Author: "Jean-Marc, Naccache" - Searchworks@Jio Institute Digital Library Search Results

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1. Differential diagnosis of pulmonary sarcoidosis: a review

Author: Dominique Valeyre, Michel Brauner, Jean-François Bernaudin, Etienne Carbonnelle, Boris Duchemann, Cécile Rotenberg, Ingrid Berger, Antoine Martin, Hilario Nunes, Jean-Marc Naccache, and Florence Jeny
Subjects: sarcoidosis, differential diagnosis, granuloma, tuberculosis, thoracic computed tomography, pathology, Medicine (General), R5-920
Abstract: Diagnosing pulmonary sarcoidosis raises challenges due to both the absence of a specific diagnostic criterion and the varied presentations capable of mimicking many other conditions. The aim of this review is to help non-sarcoidosis experts establish optimal differential-diagnosis strategies tailored to each situation. Alternative granulomatous diseases that must be ruled out include infections (notably tuberculosis, nontuberculous mycobacterial infections, and histoplasmosis), chronic beryllium disease, hypersensitivity pneumonitis, granulomatous talcosis, drug-induced granulomatosis (notably due to TNF-a antagonists, immune checkpoint inhibitors, targeted therapies, and interferons), immune deficiencies, genetic disorders (Blau syndrome), Crohn’s disease, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, and malignancy-associated granulomatosis. Ruling out lymphoproliferative disorders may also be very challenging before obtaining typical biopsy specimen. The first step is an assessment of epidemiological factors, notably the incidence of sarcoidosis and of alternative diagnoses; exposure to risk factors (e.g., infectious, occupational, and environmental agents); and exposure to drugs taken for therapeutic or recreational purposes. The clinical history, physical examination and, above all, chest computed tomography indicate which differential diagnoses are most likely, thereby guiding the choice of subsequent investigations (e.g., microbiological investigations, lymphocyte proliferation tests with metals, autoantibody assays, and genetic tests). The goal is to rule out all diagnoses other than sarcoidosis that are consistent with the clinical situation. Chest computed tomography findings, from common to rare and from typical to atypical, are described for sarcoidosis and the alternatives. The pathology of granulomas and associated lesions is discussed and diagnostically helpful stains specified. In some patients, the definite diagnosis may require the continuous gathering of information during follow-up. Diseases that often closely mimic sarcoidosis include chronic beryllium disease and drug-induced granulomatosis. Tuberculosis rarely resembles sarcoidosis but is a leading differential diagnosis in regions of high tuberculosis endemicity.
Published: 2023
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2. Study protocol: exploring the efficacy of cyclophosphamide added to corticosteroids for treating acute exacerbation of idiopathic pulmonary fibrosis; a randomized double-blind, placebo-controlled, multi-center phase III trial (EXAFIP)

Author: Jean-Marc Naccache, Melissa Montil, Jacques Cadranel, Marine Cachanado, Vincent Cottin, Bruno Crestani, Dominique Valeyre, Benoit Wallaert, Tabassome Simon, and Hilario Nunes
Subjects: Idiopathic pulmonary fibrosis, Acute exacerbation, Cyclophosphamide, Diseases of the respiratory system, RC705-779
Abstract: Abstract Background Idiopathic pulmonary fibrosis (IPF) is a fatal lung disease, with a median survival of 2–3 years and variable natural history, characterized by gradual and progressive deterioration. Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is a severe complication, associated with poor survival and a mortality > 50%. To date, no treatment has proven effective in AE-IPF, with cyclophosphamide (CYC) the only therapy suggested to be effective on survival, primarily based on retrospective series. Considering the high fatality rates of AE-IPF, evaluating the efficacy of immunosuppressive agents in a randomized controlled trial proves crucial, as the results could significantly impact treatment and prognosis of AE-IPF. Methods The EXAFIP study is a French national multicenter double-blind placebo-controlled randomized trial. Its primary objective is to evaluate the efficacy of CYC compared to placebo on early survival in patients treated with corticosteroids. We hypothesize that adding CYC to high-dose corticosteroids would reduce 3-month mortality in AE-IPF patients. The primary outcome is all-cause mortality rate at Month 3; secondary objectives are to evaluate the efficacy of CYC compared to placebo on overall survival at Months 6 and 12, respiratory disease-specific mortality, respiratory morbidity, and chest high-resolution computed tomography features, and to determine prognostic factors in AE-IPF and compare the safety of the two treatment arms during 6 months’ follow-up. Discussion There is an urgent unmet clinical need for effective AE-IPF treatment. The EXAFIP study is the first large Phase III placebo-controlled randomized trial evaluating the efficacy and safety of CYC added to corticosteroids in treating AE-IPF. The results of this study could significantly impact treatment strategy and prognosis of AE-IPF. Trial registration Clinical trials, NCT02460588; Date: June 2, 2015, prospectively; Issue date: 14/11/2017; Protocole Amendment Number: 03.
Published: 2019
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3. Rituximab for auto-immune alveolar proteinosis, a real life cohort study

Author: Berenice Soyez, Raphael Borie, Cedric Menard, Jacques Cadranel, Leonidas Chavez, Vincent Cottin, Emmanuel Gomez, Sylvain Marchand-Adam, Sylvie Leroy, Jean-Marc Naccache, Hilario Nunes, Martine Reynaud-Gaubert, Laurent Savale, Abdellatif Tazi, Lidwine Wemeau-Stervinou, Marie-Pierre Debray, and Bruno Crestani
Subjects: Whole lung lavage, GM-CSF, Surfactant, Interstitial lung disease, Therapy, Diseases of the respiratory system, RC705-779
Abstract: Abstract Background Whole lung lavage is the current standard therapy for pulmonary alveolar proteinosis (PAP) that is characterized by the alveolar accumulation of surfactant. Rituximab showed promising results in auto-immune PAP (aPAP) related to anti-GM-CSF antibody. Methods We aimed to assess efficacy of rituximab in aPAP in real life and all patients with aPAP in France that received rituximab were retrospectively analyzed. Results Thirteen patients were included. No patients showed improