Respiratory Medicine, [SHS.HISPHILSO]Humanities and Social Sciences/History, Philosophy and Sociology of Sciences, 03 medical and health sciences, 0302 clinical medicine, Impact, 030228 respiratory system, [SHS.ANTHRO-BIO]Humanities and Social Sciences/Biological anthropology, 030212 general & internal medicine, Pneumopathies interstitielles diffuses chroniques, [SDE.ES]Environmental Sciences/Environmental and Society, 3. Good health, Facteurs démographiques
Abstract: Introduction L’impact de l’âge, du sexe et du tabac est bien connu dans certaines pneumopathies interstitielles diffuses chroniques (PIDc). Concernant l’origine geographique, alors que son role dans la sarcoidose est avere, peu de donnees sont disponibles dans les autres PIDc. L’objectif du travail etait d’evaluer l’impact de l’origine geographique dans l’etiologie des PIDc en prenant en consideration les autres facteurs demographiques confondants possibles ( Fig. 1 ). Methodes Il s’agit d’une etude epidemiologique prospective estimant l’incidence et la prevalence des PIDc dans le departement de Seine Saint Denis. Mille cent soixante-dix cas de PID ont ete declares par plusieurs sources (dont les medecins generalistes, pneumologues et internistes, et la caisse primaire d’assurance maladie) ; 848 ont pu etre revus de maniere centralisee lors d’une discussion multidisciplinaire. Les caracteristiques demographiques ont ete repertoriees. Les prevalences ont ete standardisees sur l’origine geographique. Une analyse de regression logistique multinomiale a permis d’obtenir des odd ratio relatifs a l’origine geographique et independants des facteurs confondants (âge, sexe et tabac). Resultats Les diagnostics les plus frequents etaient la sarcoidose (42,6 %), les PID de connectivites (CTDs) (16 %) et la FPI (11,6 %). Les trois groupes etaient differents en termes d’âge ( p p p p Fig. 1 . Conclusion Notre etude confirme le risque accru de sarcoidose et de CTDs au sein des PIDc chez les afro-caribeens apres correction sur les facteurs confondants. Elle suggere un risque particulierement majore de developper plusieurs types de PIDc dans la population maghrebine.
Published: 2017

172. Advanced pulmonary sarcoidosis

Author: Hilario Nunes, Jean-François Bernaudin, and Dominique Valeyre
Subjects: Pulmonary and Respiratory Medicine, Spirometry, medicine.medical_specialty, medicine.diagnostic_test, business.industry, Hypertension, Pulmonary, Pulmonary Fibrosis, medicine.disease, Chronic disease, Pulmonary sarcoidosis, Sarcoidosis, Pulmonary, Usual interstitial pneumonia, Chronic Disease, Humans, Medicine, Sarcoidosis, Pulmonary pathology, Radiology, Tomography, X-Ray Computed, business
Abstract: To present an update on the most recent contributions in advanced pulmonary sarcoidosis (APS).Pathology is better described and the differences between fibrosing pulmonary sarcoidosis and usual interstitial pneumonia (UIP) are clarified. Serial spirometry is the most reliable tool for monitoring evolution. Survival may be predicted by an integrative algorithm based on pulmonary function and computed tomography (CT).APS is characterized by significant fibrocystic pulmonary lesions at CT and pathology. There are two main patterns of APS, one with predominant central bronchovascular distortion, often associated with airflow limitation, and the other with predominant honeycombing with a different location than in UIP with severe restrictive impairment and very low diffusion capacity of the lung for carbon monoxide. APS may be burnt out but is most often still active as evidenced by several findings, including on F-fluorodeoxyglucose-PET. There is an increased mortality and morbidity with chronic respiratory insufficiency, pulmonary hypertension stemming from multiple mechanisms, chronic pulmonary aspergillosis and extra infections. Acute worsening episodes are frequent. Serial spirometry, particularly forced vital capacity, is the most reliable tool for monitoring evolution. A new elegant algorithm based on pulmonary function and CT may predict survival. Despite important stakes, there is still a lack of therapeutic recommendations. However, the use of antisarcoidosis treatment is most often required at least as a temporary trial. Finally, the effect of pulmonary hypertension treatment has recently been the object of further evaluation.
Published: 2014

173. Diagnosis and management of idiopathic pulmonary fibrosis: French practical guidelines

Author: Jean-François Cordier, Philippe Carré, Romain Kessler, Nicolas Just, Bernard Aguilaniu, Serge Kouzan, François Lebargy, Philippe Delaval, Jacques Cadranel, Benoit Wallaert, Sylvain Marchand-Adam, Bruno Crestani, Dominique Valeyre, Jean-Baptiste Faivre, B. Philippe, Jean-Charles Dalphin, B. Bouquillon, Grégoire Prévot, Françoise Thivolet-Béjui, Martine Reynaud-Gaubert, B. Stach, Dominique Israel-Biet, Gilbert Ferretti, Vincent Cottin, Claire Danel, Department of Pneumology [Lyon], Hospices Civils de Lyon (HCL), Centre de Référence des Maladies Pulmonaires Rares [Hôpital Louis Pradel - HCL], Hôpital Louis Pradel [CHU - HCL], Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Infections Virales et Pathologie Comparée - UMR 754 (IVPC), École pratique des hautes études (EPHE), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE), AP-HP - Hôpital Bichat - Claude Bernard [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Hôpital Avicenne [AP-HP], Service de Pneumologie et Immuno-Allergologie [CHU LIlle], Pole Cardio-vasculaire et pulmonaire [CHU Lille], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Service de Pneumologie - Oncologie Thoracique - Maladies Pulmonaires Rares [CHU Tenon], CHU Tenon [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), CHU Pontchaillou [Rennes], Hôpital Européen Georges Pompidou [APHP] (HEGP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), CHU Strasbourg, CHU Marseille, Clinique du Mail, Opened Mind Health and Associates, Polyclinique Montréal, Partenaires INRAE, Hôpital Albert Calmette, Université de Lille, Droit et Santé-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Service de Cancérologie-Radiothérapie Hôpital Albert MICHALLON (CHU de Grenoble), Université Joseph Fourier - Grenoble 1 (UJF), Centre Hospitalier Victor Provo, Centre Hospitalier Annecy-Genevois [Saint-Julien-en-Genevois], Hôpital Maison Blanche, Centre Hospitalier Universitaire de Reims (CHU Reims), Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), Centre d’Etude des Pathologies Respiratoires (CEPR), UMR 1100 (CEPR), Université de Tours (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre Hospitalier René Dubos [Pontoise], Hopital Larrey, Physiopathologie et Epidemiologie de l'Insuffisance Respiratoire, Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de pneumologie A, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7)-Hôpital Bichat, Département Hospitalo-universtaire FIRE (Fibrosis, Inflammation and Remodeling), LabEx Inflamex, Service de Pneumologie A, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Centre de compétence des maladies pulmonaires rares, Service des maladies respiratoires, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Groupe Hospitalier Avicenne-Jean Verdier-René Muret, Interactions cellulaires tumorales et leur environnement et réponse aux agents anti-cancéreux, Université Pierre et Marie Curie - Paris 6 (UPMC), Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Intergroupe Francophone de Cancérologie Thoracique [Paris] (IFCT), Intergroupe Francophone de Cancérologie thoracique, Laboratoire Chrono-environnement - CNRS - UBFC (UMR 6249) (LCE), Université de Franche-Comté (UFC), Université Bourgogne Franche-Comté [COMUE] (UBFC)-Université Bourgogne Franche-Comté [COMUE] (UBFC)-Centre National de la Recherche Scientifique (CNRS), Institut de recherche en santé, environnement et travail (Irset), Université d'Angers (UA)-Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Service de Pneumologie, Paris, Nouvel Hôpital Civil Strasbourg, Service de chirurgie thoracique, Assistance Publique - Hôpitaux de Marseille (APHM)-Hôpital nord, HYLAB : Physiologie de l'exercice et interprétation clinique, Department of Physical Education, McGill University = Université McGill [Montréal, Canada], Service d'Anatomie et cytologie pathologique, Université Paris Diderot - Paris 7 (UPD7)-AP-HP - Hôpital Bichat - Claude Bernard [Paris], Département de radiologie, CHU Grenoble-Hôpital Michallon, Unité d'Aide Méthodologique, Hôpital Robert Debré-Centre Hospitalier Universitaire de Reims (CHU Reims), Pathologies Respiratoires : Protéolyse et Aérosolthérapie, Registre Multicentrique à Vocation Nationale des Mésothéliomes Pleuraux (MESONAT), CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN), Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique )-Institut National de la Santé et de la Recherche Médicale (INSERM)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Université d'Angers (UA), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-AP-HP - Hôpital Bichat - Claude Bernard [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7), Hospices Civils de Lyon ( HCL ), Hospices Civils de Lyon ( HCL ) -Hospices Civils de Lyon ( HCL ), Université Paris Diderot - Paris 7 ( UPD7 ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Assistance publique - Hôpitaux de Paris (AP-HP)-Université Paris Diderot - Paris 7 ( UPD7 ) -Hôpital Bichat, AP-HP - Hôpital Bichat - Claude Bernard [Paris]-Centre de compétence des maladies pulmonaires rares, Assistance publique - Hôpitaux de Paris (AP-HP)-Groupe Hospitalier Avicenne-Jean Verdier-René Muret, Service de Pneumologie et Immuno-Allergologie, Centre Hospitalier Régional Universitaire [Lille] ( CHRU Lille ), Université Pierre et Marie Curie - Paris 6 ( UPMC ), Service de pneumologie et réanimation [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Tenon [APHP], Intergroupe Francophone de Cancérologie Thoracique [Paris] ( IFCT ), Laboratoire Chrono-environnement ( LCE ), Université Bourgogne Franche-Comté ( UBFC ) -Centre National de la Recherche Scientifique ( CNRS ) -Université de Franche-Comté ( UFC ), Institut de recherche, santé, environnement et travail ( Irset ), Université d'Angers ( UA ) -Université de Rennes 1 ( UR1 ), Université de Rennes ( UNIV-RENNES ) -Université de Rennes ( UNIV-RENNES ) -École des Hautes Études en Santé Publique [EHESP] ( EHESP ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ) -Université des Antilles ( UA ), Assistance Publique - Hôpitaux de Marseille ( APHM ) -Hôpital nord, McGill University-Université de Montréal, Assistance publique - Hôpitaux de Paris (AP-HP)-AP-HP - Hôpital Bichat - Claude Bernard [Paris]-Université Paris Diderot - Paris 7 ( UPD7 ), Université Joseph Fourier - Grenoble 1 ( UJF ) -CHU Grenoble-Hôpital Michallon, Hôpital Robert Debré-Centre Hospitalier Universitaire de Reims ( CHU Reims ), Université de Tours-Institut National de la Santé et de la Recherche Médicale ( INSERM ), Centre national référent cancers rares - mésothéliomes malins pleuraux et tumeurs péritonéales rares ( MESOPATH ), CHU Caen-Hôpital côte de nacre, Centre National de la Recherche Scientifique (CNRS)-Université de Franche-Comté (UFC), Université Bourgogne Franche-Comté [COMUE] (UBFC)-Université Bourgogne Franche-Comté [COMUE] (UBFC), and Université de Tours-Institut National de la Santé et de la Recherche Médicale (INSERM)
Subjects: Diagnostic Imaging, Pulmonary and Respiratory Medicine, medicine.medical_specialty, Pediatrics, Consensus, MEDLINE, [ SDV.EE.SANT ] Life Sciences [q-bio]/Ecology, environment/Health, 03 medical and health sciences, Idiopathic pulmonary fibrosis, 0302 clinical medicine, Predictive Value of Tests, Risk Factors, Pulmonary Medicine, medicine, Humans, 030212 general & internal medicine, Idiopathic interstitial pneumonia, Competence (human resources), Pulmonologists, lcsh:RC705-779, [SDV.EE.SANT]Life Sciences [q-bio]/Ecology, environment/Health, Evidence-Based Medicine, Modalities, Executive summary, business.industry, lcsh:Diseases of the respiratory system, Evidence-based medicine, medicine.disease, Idiopathic Pulmonary Fibrosis, 3. Good health, Treatment Outcome, 030228 respiratory system, Family medicine, France, business
Abstract: Initiative that emanated from the French National Reference Centre and the Network of Competence Centres for Rare Lung Diseases; International audience; Idiopathic pulmonary fibrosis (IPF) is the most frequent chronic idiopathic interstitial pneumonia in adults. The management of rare diseases in France has been organised by a national plan for rare diseases, which endorsed a network of expert centres for rare diseases throughout France. This article is an overview of the executive summary of the French guidelines for the management of IPF, an initiative that emanated from the French National Reference Centre and the Network of Regional Competence Centres for Rare Lung Diseases. This review aims at providing pulmonologists with a document that: 1) combines the current available evidence; 2) reviews practical modalities of diagnosis and management of IPF; and 3) is adapted to everyday medical practice. The French practical guidelines result from the combined efforts of a coordination committee, a writing committee and a multidisciplinary review panel, following recommendations from the Haute Autorité de Santé. All recommendations included in this article received at least 90% agreement by the reviewing panel. Herein, we summarise the main conclusions and practical recommendations of the French guidelines.
Published: 2014

174. FDG-PET/CT in patients with ANCA-associated vasculitis: Case-series and literature review

Author: A. Mekinian, G. Pop, Hilario Nunes, Rebecca Sberro-Soussan, Sébastien Abad, Michael Soussan, Benjamin Terrier, Véronique Eder, Olivier Fain, Loïc Guillevin, Noémie Abisror, Dominique Valeyre, and Robin Dhote
Subjects: Male, Paris, medicine.medical_specialty, Immunology, Microscopic Polyangiitis, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, Birmingham Vasculitis Activity Score, Eosinophilic, medicine, Humans, Immunology and Allergy, In patient, Aged, Retrospective Studies, Lung, business.industry, Middle Aged, medicine.disease, Occult, medicine.anatomical_structure, Positron-Emission Tomography, Female, Radiology, Granulomatosis with polyangiitis, business, Microscopic polyangiitis, Vasculitis
Abstract: Objectives We aimed to assess the clinical value of FDG-PET/CT in patients with ANCA-associated vasculitis. Materials and methods We retrospectively included 16 patients with ANCA-associated vasculitis who underwent 21 FDG-PET/CT between 2009 and 2013, in 2 university hospitals from the Paris suburb area. All FDG-PET/CTs were retrospectively analyzed and compared to clinical, biological and conventional imaging data at baseline and during the follow-up. Results ANCA-associated vasculitis was granulomatosis with polyangiitis (GPA, n = 10), microscopic polyangiitis (MPA, n = 4), and eosinophilic GPA (EGPA, n = 2). PET was performed at initial presentation in 14 cases and during the follow-up in 7 cases. At baseline, PET was positive in 100% of GPA patients (8/8) and in 50% (3/6) of patients with other ANCA-vasculitis (p = 0.05). FDG uptake tended to be higher in patients with GPA in comparison to patients with MPA/EGPA (median SUVmax: 5 versus 2.5; p = 0.08). Sinonasal, lung, cardio-vascular and kidney involvements were all accurately identified by PET, except in one MPA patient with glomerulonephritis. As expected, skin, joint, eye and peripheral nervous system impairments were not detected by PET. No occult site was detected by PET, except in 2 salivary gland FDG uptake without clinical abnormalities. Patients with GPA exhibited a higher number of positive sites on PET (2 [1.75–2.25] versus 0.5 [0–1], p = 0.006) than patients with MPA/EGPA. In pooled data including our study and the literature data of GPA patients (n=31), SUVmax was associated with Birmingham Vasculitis Activity Score (BVAS) (r=0.49; p=0.03). Conclusion FDG-PET/CT accurately identifies organ localizations in GPA, other than in nervous system, eye and skin, but do not bring additional benefit to the usual organ screening. The value of FDG-PET/CT in other ANCA-associated vasculitis need to be further addressed.
Published: 2014

175. Sarcoidosis-related mortality in France: a multiple-cause-of-death analysis

Author: Sébastien Kerever, Christiane Broussolle, Mathieu Gerfaud-Valentin, Yvan Jamilloux, Pascal Sève, Delphine Maucort-Boulch, Dominique Valeyre, Mireille Eb, Service de Biostatistiques [Lyon], Hospices Civils de Lyon (HCL), Biostatistiques santé, Département biostatistiques et modélisation pour la santé et l'environnement [LBBE], Laboratoire de Biométrie et Biologie Evolutive - UMR 5558 (LBBE), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National de Recherche en Informatique et en Automatique (Inria)-VetAgro Sup - Institut national d'enseignement supérieur et de recherche en alimentation, santé animale, sciences agronomiques et de l'environnement (VAS)-Centre National de la Recherche Scientifique (CNRS)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National de Recherche en Informatique et en Automatique (Inria)-VetAgro Sup - Institut national d'enseignement supérieur et de recherche en alimentation, santé animale, sciences agronomiques et de l'environnement (VAS)-Centre National de la Recherche Scientifique (CNRS)-Laboratoire de Biométrie et Biologie Evolutive - UMR 5558 (LBBE), Université de Lyon-Université de Lyon-Institut National de Recherche en Informatique et en Automatique (Inria)-VetAgro Sup - Institut national d'enseignement supérieur et de recherche en alimentation, santé animale, sciences agronomiques et de l'environnement (VAS)-Centre National de la Recherche Scientifique (CNRS), Service des maladies respiratoires, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Groupe Hospitalier Avicenne-Jean Verdier-René Muret, Centre de Recherche en Cancérologie de Lyon (UNICANCER/CRCL), Centre Léon Bérard [Lyon]-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM), and Université de Lyon-Université de Lyon-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)
Subjects: Pulmonary and Respiratory Medicine, Male, medicine.medical_specialty, Pediatrics, Tuberculosis, Sarcoidosis, analysis, Population, [SDV.CAN]Life Sciences [q-bio]/Cancer, 030204 cardiovascular system & hematology, 03 medical and health sciences, 0302 clinical medicine, Cause of Death, Neoplasms, Epidemiology, medicine, Young adult, education, Cause of death, Aged, education.field_of_study, business.industry, Mortality rate, medicine.disease, mortality, 3. Good health, 030228 respiratory system, Infectious disease (medical specialty), Cardiovascular Diseases, Medicine, France, business
Abstract: We evaluated mortality rates and underlying causes of death among French decedents with sarcoidosis from 2002 to 2011.We used data from the French Epidemiological Centre for the Medical Causes of Death to 1) calculate sarcoidosis-related mortality rates, 2) examine differences by age and gender, 3) determine underlying and nonunderlying causes of death, 4) compare with the general population (observed/expected ratios), and 5) analyse regional differences.1662 death certificates mentioning sarcoidosis were recorded. The age-standardised mortality rate was 3.6 per million population and significantly increased over the study period. The mean age at death was 70.4 years (versus 76.2 years for the general population). The most common underlying cause of death was sarcoidosis. Sarcoidosis decedents were more likely to be males when aged 1 for infectious disease, tuberculosis and chronic respiratory disease, and Despite the limitation of possibly capturing the more severe cases of sarcoidosis, this study may help define and prioritise preventive interventions.
Published: 2016

176. Prevalence and characteristics of TERT and TERC mutations in suspected genetic pulmonary fibrosis

Author: Lidwine Wemeau-Stervinou, Julie Mankikian, Patrick Revy, Martine Reynaud Gaubert, Christelle Ménard, Jacques Cadranel, Dominique Israel-Biet, Hervé Mal, Grégoire Prévot, Bruno Crestani, Caroline Kannengiesser, Hilario Nunes, Abdellatif Tazi, Dominique Valeyre, Gabriel Thabut, Vincent Cottin, Marion Reocreux, Raphael Borie, Clément Picard, Stéphane Jouneau, Bernard Grandchamp, Anne Bergeron Lafaurie, Laure Tabèze, Sylvain Marchand-Adam, Jean-François Cordier, Jean-Marc Naccache, Physiopathologie et Epidémiologie des Maladies Respiratoires (PHERE (UMR_S_1152 / U1152)), Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Pneumologie A, AP-HP - Hôpital Bichat - Claude Bernard [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Centre de compétence des maladies pulmonaires rares, Service de pneumologie B, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-AP-HP - Hôpital Bichat - Claude Bernard [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7), Service de médecine nucléaire [Bobigny], Université Paris 13 (UP13)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Avicenne [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre de Référence des Maladies Pulmonaires Rares [Hôpital Louis Pradel - HCL], Hôpital Louis Pradel [CHU - HCL], Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Department of Pneumology [Lyon], Hospices Civils de Lyon (HCL), Pathologies Respiratoires : Protéolyse et Aérosolthérapie, Université de Tours (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM), Hôpital Larrey [Toulouse], CHU Toulouse [Toulouse], Service de pneumologie [Saint-Louis], Université Paris Diderot - Paris 7 (UPD7)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de Pneumologie - Oncologie Thoracique - Maladies Pulmonaires Rares [CHU Tenon], CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Theranoscan, Université Pierre et Marie Curie - Paris 6 (UPMC), Service d'oncologie médicale (CHRU Lille), Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Service de Pneumologie, Paris, Etablissement Français du Sang - Alpes-Méditerranée (EFS - Alpes-Méditerranée), Etablissement Français du Sang, Anthropologie bio-culturelle, Droit, Ethique et Santé (ADES), Aix Marseille Université (AMU)-EFS ALPES MEDITERRANEE-Centre National de la Recherche Scientifique (CNRS), CHU Marseille, Institut de recherche en santé, environnement et travail (Irset), Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique )-Institut National de la Santé et de la Recherche Médicale (INSERM)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Université d'Angers (UA), Centre d'Investigation Clinique [Rennes] (CIC), Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Hôpital Pontchaillou-Institut National de la Santé et de la Recherche Médicale (INSERM), Microenvironnement et cancer (MiCa), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon, Service des maladies respiratoires, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Groupe Hospitalier Avicenne-Jean Verdier-René Muret, Hôpital Bichat - Claude Bernard, OMMIC S.A.S., OMMIC SAS, Developpement Normal et Pathologique du Système Immunitaire, Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Physiopathologie et Epidemiologie de l'Insuffisance Respiratoire, Département Hospitalo-universtaire FIRE (Fibrosis, Inflammation and Remodeling), LabEx Inflamex, Service de pneumologie A, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7)-Hôpital Bichat, Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Université d'Angers (UA)-Université de Rennes (UR)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Université de Rennes (UR)-Hôpital Pontchaillou-Institut National de la Santé et de la Recherche Médicale (INSERM), Université de Rennes (UR)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), OMMIC, Chard-Hutchinson, Xavier, Service de Pneumologie = Pneumologie - Oncologie Thoracique - Maladies Pulmonaires Rares [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Physiopathologie et Epidémiologie des Maladies Respiratoires, Université Paris Diderot - Paris 7 ( UPD7 ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), AP-HP - Hôpital Bichat - Claude Bernard [Paris]-Centre de compétence des maladies pulmonaires rares, Assistance publique - Hôpitaux de Paris (AP-HP)-AP-HP - Hôpital Bichat - Claude Bernard [Paris]-Université Paris Diderot - Paris 7 ( UPD7 ), Assistance publique - Hôpitaux de Paris (AP-HP)-Hôpital Avicenne-Université Paris 13 ( UP13 ), Hospices Civils de Lyon ( HCL ) -Hospices Civils de Lyon ( HCL ), Hospices Civils de Lyon ( HCL ), Université de Tours-Institut National de la Santé et de la Recherche Médicale ( INSERM ), CHU, Hôpital Larrey, Assistance publique - Hôpitaux de Paris (AP-HP)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris]-Université Paris Diderot - Paris 7 ( UPD7 ), Service de pneumologie et réanimation [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Tenon [APHP], Université Pierre et Marie Curie - Paris 6 ( UPMC ), Centre Hospitalier Régional Universitaire [Lille] ( CHRU Lille ), Etablissement Français du Sang - Alpes-Méditerranée ( EFS - Alpes-Méditerranée ), Anthropologie bio-culturelle, Droit, Ethique et Santé ( ADES ), Aix Marseille Université ( AMU ) -EFS ALPES MEDITERRANEE-Centre National de la Recherche Scientifique ( CNRS ), Institut de recherche, santé, environnement et travail ( Irset ), Université d'Angers ( UA ) -Université de Rennes 1 ( UR1 ), Université de Rennes ( UNIV-RENNES ) -Université de Rennes ( UNIV-RENNES ) -École des Hautes Études en Santé Publique [EHESP] ( EHESP ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ) -Université des Antilles ( UA ), Centre d'Investigation Clinique [Rennes] ( CIC ), Université de Rennes 1 ( UR1 ), Université de Rennes ( UNIV-RENNES ) -Université de Rennes ( UNIV-RENNES ) -Hôpital Pontchaillou-Institut National de la Santé et de la Recherche Médicale ( INSERM ), Microenvironnement et cancer ( MiCa ), Université de Rennes ( UNIV-RENNES ) -Université de Rennes ( UNIV-RENNES ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Université Claude Bernard Lyon 1 ( UCBL ), Assistance publique - Hôpitaux de Paris (AP-HP)-Groupe Hospitalier Avicenne-Jean Verdier-René Muret, Université Paris Descartes - Paris 5 ( UPD5 ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Centre National de la Recherche Scientifique ( CNRS ), Assistance publique - Hôpitaux de Paris (AP-HP)-Université Paris Diderot - Paris 7 ( UPD7 ) -Hôpital Bichat, Université Paris Diderot - Paris 7 (UPD7)-AP-HP - Hôpital Bichat - Claude Bernard [Paris], Université de Tours-Institut National de la Santé et de la Recherche Médicale (INSERM), Université d'Angers (UA)-Université de Rennes 1 (UR1), and Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique )
Subjects: Pulmonary and Respiratory Medicine, Adult, Male, Pathology, medicine.medical_specialty, [SDV]Life Sciences [q-bio], Macrocytosis, 030204 cardiovascular system & hematology, Gene mutation, medicine.disease_cause, Gastroenterology, [ SDV.EE ] Life Sciences [q-bio]/Ecology, environment, 03 medical and health sciences, Young Adult, 0302 clinical medicine, Internal medicine, Cause of Death, Pulmonary fibrosis, Telomerase rna, medicine, Humans, Interstitial pneumonia, Telomerase reverse transcriptase, Telomerase, Aged, Retrospective Studies, Aged, 80 and over, [SDV.EE]Life Sciences [q-bio]/Ecology, environment, Mutation, [ SDV ] Life Sciences [q-bio], business.industry, Middle Aged, Telomere, medicine.disease, Survival Analysis, Idiopathic Pulmonary Fibrosis, 3. Good health, [SDV] Life Sciences [q-bio], [SDV.EE] Life Sciences [q-bio]/Ecology, environment, Logistic Models, 030228 respiratory system, Multivariate Analysis, RNA, Female, France, business
Abstract: Telomerase reverse transcriptase (TERT) or telomerase RNA (TERC) gene mutation is a major monogenic cause of pulmonary fibrosis. Sequencing of TERT/TERC genes is proposed to patients with familial pulmonary fibrosis. Little is known about the possible predictors of this mutation and its impact on prognosis.We retrospectively analysed all the genetic diagnoses made between 2007–2014 in patients with pulmonary fibrosis. We evaluated the prevalence of TERT/TERC disease-associated variant (DAV), factors associated with a DAV, and the impact of the DAV on survival.237 patients with pulmonary fibrosis (153 with familial pulmonary fibrosis, 84 with telomere syndrome features without familial pulmonary fibrosis) were tested for TERT/TERC DAV. DAV was diagnosed in 40 patients (16.8%), including five with non-idiopathic interstitial pneumonia. Prevalence of TERT/TERC DAV did not significantly differ between patients with familial pulmonary fibrosis or with only telomere syndrome features (18.2% versus 16.4%). Young age, red blood cell macrocytosis, and low platelet count were associated with the presence of DAV; the probability of DAV was increased for patients 40–60 years. Transplant-free survival was lower with than without TERT/TERC DAV (4.2 versus 7.2 years; p=0.046).TERT/TERC DAV were associated with specific clinical and biological features and reduced transplant-free survival.
Published: 2016

177. Caractéristiques, devenir et réponse aux traitements chez les patients ayant une sarcoïdose réfractaire aux anti-TNF : analyse des données du registre STAT

Author: C. Thery-Casari, F. Bonnet, Philip Bielefeld, A. Marquet, Nicolas Schleinitz, Pascal Sève, Nicolas Girszyn, A.C. Chapelon, Dominique Valeyre, Diane Bouvry, Yvan Jamilloux, and Olivier Fain
Subjects: 030203 arthritis & rheumatology, 03 medical and health sciences, 0302 clinical medicine, Gastroenterology, Internal Medicine, 030212 general & internal medicine
Abstract: Introduction Les biotherapies anti-TNFα (infliximab et adalimumab, principalement) sont utilisees pour traiter les sarcoidoses severes et/ou refractaires. L’etude STAT a montre une reponse clinique dans 64 % des cas, principalement pour les atteintes neurologiques, cardiaques, cutanees, et ORL [1] . Il n’y a, cependant, pas de donnees concernant le profil et le devenir des patients non repondeurs aux anti-TNFα. Objectif Decrire les patients non repondeurs inclus dans le registre national francais des sarcoidoses traitees par anti-TNFα (STAT). Patients et methodes Etude de cohorte retrospective a partir d’un appel a observations national en juillet 2014. Etaient inclus les patients atteints de sarcoidose histologiquement prouvees et/ou de neurosarcoidose probable selon la classification de Zajicek consideres comme non-repondeurs aux anti-TNFα. Resultats Parmi 132 patients, 14 patients etaient identifies comme refractaires aux anti-TNFα. Cinq patients ont ete exclus (un deces, 3 donnees manquantes, 1 diagnostic final alternatif). L’âge median des 9 patients inclus, (6 femmes) etait de 48 ans (29–70) a l’initiation des anti-TNFα. La maladie atteignait en moyenne 4,2 organes et les patients avaient recu en moyenne 2 traitements immunosuppresseurs (IS) avant les anti-TNFα. Huit patients avaient recus de l’infliximab et un patient avait recu 3 anti-TNFα differents. Quatre patients recevaient un IS en meme temps que les anti-TNFα. La duree mediane de traitement anti-TNFα etait de 7 mois (3–24). Apres un suivi median de 35 mois (19–128) post-traitement anti-TNFα, 2 patients avaient finalement une reponse complete, 5 avaient une reponse partielle et 2 avait une maladie evolutive. Les patients recevaient en moyenne 2 nouvelles lignes de traitements IS. Les traitements de premiere ligne etaient le methotrexate (n = 5) et le cyclophosphamide (n = 2). Dans 6 cas, la reponse clinique etait obtenue avec un traitement IS recu anterieurement par le patient. Conclusion Dans les sarcoidoses refractaires, les immunosuppresseurs conservent leur utilite meme en cas d’echec des anti-TNFα.
Published: 2018

178. Incertitude diagnostique, diagnostic de travail et évolution des recommandations sur la fibrose pulmonaire

Author: Sylvain Marchand-Adam, G. Prévot, Vincent Cottin, Jacques Cadranel, Bruno Crestani, Benoit Wallaert, and Dominique Valeyre
Subjects: Pulmonary and Respiratory Medicine, medicine.medical_specialty, medicine.diagnostic_test, business.industry, MEDLINE, Computed tomography, Working diagnosis, medicine.disease, Clinical trial, Idiopathic pulmonary fibrosis, X ray computed, Pulmonary fibrosis, medicine, Tomography, Radiology, business
Published: 2018

179. Analyse génétique en Whole Exome Sequencing (WES) de 6 familles prédisposées à la sarcoïdose : une grande hétérogénéité génétique mais une focalisation fonctionnelle autour des voies de signalisation de l’autophagie

Author: Serge Lebecque, Carole Planès, Claire Bardel, A. Bentaher, Hilario Nunes, Valérie Besnard, D. Israel Biet, P.A. Rollat Farnier, Vincent Cottin, Pascal Roy, Dominique Valeyre, G. Devouassoux, Alain Calender, Adrien Buisson, and Yves Pacheco
Subjects: 0301 basic medicine, Pulmonary and Respiratory Medicine, 03 medical and health sciences, 0302 clinical medicine, 030105 genetics & heredity, 030217 neurology & neurosurgery
Abstract: Introduction La sarcoidose reste une maladie d’etiologie meconnue. Les approches explicatives sont actuellement environnementales et/ou genetiques. Les hypotheses fonctionnelles et biochimiques ont aborde les voies de signalisation : NFkB, STAT1, Galphai, RIP2, IRAK et plus recemment mTOR. L’etude nationale SARCFAM [1] a permis de collecter plus de 150 familles presentant une predisposition a la maladie. Methodes Au sein de cette cohorte, six familles reunissant au moins deux ou trois sujets malades et un ou deux temoins sains ont pu beneficier d’une etude en WES. Le sequencage a ete realise sur Illumina HiSEQ2500 en cooperation avec INTEGRAGEN et les donnees sources ont ete analysees par un pipeline informatique developpe au sein de l’equipe suivant les recommandations du Broad Institute ( https://www.broadinstitute.org/gatk/guide/best-practices ). Tous les cas et temoins ont ete sequences deux fois dans des protocoles independants et seuls les resultats reproductibles ont ete retenus. Une selection de variants geniques a ete confirmee par sequencage SANGER. Resultats Les resultats montrent : (a) que cette maladie presente une tres forte heterogeneite genetique, (b) que des variations genotypiques entre patients d’une meme famille sont susceptibles d’expliquer une expressivite variable de la maladie, (c) qu’il existe une forte concentration de mutations dans la sphere immunologique, (d) que ces mutations interessent les voies de signalisation et de regulation de l’autophagie autour des voies Rac1 et mTOR, (e) qu’il existe une concentration par famille de variants pathogenes in silico dans ces voies fonctionnelles, 3 a 8 pour Rac1, 2 a 6 pour mTOR et l’autophagie, suggerant une complementation des effets pathogenes a l’origine de la predisposition. Conclusion Ces observations conduisent a des hypotheses seduisantes sur un dysfonctionnement du processus d’autophagie rendant les malades plus vulnerables a divers facteurs de l’environnement. Elles permettent d’envisager des etudes fonctionnelles et des analyses genetiques sur une grande cohorte.
Published: 2018

180. Pneumopathies interstitielles diffuses (PID) fibrosantes prédominant aux lobes supérieurs : étiologies, caractéristiques et pronostic

Author: Pierre-Yves Brillet, Marianne Kambouchner, Lucile Sesé, Dominique Valeyre, L. Mourtada, Diane Bouvry, and Hilario Nunes
Subjects: Pulmonary and Respiratory Medicine
Abstract: Introduction Les causes de PID fibrosantes predominant aux lobes superieurs sont connues pour etre variees dans la litterature, mais elles sont mal decrites. Parmi elles, la fibroelastose pleuroparenchymateuse (FEPP) a ete recemment individualisee comme une entite bien definie. Notre etude avait pour objectif de determiner la prevalence des PID predominant aux lobes superieurs, la repartition de leurs etiologies, leurs caracteristiques et leur pronostic. Methodes Etude retrospective monocentrique basee sur une cohorte de 1132 patients ayant une PID chronique fibrosante adresses entre 2006 et 2016. Etaient exclus les cas de sarcoidose d’evolution fibrosante dont le diagnostic etait deja connu avant la prise en charge dans le service. Resultats Quatre-vingt-sept patients avaient une predominance aux lobes superieurs sur la tomodensitometrie (TDM) thoracique, soit une prevalence de 7,69 %. La gamme diagnostique apres une discussion multidisciplinaire etait la suivante : sarcoidose : n = 21, FEPP : n = 19, pneumopathie d’hypersensibilite (PHS) : n = 15, PID inclassable : n = 11, PID du fumeur : n = 6, silicose : n = 5 ; et autres diagnostics : n = 10. Les quatre groupes les plus prevalents ont ete compares (sarcoidose, FEPP, PHS, et PID inclassable). Les patients avec une FEPP avaient un antecedent familial de PID significativement plus frequent (7/19, 37 %), un IMC plus bas, la survenue plus frequente de pneumothorax et/ou de pneumomediastins (8/19, 42 %) et pas de fixation a la tomographie par emission de positrons. Sur la TDM, les masses de fibrose, les adenopathies et la predominance centrale etaient plus frequentes en cas de sarcoidose. Les FEPP avaient plus souvent une perte de volume, une asymetrie, des condensations sous pleurales et un thorax plus plat. Les lobules clairs distinguaient les PHS. Le declin annuel de la capacite vitale etait moins marque dans la sarcoidose. La survie de la FEPP etait significativement plus mauvaise que celle des autres groupes ( p = 0,02), estimee a 60 % a 5 ans, en particulier en presence d’une PID associee des bases. Conclusion Certaines caracteristiques cliniques et radiologiques aident a differencier les principales causes de PID fibrosantes predominant aux lobes superieurs. La frequence des formes familiales doit inciter a faire une recherche genetique chez les patients ayant une FEPP, dont le pronostic est sombre.
Published: 2018

181. EpiSarc : étude phénotypique des atteintes extrapulmonaires au cours de la sarcoïdose

Author: Fleur Cohen-Aubart, M. Hamidou, D. Launay, Isabella Annesi-Maesano, J. Haroche, M. Mahevas, Dominique Valeyre, Hilario Nunes, Nicolas Schleinitz, Raphael Borie, T. Papo, K. Sacre, Raphael Lhote, and Zahir Amoura
Subjects: Pulmonary and Respiratory Medicine
Abstract: Introduction Les atteintes extra thoraciques sont frequentes au cours de la sarcoidose (30 a 50 %). Si l’expression clinique dans chaque organe est decrite, on a peu de donnees sur l’association de ces atteintes entre elles. L’objectif etait de definir les atteintes extrapulmonaires de la sarcoidose ( Tableau 1 ). Methodes Il s’agissait d’une etude observationnelle transversale multicentrique. Les patients etaient selectionnes a partir des bases de donnees du programme de medicalisation des systemes d’informations (PMSI) entre janvier 2013 et decembre 2016. Les dossiers medicaux etaient revus pour valider les inclusions et determiner les caracteristiques des patients. L’analyse statistique comportait une partie descriptive et une classification ascendante hierarchique (analyse en cluster). Resultats Mille quatre vingt un patients etaient inclus, 508 hommes et 573 femmes d’âge moyen 43(±13) an, 27 % etaient nes ou d’origine afro-antillaise et 25,5 % d’Afrique du Nord. L’analyse en cluster montrait 5 phenotypes cliniques distincts associes a des differences genetiques, des differences d’expositions socioprofessionnelles et de charge therapeutique ( Tableau 1 ). Les phenotypes cliniques etaient : association d’un erytheme noueux et l’atteinte articulaire (cluster 1), presence d’un lupus pernio (cluster 2), association d’une atteinte du systeme nerveux central avec une uveite (cluster 3), association d’une atteinte hepato-splenique a des adenopathies extra-mediastinales (cluster 4) et l’association d’une atteinte pulmonaire potentiellement fibrosante a l’atteinte cardiaque. Les differences genetiques et socioprofessionnel entre cluster etaient definies par plus de non europeens dans les clusters 3 (62 %), 4 (81 %) et 5 (60 %) que dans les clusters 1 (43 %) et 2 (48 %) (p = 0,04) et plus d’ouvrier dans le cluster 3 (41 %) que dans les clusters 1 et 5 (35 % et 36 %) et que dans les clusters 2 et 4 (23 % et 26 %) (p Conclusion Cette etude confirme l’heterogeneite de la sarcoidose extrapulmonaire et met en evidence des phenotypes distincts de la pathologie a la fois sur la presentation clinique et les caracteristiques sociodemographiques et geographiques mais aussi sur les consequences therapeutiques.
Published: 2018

182. Correspondence for 'clinical epidemiology of familial sarcoidosis: A systematic literature review'

Author: Yves Pacheco, Dominique Valeyre, Dominique Israel-Biet, and Alain Calender
Subjects: Pulmonary and Respiratory Medicine, medicine.medical_specialty, Systematic review, business.industry, Epidemiology, Medicine, Sarcoidosis, Clinical epidemiology, business, medicine.disease, Intensive care medicine
Published: 2019

183. Computed tomography assessment of peripheral traction bronchiolectasis: impact of minimal intensity projection

Author: Kim Levand, Pierre-Yves Brillet, Marie-Laure Chabi, Marie-Pierre Debray, Dominique Israël-Biet, Dominique Valeyre, Antoine Khalil, Bruno Crestani, Samia Boussouar, Hilario Nunes, Philippe Grenier, Constance de Margerie-Mellon, Philippe Khafagy, Jean-Marc Naccache, Amira Benattia, Jacques Cadranel, and Lisa Belin
Subjects: Pulmonary and Respiratory Medicine, medicine.diagnostic_test, Download, business.industry, Conflict of interest, Cornerstone, Computed tomography, White paper, Traction, Nothing, Law, medicine, Humans, Tomography, X-Ray Computed, business, Production team
Abstract: The Fleischner Society White Paper and the ATS/ERS/JRS/ALAT guidelines recently redefined the computed tomography (CT) scanning patterns of usual interstitial pneumonia (UIP) [1,2]. Both publications confirmed honeycombing as the cornerstone of UIP pattern diagnosis, and introduced peripheral traction bronchiolectasis (PTB) as a key feature of the new “probable UIP” category. Therefore, improving the distinction between these two features may be critical, especially when the clinical likelihood of IPF is uncertain: a lung biopsy should be discussed for patients with PTB without honeycombing, but is not recommended for patients presenting with clear honeycombing and typical UIP pattern [2]. Footnotes This manuscript has recently been accepted for publication in the European Respiratory Journal . It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article. Conflict of interest: Dr. de Margerie-Mellon reports grants from Institut Servier, grants from Olea Medical, outside the submitted work. Conflict of interest: Dr. Belin has nothing to disclose. Conflict of interest: Dr. Boussouar has nothing to disclose. Conflict of interest: Dr. Khafagy has nothing to disclose. Conflict of interest: Dr. Debray reports personal fees and non-financial support from Boehringer-Ingelheim, personal fees and non-financial support from Roche, outside the submitted work. Conflict of interest: Dr. Levand has nothing to disclose. Conflict of interest: Dr. Chabi has nothing to disclose. Conflict of interest: Dr. KHALIL has nothing to disclose. Conflict of interest: Dr. Benattia has nothing to disclose. Conflict of interest: Dr. Israel-Biet has nothing to disclose. Conflict of interest: Dr. crestani reports personal fees and non-financial support from astra zeneca, grants, personal fees and non-financial support from boehringer ingelheim, grants from MedImmune, grants, personal fees and non-financial support from Roche, personal fees from Sanofi, outside the submitted work. Conflict of interest: Dr. Nunes reports grants and personal fees from Roche/Genentech, grants and personal fees from Boehringer Ingelheim, outside the submitted work. Conflict of interest: Dr. Cadranel has nothing to disclose. Conflict of interest: Dr. GRENIER has nothing to disclose. Conflict of interest: Dr. Valeyre reports personal fees from Boehringer ingelheim, personal fees from Roche, personal fees from Boehringer ingelheim Roche, outside the submitted work. Conflict of interest: Dr. Naccache has nothing to disclose. Conflict of interest: PYB has received personal fees for teaching actions from Boehringer Ingelheim and Roche.
Published: 2019

184. Apport de la discussion multidisciplinaire pour le diagnostic de sarcoïdose cardiaque

Author: Olivia Freynet, Michael Soussan, C. Meune, Dominique Valeyre, Pierre-Yves Brillet, S. Chauveau, Diane Bouvry, Hilario Nunes, and F.X. Goudot
Subjects: Pulmonary and Respiratory Medicine
Abstract: Introduction Le diagnostic de sarcoidose cardiaque est difficile et crucial car il engage le pronostic vital. Plusieurs societes savantes ont propose des criteres diagnostiques s’appuyant sur des cohortes heterogenes, notamment le Japanese Ministry of Health and Welfare (JMWH) et l’Heart and Rythm Society (HRS). Le but etait d’evaluer l’apport d’une discussion multidisciplinaire (DMD) pour la mise en evidence de l’atteinte cardiaque par rapport aux criteres diagnostiques JMWH et HRS, de determiner la performance diagnostique des differents examens et d’analyser les facteurs predictifs d’evenements cardiaques severes (ECS). Methodes Entre mars 2015 et fevrier 2018, une DMD a confronte les donnees cliniques, electrocardiographiques (ECG), du Holter ECG, de l’echographie cardiaque, de l’imagerie par resonnance magnetique cardiaque (IRMc) et de la tomographie a emission de positons (TEP-scanner) dediee chez tous les patients consecutifs ayant une suspicion de sarcoidose cardiaque suivis dans le service de pneumologie de l’hopital Avicenne. Resultats La DMD a retenu une atteinte cardiaque chez 26 des 105 patients discutes (24,7 %) contre 8 patients (7,6 %) et 40 patients (38 %) en appliquant les criteres JMWH et HRS, respectivement. Des investigations complementaires de controle ou necessaires a l’exclusion d’un diagnostic differentiel ont ete demandees dans 18 cas. La presence de symptomes cliniques et/ou electriques et/ou echographiques evocateurs d’une atteinte specifique avait une sensibilite (Se) de 65 % et une specificite (Sp) de 36 %. La Se et la Sp de l’IRMc etaient de 92 % et 86 %, et de 40 % et 94 % pour le TEP scanner. Au terme d’un suivi de 23,4 ± 1,6 mois, 13 patients ont presente des evenements cardiaques severes. Les facteurs predictifs de leur survenue en analyse multivariee etaient l’atteinte cardiaque retenue en DMD (HR = 17 [1,61–187]) et celle selon les criteres JMWH (HR = 3,77 [1,093–13,01]). Dans les cas de sarcoidose cardiaque confirmee, l’existence d’une fixation myocardique au TEP-scanner etait correlee aux evenements cardiaques severes. (p Conclusion Le diagnostic de sarcoidose cardiaque pose en DMD, plus precis que celui base sur les criteres d’une societe cardiologique (HRS 2014), est plus associe aux evenements cardiaques severes. Cette etude souligne la necessite d’adapter l’algorithme diagnostique a la population pneumologique et le role d’une approche multi-modale combinant IRMc et TEP-scanner lors d’une DMD.
Published: 2019

185. Infections pulmonaires à mycobactéries non tuberculeuses sans facteur favorisant

Author: Jacinta Bustamante, P. Devilliers, Dominique Valeyre, E. Cardot-Martin, Jérémie Rosain, Elisabeth Rivaud, Pierre Cahen, G. Bonan, Stéphanie Boisson-Dupuis, Louis-Jean Couderc, Capucine Picard, Camille Bron, Hélène Salvator, Colas Tcherakian, Emilie Catherinot, G. Le Bourdelles, Jean-Laurent Casanova, F. Pourtout, Eric Farfour, and Evelyne Balloul
Subjects: Pulmonary and Respiratory Medicine
Abstract: Introduction Les mycobacteries non tuberculeuses (MNT), environnementales et peu virulentes, peuvent etre responsables d’infection pulmonaire. Aucun facteur de risque (FDR) n’est retrouve chez 30 % des patients, notamment chez les femmes apres 60 ans . Ces patients sans FDR presentent une grande homogeneite phenotypique : plus grands et plus minces, plus grande frequence de scoliose, pectus excavatum et prolapsus valvulaire mitral faisant suspecter une maladie genetique multisystemique. Methodes Les patients avec infection pulmonaire a MNT selon les criteres de l’ATS sans FDR vu a l’hopital Foch et l’hopital Avicenne entre 2000 et 2016 ont ete inclus. Deux controles matches sur l’âge ont ete recrutes a l’hopital Foch. Analyse retrospective du dossier medical. Approbation du CEPRO. Les patients ont ete inclus dans l’etude C.10.16. du Laboratoire de genetique humaine des maladies infectieuses (Necker) approuvee par le CPP pour l’exploration immunologique. Resultats Cinquante patients ont ete inclus (âge median 65 ans, femmes 90 %) (Tableau 1). Pattern micronodules et bronchectasies : 44 (40 femmes). Microbiologie : complexe aviaire = 42, M. kansasii = 6, Complexe abscessus = 5, M. xenopi = 3, autres = 2. Caracteristiques morphologiques des patients et controles : Tableau 1. Dosage des immunoglobulines : 13 patients (27,6 %) ont une diminution d’au moins un sous-type. IgG Conclusion Nos patients presentent le morphotype classiquement associe aux infections pulmonaires a MNT sans FDR. Ils ont une production diminuee d’IFN-gamma apres stimulation sans anomalie retrouvee dans les genes connus du MSMD. Une proportion importante presente une diminution de la production d’anticorps et un taux diminue de lymphocytes B memoires. Ceci peut refleter un deficit immunitaire plus large qu’anterieurement suspecte. L’analyse de l’exome est en cours.
Published: 2019

186. Recommandations pratiques pour le diagnostic et la prise en charge de la fibrose pulmonaire idiopathique. Élaborées par le centre national de référence et les centres de compétence pour les maladies pulmonaires rares sous l’égide de la Société de pneumologie de langue française

Author: Philippe Delaval, Philippe Carré, B. Philippe, S. Kouzan, Jacques Cadranel, Benoit Wallaert, N. Just, Dominique Israel-Biet, Dominique Valeyre, Jean-François Cordier, François Lebargy, B. Bouquillon, Bruno Crestani, Vincent Cottin, Martine Reynaud-Gaubert, J.-C. Dalphin, G. Prévot, S. Marchand Adam, G. Ferreti, B. Stach, Bernard Aguilaniu, Jean-Baptiste Faivre, Claire Danel, Ronald C. Kessler, Françoise Thivolet-Béjui, Centre de Référence des Maladies Pulmonaires Rares [Hôpital Louis Pradel - HCL], Hôpital Louis Pradel [CHU - HCL], Hospices Civils de Lyon ( HCL ) -Hospices Civils de Lyon ( HCL ), Department of Pneumology [Lyon], Hospices Civils de Lyon ( HCL ), Physiopathologie et Epidemiologie de l'Insuffisance Respiratoire, Université Paris Diderot - Paris 7 ( UPD7 ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Service de Pneumologie A, AP-HP - Hôpital Bichat - Claude Bernard [Paris]-Centre de compétence des maladies pulmonaires rares, Département Hospitalo-universtaire FIRE (Fibrosis, Inflammation and Remodeling), LabEx Inflamex, Service de pneumologie A, Assistance publique - Hôpitaux de Paris (AP-HP)-Université Paris Diderot - Paris 7 ( UPD7 ) -Hôpital Bichat, Service des maladies respiratoires, Assistance publique - Hôpitaux de Paris (AP-HP)-Groupe Hospitalier Avicenne-Jean Verdier-René Muret, Service de Pneumologie et Immuno-Allergologie, Centre Hospitalier Régional Universitaire [Lille] ( CHRU Lille ), Intergroupe Francophone de Cancérologie Thoracique [Paris] ( IFCT ), Intergroupe Francophone de Cancérologie thoracique, Service de pneumologie et réanimation [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Tenon [APHP], Hôpital Jean Minjoz, Institut de recherche, santé, environnement et travail ( Irset ), Université d'Angers ( UA ) -Université de Rennes 1 ( UR1 ), Université de Rennes ( UNIV-RENNES ) -Université de Rennes ( UNIV-RENNES ) -École des Hautes Études en Santé Publique [EHESP] ( EHESP ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ) -Université des Antilles ( UA ), Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Service de Pneumologie, Paris, Nouvel Hôpital Civil Strasbourg, Service de chirurgie thoracique, Assistance Publique - Hôpitaux de Marseille ( APHM ) -Hôpital nord, Department of Physical Education, McGill University-Université de Montréal, HYLAB : Physiologie de l'exercice et interprétation clinique, Clinique du Mail, Laboratoire Chrono-environnement ( LCE ), Université Bourgogne Franche-Comté ( UBFC ) -Université de Franche-Comté ( UFC ) -Centre National de la Recherche Scientifique ( CNRS ), Centre national référent cancers rares - mésothéliomes malins pleuraux et tumeurs péritonéales rares ( MESOPATH ), CHU Caen-Hôpital côte de nacre, Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Hospices Civils de Lyon (HCL), Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM), AP-HP - Hôpital Bichat - Claude Bernard [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Centre de compétence des maladies pulmonaires rares, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7)-Hôpital Bichat, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Groupe Hospitalier Avicenne-Jean Verdier-René Muret, Service de Pneumologie et Immuno-Allergologie [CHU LIlle], Pole Cardio-vasculaire et pulmonaire [CHU Lille], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Intergroupe Francophone de Cancérologie Thoracique [Paris] (IFCT), Service de Pneumologie - Oncologie Thoracique - Maladies Pulmonaires Rares [CHU Tenon], CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), Institut de recherche en santé, environnement et travail (Irset), Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique )-Institut National de la Santé et de la Recherche Médicale (INSERM)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Université d'Angers (UA), Assistance Publique - Hôpitaux de Marseille (APHM)-Hôpital nord, McGill University = Université McGill [Montréal, Canada], Laboratoire Chrono-environnement - CNRS - UBFC (UMR 6249) (LCE), Université de Franche-Comté (UFC), Université Bourgogne Franche-Comté [COMUE] (UBFC)-Université Bourgogne Franche-Comté [COMUE] (UBFC)-Centre National de la Recherche Scientifique (CNRS), Registre Multicentrique à Vocation Nationale des Mésothéliomes Pleuraux (MESONAT), CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Laboratoire Chrono-environnement (UMR 6249) (LCE), Centre National de la Recherche Scientifique (CNRS)-Université de Franche-Comté (UFC), Université Bourgogne Franche-Comté [COMUE] (UBFC)-Université Bourgogne Franche-Comté [COMUE] (UBFC), Université d'Angers (UA)-Université de Rennes (UR)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E)-Assistance Publique - Hôpitaux de Marseille (APHM), and Centre Hospitalier René Dubos [Pontoise]
Subjects: Pulmonary and Respiratory Medicine, Patient care team, [ SDV ] Life Sciences [q-bio], business.industry, [SDV]Life Sciences [q-bio], 3. Good health, 03 medical and health sciences, 0302 clinical medicine, 030228 respiratory system, Medicine, Interdisciplinary communication, 030212 general & internal medicine, business, Humanities
Abstract: a Inra, UMR754, IFR 128, centre national de reference des maladies pulmonaires rares, centre de competences de l’hypertension pulmonaire, service de pneumologie, universite de Lyon, universite Claude-Bernard Lyon 1, hospices civils de Lyon, hopital Louis-Pradel, 28, rue du Doyen-Lepine, 69000 Lyon, France b CHU Bichat, centre de competences pour les maladies pulmonaires rares, 46, rue Henri-Huchard, 75877 Paris cedex 18, France
Published: 2013

187. Analyse phénotypique des atteintes extrapulmonaires de la sarcoïdose : données hospitalières multicentriques du réseau GSF

Author: M. Hamidou, Karim Sacre, Dominique Valeyre, Matthieu Mahevas, F. Cohen Aubart, Z. Amoura, Groupe sarcoïdose francophone, Raphael Lhote, Hilario Nunes, Isabella Annesi-Maesano, D. Launay, Nicolas Schleinitz, and Raphael Borie
Subjects: Gastroenterology, Internal Medicine
Abstract: Introduction La sarcoidose est une granulomatose multisystemique de cause inconnue caracterisee par une atteinte mediastino-pulmonaire frequente (90 % des cas). Les atteintes extrapulmonaires s’observent chez 30 a 50 % des patients. Si l’expression clinique de la sarcoidose dans chaque organe a ete bien decrite, on a peu de donnees sur l’association de ces atteintes entre elles. L’objectif etait donc de determiner si les atteintes extrapulmonaires de la sarcoidose s’associent selon des phenotypes differents. Patients et methodes Il s’agissait d’une etude observationnelle transversale multicentrique. Les patients etaient selectionnes a partir des bases de donnees du programme de medicalisation des systemes d’information (PMSI) entre janvier 2013 et decembre 2016. Les dossiers medicaux etaient revus pour valider les inclusions et determiner les caracteristiques des patients. Les criteres d’inclusion etaient : une sarcoidose avec documentation histologique et au moins une atteinte extrapulmonaire, ou un syndrome de Lofgren. L’analyse statistique comportait une partie descriptive et une classification ascendante hierarchique (analyse en cluster) Resultats Huit centres du reseau GSF ont participe permettant l’inclusion de 1081 patients, 508 hommes et 573 femmes d’âge moyen 43 (±13) ans. Parmi eux, 27 % etaient nes ou d’origine afro-antillaise et 25,5 % d’Afrique du Nord. L’analyse en cluster permettait d’identifier 5 phenotypes associes a des differences de sex-ratio (p Conclusion Cette etude multicentrique de donnees hospitalieres obtenues grâce au reseau GSF a permis de demontrer l’existence de phenotypes distincts de la sarcoidose. Cette etude permet de demontrer que la distribution des atteintes ne se fait pas au hasard au cours de la sarcoidose. Elle soutient fortement l’hypothese de la diffusion des granulomes par contiguite. Enfin, elle permet de demontrer que le sexe et l’origine geographique jouent un role dans le phenotype de la sarcoidose et que les phenotypes ont des pronostics variables (en confirmant notamment l’impact negatif du lupus pernio et le bon pronostic du syndrome de Lofgren qui est redecouvert ici comme un phenotype distinct de la sarcoidose 65 ans apres sa premiere description). En conclusion, la sarcoidose est une maladie heterogene dont les atteintes extrapulmonaires se distribuent selon des phenotypes distincts.
Published: 2017

188. Sarcoïdose du sujet âgé : étude rétrospective de 78 cas

Author: Diane Bouvry, Catherine Chapelon-Abric, Patrice Cacoub, P. Garrigues, Hilario Nunes, Dominique Valeyre, and F. Cohen
Subjects: 03 medical and health sciences, 0302 clinical medicine, Gastroenterology, Internal Medicine, 030212 general & internal medicine
Abstract: Introduction La sarcoidose (S) est une granulomatose rare, multisystemique d’etiologie inconnue qui debute dans 70 % des cas entre 25 et 45 ans. Elle est tres rare avant 15 ans et apres 70 ans. Les objectifs de ce travail sont de decrire les particularites cliniques, therapeutiques et evolutives de la sarcoidose du sujet âge. Patients et methodes Analyse retrospective, multicentrique de 78 cas de S du sujet âge, la limite de 6 ans repondant a la definition de l’OMS du sujet âge. Resultats La population est caucasienne dans 81 %. La sex-ratio femme/homme est de 1,51. L’âge du diagnostic de S se situe entre 65 et 74 ans dans 76 % des cas, entre 75 et 84 ans dans 20 % et 4 % ont 85 ans ou plus. Dans pres de 50 % des cas, il existait une ou plusieurs maladies associees (HTA, diabete, neoplasie…). Le delai diagnostic est inferieur a 1 an dans 81 %. Une asthenie, un amaigrissement et/ou des signes respiratoires ont ete revelateurs respectivement dans 56 %, 15 % et 27 % des cas. Le nombre d’organes atteints n’excedait pas 3 dans 86 %. Les localisations preferentielles sont mediastino-pulmonaires (51 %) et oculaires (31 %). Des atteintes cutanees, ganglionnaires, neuromusculaires, osseuses et cardiaques ont ete notees dans moins de 10 % des cas. Une preuve histologique a ete obtenue dans 82 % des cas (nombre de sites biopsies variant de 1 a 4). Lors de la premiere poussee, seuls 25 patients (32 %) ont ete traites, soit en monotherapie (12/32) (prednisone [12], methotrexate [3], plaquenil [1], cyclophosphamide [1], clarithromycine [1]) soit en bitherapie (8/32) : (prednisone + methotrexate [6] ou azathioprine [1]). Une ou plusieurs rechutes ont ete notees dans 44 % des cas (jusqu’a 8). Celles-ci ont entraine des adaptations therapeutiques aboutissant a un traitement immunosuppresseur chez 63 patients (81 %) soit en monotherapie (33/63), soit en bitherapie voire en tritherapie immunomodulatrice (30/63). La duree moyenne du suivi est de 69 mois (12 a 204 mois). Au terme de ce suivi, 49 patients sont traites (63 %) : 37 sont sous monotherapie immunosuppressive (prednisone : 29 ; methotrexate : 1, plaquenil : 2, clarithromycine : 2) et 12 recoivent une association prednisone plus methotrexate (5), azathioprine (1), cyclophosphamide (1), mycophenolate mofetil (2), anti-TNFα (1), plaquenil (2). Une evolution favorable sans traitement n’a ete notee que dans 19 % des cas. L’evolution lors de la derniere visite est marquee par une remission avec ou sans sequelles dans 87 %, une maladie active dans 13 % des cas. Deux patients sont decedes, seul un deces est lie a la sarcoidose. Des effets secondaires ou comorbidites ont ete notes chez 30 % des patients, dont 14 etaient indemnes avant la prise en charge de la S, principalement de l’hypertension arterielle et/ou une fibrillation auriculaire (10 cas), de l’osteoporose (4 cas) un diabete non insulino-requerant (2 cas), cataracte (2 cas) et des cancers (4). Conclusion La sarcoidose du sujet âge est rare et par consequent peu evoquee dans les services de medecine interne ou de geriatrie. Les signes cliniques ne different pas de ceux du sujet jeune, en dehors de signes generaux plus marques. Meme si le diagnostic est evoque, il faut eliminer les diagnostics differentiels habituels. Les investigations et l’approche therapeutique doivent etre superposables a celle du sujet jeune, compte tenu du taux faible d’evolution spontanement favorable.
Published: 2017

189. Elderly-Onset Sarcoidosis: Prevalence, Clinical Course, and Treatment

Author: Dominique Valeyre, Marc Bonnefoy, Christiane Broussolle, Yvan Jamilloux, Pascal Sève, and L. Varron
Subjects: medicine.medical_specialty, Tuberculosis, Sarcoidosis, Disease-Free Survival, Diagnosis, Differential, Pharmacotherapy, Adrenal Cortex Hormones, Epidemiology, Prevalence, medicine, Humans, Pharmacology (medical), Pathological, Aged, Aged, 80 and over, Erythema nodosum, Tumor Necrosis Factor-alpha, business.industry, Incidence (epidemiology), Age Factors, medicine.disease, Dermatology, Surgery, Practice Guidelines as Topic, Geriatrics and Gerontology, Granulomatosis with polyangiitis, business, Immunosuppressive Agents
Abstract: Sarcoidosis is a systemic disorder of unknown cause characterized by its pathological hallmark, the non-caseating granulomas, and by variable clinical course. While most of the cases affect people aged between 25 and 40 years, approximately 30 % of cases occur in older patients. Elderly-onset sarcoidosis (EOS) is defined as the onset of sarcoidosis in people over 65 years of age. Specific studies on the incidence and prevalence of sarcoidosis in this subgroup are scarce. Several studies suggest that the clinical features of EOS differ from those of sarcoidosis in younger patients. Compared with younger patients, fatigue, uveitis and specific skin lesions are more common, while erythema nodosum and chest x-ray abnormalities are less frequent. The diagnosis of EOS is challenging and may be delayed for many months because of its insidious onset, low prevalence and similarity to other more common disorders. When there is a granulomatous reaction in the elderly, clinicians should doubt the diagnosis and first think of tuberculosis, neoplasia or rare settings such as granulomatosis with polyangiitis or granulomatous reaction due to interferon and tumour necrosis factor-α (TNFα) blockers. A minor salivary gland biopsy also has a higher accuracy for diagnosis in the elderly. The current management of EOS remains empiric because of the lack of randomized, controlled studies. However, the approach to treatment is similar, regardless of the age of the patient. The treatment may be complicated by co-morbidities and increased risk of toxicities from usual treatments, particularly steroids. This review discusses the epidemiology, clinical course, prognosis and treatment of EOS.
Published: 2013

190. Quels rôles les anomalies de la voie des nucléotides cycliques et des protéines G jouent-elles dans le désordre biochimique des lymphocytes T dans la sarcoïdose ?

Author: Serge Lebecque, Yves Pacheco, Alain Calender, and Dominique Valeyre
Subjects: Pulmonary and Respiratory Medicine, MAPK/ERK pathway, G protein, T lymphocyte, Biology, medicine.disease, Molecular biology, GTP-binding protein regulators, biology.protein, medicine, STAT1, Sarcoidosis, Signal transduction, Protein kinase A
Abstract: CD4+ T lymphocytes play a major role in the pathophysiology of sarcoidosis. Many studies have investigated the immunological and genetic abnormalities in this disease. There are few studies concerning the metabolic pathways. Essentially they concern the pathways: STAT1, MAPK38, NF-κB, Galphai, cAMP and cGMP PDE and PEMT1. Using studies in the literature and results of our own work concerning some metabolic aspects of T lymphocytes in sarcoidosis, we present a revue of the various hypotheses, which involve dysfunction of cAMP signaling pathways, such as RAS/RAF/MEK/ERK in T lymphocytes, leading to a disorder of immunity.
Published: 2013

191. 18F-fluorodeoxyglucose positron emission tomography/computer tomography as an objective tool for assessing disease activity in Sjögren's syndrome

Author: Véronique Eder, Anne-Laure Fauchais, Michael Soussan, Hilario Nunes, G. Pop, Olivier Fain, A. Mekinian, Camille Cohen, Pierre-Yves Brillet, Yurdagul Uzunhan, Dominique Valeyre, Robin Dhote, Service de Médecine interne A et polyclinique médicale [CHU Limoges], CHU Limoges, Homéostasie Cellulaire et Pathologies (HCP), Université de Limoges (UNILIM)-CHU Limoges-Génomique, Environnement, Immunité, Santé, Thérapeutique (GEIST FR CNRS 3503), Laboratoire de physiopathologie de la paroi artérielle, Université de Tours-Institut National de la Santé et de la Recherche Médicale (INSERM), Réponses Cellulaires et Fonctionnelles à l'Hypoxie (LRPH), Université Paris 13 (UP13)-Université Sorbonne Paris Cité (USPC)-UFR SMBH, Service de Pneumologie, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Avicenne [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de médecine interne, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Jean Verdier [AP-HP], and Université de Tours (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM)
Subjects: Male, Immunology, Amino Acyl-tRNA Synthetases, Arthritis, Rheumatoid, 03 medical and health sciences, 0302 clinical medicine, Fluorodeoxyglucose F18, medicine, Humans, Lupus Erythematosus, Systemic, Immunology and Allergy, Lymph node, Aged, Autoantibodies, Retrospective Studies, 030304 developmental biology, 030203 arthritis & rheumatology, 0303 health sciences, Scleroderma, Systemic, Lupus erythematosus, medicine.diagnostic_test, business.industry, Beta-2 microglobulin, Thyroid, Interstitial lung disease, Middle Aged, medicine.disease, Cryoglobulinemia, 3. Good health, Lymphoma, Sjogren's Syndrome, medicine.anatomical_structure, Positron emission tomography, Positron-Emission Tomography, Female, Tomography, X-Ray Computed, Nuclear medicine, business, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
Abstract: Objective This study aims to determine the value of FDG-PET/CT to assess disease activity in patients with Sjogren's syndrome (SS). Methods Thirty-two patients with SS who underwent PET/CT were retrospectively analyzed. PET/CT activity score was measured using a 6-point scale including the 6 following items (0/1: absence or presence of an item): lymphadenopathy on CT, high-resolution CT (HRCT) evidence of interstitial lung disease (ILD), parotid glands SUVmax > 3, submandibular glands SUVmax > 3, lymph node uptake, ILD uptake. Combined PET/CT score was correlated to ESSDAI (EULAR Sjogren's Syndrome Disease Activity Index) score and other parameters of SS activity. Results Pathological FDG uptake was observed in 75% of patients (24/32): lymph-nodes (n = 19, 60%), salivary glands (n = 17, 53%), lungs (n = 9, 28%), and thyroid (n = 2). Median ESSDAI and PET/CT activity scores were 9.5 [5–12] and 2 [0–3], respectively. PET/CT activity score correlated with ESSDAI (r = 0.49, p = 0.005), unlike SUVmax. Patients with a high ESSDAI score had a higher PET/CT activity score than patients with a low ESSDAI score (3 vs 1, p = 0.004). PET was also correlated with gammaglobulin levels (r = 0.43, p = 0.02), but not with the presence of cryoglobulinemia, activated complement or beta-2 microglobulin levels. The FDG uptake in patients with lymphoma (n = 4) was higher than in patients without lymphoma (SUVmax = 5.4 vs. 3.2, p = 0.05). Conclusion We described a new PET/CT activity score, which correlates to ESSDAI and could help to assess disease activity in SS patients. PET can also help in the diagnosis of lymphoma, even if inflammatory lymph nodes can be frequently observed in SS patients.
Published: 2013

192. Actualités dans la prise en charge de la fibrose pulmonaire idiopathique

Author: G. Prévot, Julie Traclet, Vincent Cottin, Bruno Crestani, Dominique Valeyre, and Raphael Borie
Subjects: Pulmonary and Respiratory Medicine, business.industry, Medicine, business
Published: 2013

193. Efficacy and safety of tumor necrosis factor antagonists in refractory sarcoidosis: A multicenter study of 132 patients

Author: Marc André, Boris Bienvenu, Patrice Cacoub, Nicolas Noel, Zahir Amoura, Fleur Cohen-Aubart, Alicia Marquet, Delphine Maucort-Boulch, Laurent Perard, Catherine Chapelon-Abric, Laurence Bouillet, Françoise Sarrot-Reynauld, Bahram Bodaghi, Pascal Sève, Yvan Jamilloux, Jean Iwaz, Alban Deroux, Alice Proux, Philip Bielefeld, Sébastien Abad, Sandra Vukusic, Christiane Broussolle, Dominique Valeyre, David Saadoun, and Diane Bouvry
Subjects: Adult, Male, medicine.medical_specialty, Adolescent, Sarcoidosis, Gastroenterology, 03 medical and health sciences, Young Adult, 0302 clinical medicine, Rheumatology, Refractory, Prednisone, Internal medicine, medicine, Humans, Adverse effect, Aged, Retrospective Studies, 030203 arthritis & rheumatology, business.industry, Tumor Necrosis Factor-alpha, Middle Aged, medicine.disease, Infliximab, Surgery, Anesthesiology and Pain Medicine, medicine.anatomical_structure, Treatment Outcome, 030228 respiratory system, Multicenter study, Tumor necrosis factor alpha, Female, business, Immunosuppressive Agents, medicine.drug, Respiratory tract
Abstract: The off-label use of TNF antagonists in refractory sarcoidosis is increasingly reported but data on their efficacy and safety are still insufficient.To report on efficacy and safety of TNF antagonists in severe and refractory sarcoidosis.Examination of retrospective demographic, clinical, therapeutic, and adverse event data on 132 sarcoidosis patients (58% women; mean (min-max) age = 45.5 (14-78) years) given TNF antagonists (mainly infliximab, 91%) and investigation of response-linked factors.The overall clinical response (complete and partial) rate was 64%. TNF-antagonist efficacy (i.e., significant decrease of the ePOST score) was noted in cases with neurologic, heart, skin, and upper respiratory tract involvements. No significant difference in efficacy was found between anti-TNF used alone and TNF with immunosuppressant. The use of anti-TNF allowed reducing prednisone dosage at end of follow-up (p0.001). Adverse events were observed in 52% of the patients; they included infections (36%) and allergic reactions (8%) and required treatment interruption in 31 cases (23%). When TNF antagonists were interrupted, 13 patients experienced relapses within 14 months on average (median follow-up: 20.5 months).TNF antagonists were efficacious in about two-thirds of patients with severe/refractory sarcoidosis but their use led to a high rate of adverse events.
Published: 2016

194. Pleuroparenchymal fibroelastosis associated with telomerase reverse transcriptase mutations

Author: Christelle Ménard, Hilario Nunes, Florence Jeny, Pierre-Yves Brillet, Caroline Kannengiesser, Clément Picard, Jean-François Bernaudin, Dominique Valeyre, Diane Bouvry, and Marianne Kambouchner
Subjects: Pulmonary and Respiratory Medicine, Mutation, Telomerase, Lung, business.industry, medicine.disease, medicine.disease_cause, Article, Idiopathic pulmonary fibrosis, 03 medical and health sciences, Text mining, medicine.anatomical_structure, 0302 clinical medicine, 030228 respiratory system, 030220 oncology & carcinogenesis, Mutation (genetic algorithm), Cancer research, Medicine, Telomerase reverse transcriptase, 030212 general & internal medicine, business, Gene
Abstract: A diagnosis of PPFE is linked to pathogenic variants in three different telomere-related genes http://ow.ly/eGOq30aPkgx
Published: 2016

195. Safety and efficacy of pirfenidone in patients with lung fibrosis and TERT mutation

Author: Vincent Cottin, Bruno Crestani, Sylvain Marchand Adam, Emmanuel Bergot, Jean Marc Naccache, Anas Medhaoui, Jacques Cadranel, I. Frachon, Eline Magois, Anne Gondouin, Dominique Valeyre, Caroline Kanengiesser, Aurélien Justet, Hilario Nunes, Bernard Grandchamp, Jean-François Cordier, Lidwine Wemeau-Stervineau, and Raphael Borie
Subjects: medicine.medical_specialty, education.field_of_study, Exacerbation, business.industry, Mortality rate, Population, Pirfenidone, 030204 cardiovascular system & hematology, medicine.disease, Gastroenterology, Surgery, 03 medical and health sciences, FEV1/FVC ratio, Idiopathic pulmonary fibrosis, 0302 clinical medicine, 030228 respiratory system, DLCO, Internal medicine, Pulmonary fibrosis, Medicine, business, education, medicine.drug
Abstract: Background Mutations within TERT and TERC are found in 15% of familial pulmonary fibrosis and are associated with hematologic, liver and dermatological disorders. Pirfenidone has been shown to limit FVC decline and to decrease 12 months mortality rate in idiopathic pulmonary fibrosis (IPF). To our knowledge, pirfenidone has not been specifically evaluated in carriers of TERT / TERC mutations. Objectives The aim of this multicenter retrospective French study was to evaluate the safety and efficacy of pirfenidone in carriers of TERT mutations. Results We identified 18 patients (11 men) with a TERT mutation who received pirfenidone: 16 IPF, 1 pneumoconiosis and 1 rheumatoid arthritis-associated ILD. At diagnosis, mean age was 59 ± 6 years, mean FVC was 78.0% ± 16.4 % and mean DLCO was 52.4± 10.2%. The median duration of treatment was 304 days [14 -791]. Gastro intestinal intolerance was observed in 6 patients (33%). One patient showed a transient thrombocytopenia. Pirfenidone was discontinued in 10 patients (55%) due to disease progression (n=5, 28%), exacerbation (n=2, 11%), increased liver enzymes (n=2, 11%) or gastro intestinal intolerance (n=1, 5%). The median FVC decline was 32.6 mL [0-165] per month before pirfenidone initiation versus 35.7 mL [5-166] per month after treatment initiation; 7 patients showed an FVC decline >10% during treatment. Conclusion These preliminary data suggest that pirfenidone is safe in patients with TERT mutation. Disease progression is frequent in this population and does not seem to be influenced by pirfenidone.
Published: 2016

196. Experimental models of sarcoidosis

Author: Yves Pacheco, Valérie Besnard, Jean-François Bernaudin, Dominique Valeyre, and Florence Jeny
Subjects: 0301 basic medicine, Pulmonary and Respiratory Medicine, Sarcoidosis, business.industry, Systems biology, Experimental Animal Models, Disease, Computational biology, In Vitro Techniques, medicine.disease, Models, Biological, Clinical trial, 03 medical and health sciences, Disease Models, Animal, 030104 developmental biology, 0302 clinical medicine, Genetic predisposition, Medicine, Animals, Humans, Systemic approach, business, 030215 immunology
Abstract: Purpose of review Sarcoidosis is a disease caused by a complex combination of genetic susceptibility, immune networks and infectious and/or environmental agents. The onset and phenotypic variability of sarcoidosis remain poorly elucidated, not only due to the lack of clearly identified causes, but also because it is widely considered that no reliable model of this disease is available. In this review, we discuss the various models of granulomatous diseases in order to challenge this assertion. Recent findings A large number of models of granulomatous diseases are available, both cellular models used to study the natural history of granulomas and experimental animal models mostly developed in rodents. Summary Although none of the available models fully reproduces sarcoidosis, most of them generate various data supporting key concepts. Selected models with a high level of confidence among those already published may provide various pieces of the sarcoidosis jigsaw puzzle, whereas clinical data can provide other elements. A 'systems biology' approach for modelling may be a way of piecing together the various pieces of the puzzle. Finally, experimental models and a systemic approach should be considered to be tools for preclinical evaluation of the efficacy of drugs prior to testing in clinical trials.
Published: 2016

197. FDG-PET/CT in the prediction of pulmonary function improvement in nonspecific interstitial pneumonia. A Pilot Study

Author: Olivier Fain, Michael Soussan, A. Mekinian, V. Jacquelin, Dominique Valeyre, Hilario Nunes, and Pierre-Yves Brillet
Subjects: Adult, Male, medicine.medical_specialty, Vital capacity, Pulmonary Fibrosis, Vital Capacity, Standardized uptake value, Pilot Projects, Sensitivity and Specificity, 030218 nuclear medicine & medical imaging, Pulmonary function testing, 03 medical and health sciences, 0302 clinical medicine, Fluorodeoxyglucose F18, Forced Expiratory Volume, Positron Emission Tomography Computed Tomography, Pulmonary fibrosis, medicine, Humans, Radiology, Nuclear Medicine and imaging, Lung volumes, Honeycombing, Idiopathic interstitial pneumonia, Lung, Aged, Retrospective Studies, Aged, 80 and over, Receiver operating characteristic, business.industry, Total Lung Capacity, General Medicine, respiratory system, Middle Aged, medicine.disease, respiratory tract diseases, Radiographic Image Enhancement, Treatment Outcome, ROC Curve, 030220 oncology & carcinogenesis, Area Under Curve, Female, Radiology, Radiopharmaceuticals, Nuclear medicine, business, Lung Diseases, Interstitial, Tomography, X-Ray Computed, Follow-Up Studies, Forecasting
Abstract: Purpose Our study aimed to analyse the characteristics of nonspecific interstitial pneumonia (NSIP) using FDG-PET/CT (PET) and to evaluate its ability to predict the therapeutic response. Procedures Eighteen NSIP patients were included. Maximum standardized uptake value (SUV max ), FDG uptake extent (in percentage of lung volume), high resolution CT scan (HRCT) elementary lesions, and HRCT fibrosis score were recorded. The predictive value of the parameters for lung function improvement was evaluated using logistic regression and Receiver Operating Characteristic (ROC) curve analysis (n=13/18). Results All patients had an increased pulmonary FDG uptake (median SUV max =3.1 [2–7.6]), with a median extent of 19% [6–67]. Consolidations, ground-glass opacities, honeycombing and reticulations showed uptake in 90%, 89%, 85% and 76%, respectively. FDG uptake extent was associated with improvement of pulmonary function under treatment (increase in forced vital capacity>10%, p=0.03), whereas SUV max and HRCT fibrosis score were not (p>0.5). For FDG uptake extent, ROC analysis showed an area under the curve at 0.85±0.11 and sensitivity/specificity was 88%/80% for a threshold fixed at 21%. Conclusions Increased FDG uptake was observed in all NSIP patients, both in inflammatory and fibrotic HRCT lesions. The quantification of FDG uptake extent might be useful to predict functional improvement under treatment.
Published: 2016

198. Multicentre evaluation of multidisciplinary team meeting agreement on diagnosis in diffuse parenchymal lung disease: a case-cohort study

Author: Simon L.F. Walsh, David M. Hansell, Jan C. Grutters, Daniel A F van den Heuvel, Andrew G. Nicholson, Venerino Poletti, Sabine Pomplun, Kevin R. Flaherty, Elisabeth Bendstrup, Pierre Yves Brillet, Line Bille Madsen, Alessandra Dubini, Junya Fukuoka, Cornelis A Seldenrijk, António Morais, Athol U. Wells, Finn Rasmussen, Hendrik W. van Es, Hiroyuki Taniguchi, Takeshi Johkoh, Charlie Sayer, Conceição Souto Moura, Sujal R. Desai, Lilian Edmunds, Bibek Gooptu, Hilario Nunes, Sara Piciucchi, Dominique Valeyre, Joseph Jacob, Maria Kokosi, Matthijs F.M. van Oosterhout, Jeffrey L. Myers, Marianne Kambouchner, and José Manuel Pereira
Subjects: Male, Respiratory System, Cohort Studies, Idiopathic pulmonary fibrosis, 0302 clinical medicine, Cohen's kappa, Japan, Medicine, 030212 general & internal medicine, Medical diagnosis, Observer Variation, medicine.diagnostic_test, NONSPECIFIC INTERSTITIAL PNEUMONIA, Interstitial lung disease, respiratory system, Middle Aged, Europe, Regression Analysis, Female, Radiology, Life Sciences & Biomedicine, HYPERSENSITIVITY PNEUMONITIS, Cohort study, Alveolitis, Extrinsic Allergic, Pulmonary and Respiratory Medicine, medicine.medical_specialty, Clinical Decision-Making, Lung biopsy, 1117 Public Health and Health Services, Diagnosis, Differential, 03 medical and health sciences, Critical Care Medicine, General & Internal Medicine, Biopsy, Journal Article, Humans, Intensive care medicine, Aged, Probability, Proportional Hazards Models, Patient Care Team, Science & Technology, business.industry, Patient Selection, 1103 Clinical Sciences, medicine.disease, Idiopathic Pulmonary Fibrosis, respiratory tract diseases, Clinical trial, 030228 respiratory system, INTEROBSERVER AGREEMENT, IDIOPATHIC PULMONARY-FIBROSIS, Interdisciplinary Communication, business, Lung Diseases, Interstitial, 1199 Other Medical and Health Sciences
Abstract: BACKGROUND: Diffuse parenchymal lung disease represents a diverse and challenging group of pulmonary disorders. A consistent diagnostic approach to diffuse parenchymal lung disease is crucial if clinical trial data are to be applied to individual patients. We aimed to evaluate inter-multidisciplinary team agreement for the diagnosis of diffuse parenchymal lung disease.METHODS: We did a multicentre evaluation of clinical data of patients who presented to the interstitial lung disease unit of the Royal Brompton and Harefield NHS Foundation Trust (London, UK; host institution) and required multidisciplinary team meeting (MDTM) characterisation between March 1, 2010, and Aug 31, 2010. Only patients whose baseline clinical, radiological, and, if biopsy was taken, pathological data were undertaken at the host institution were included. Seven MDTMs, consisting of at least one clinician, radiologist, and pathologist, from seven countries (Denmark, France, Italy, Japan, Netherlands, Portugal, and the UK) evaluated cases of diffuse parenchymal lung disease in a two-stage process between Jan 1, and Oct 15, 2015. First, the clinician, radiologist, and pathologist (if lung biopsy was completed) independently evaluated each case, selected up to five differential diagnoses from a choice of diffuse lung diseases, and chose likelihoods (censored at 5% and summing to 100% in each case) for each of their differential diagnoses, without inter-disciplinary consultation. Second, these specialists convened at an MDTM and reviewed all data, selected up to five differential diagnoses, and chose diagnosis likelihoods. We compared inter-observer and inter-MDTM agreements on patient first-choice diagnoses using Cohen's kappa coefficient (κ). We then estimated inter-observer and inter-MDTM agreement on the probability of diagnosis using weighted kappa coefficient (κw). We compared inter-observer and inter-MDTM confidence of patient first-choice diagnosis. Finally, we evaluated the prognostic significance of a first-choice diagnosis of idiopathic pulmonary fibrosis (IPF) versus not IPF for MDTMs, clinicians, and radiologists, using univariate Cox regression analysis.FINDINGS: 70 patients were included in the final study cohort. Clinicians, radiologists, pathologists, and the MDTMs assigned their patient diagnoses between Jan 1, and Oct 15, 2015. IPF made up 88 (18%) of all 490 MDTM first-choice diagnoses. Inter-MDTM agreement for first-choice diagnoses overall was moderate (κ=0·50). Inter-MDTM agreement on diagnostic likelihoods was good for IPF (κw=0·71 [IQR 0·64-0·77]) and connective tissue disease-related interstitial lung disease (κw=0·73 [0·68-0·78]); moderate for non-specific interstitial pneumonia (NSIP; κw=0·42 [0·37-0·49]); and fair for hypersensitivity pneumonitis (κw=0·29 [0·24-0·40]). High-confidence diagnoses (>65% likelihood) of IPF were given in 68 (77%) of 88 cases by MDTMs, 62 (65%) of 96 cases by clinicians, and in 57 (66%) of 86 cases by radiologists. Greater prognostic separation was shown for an MDTM diagnosis of IPF than compared with individual clinician's diagnosis of this disease in five of seven MDTMs, and radiologist's diagnosis of IPF in four of seven MDTMs.INTERPRETATION: Agreement between MDTMs for diagnosis in diffuse lung disease is acceptable and good for a diagnosis of IPF, as validated by the non-significant greater prognostic separation of an IPF diagnosis made by MDTMs than the separation of a diagnosis made by individual clinicians or radiologists. Furthermore, MDTMs made the diagnosis of IPF with higher confidence and more frequently than did clinicians or radiologists. This difference is of particular importance, because accurate and consistent diagnoses of IPF are needed if clinical outcomes are to be optimised. Inter-multidisciplinary team agreement for a diagnosis of hypersensitivity pneumonitis is low, highlighting an urgent need for standardised diagnostic guidelines for this disease.FUNDING: National Institute of Health Research, Imperial College London.
Published: 2016

199. Comment on: rituximab in autoimmune connective tissue disease-associated interstitial lung disease

Author: Yurdagül, Uzunhan, Lila, Ferrah, Pierre-Yves, Brillet, Robin, Dhote, Dominique, Valeyre, and Hilario, Nunes
Subjects: Humans, Connective Tissue Diseases, Lung Diseases, Interstitial, Rituximab
Published: 2016

200. Connective-Tissue Disease-Associated Interstitial Lung Disease

Author: Marianne Kambouchner, Hilario Nunes, Pierre-Yves Brillet, Dominique Valeyre, and Yurdagul Uzunhan
Subjects: Pathology, medicine.medical_specialty, business.industry, Interstitial lung disease, medicine, medicine.disease, business, Connective tissue disease
Published: 2016

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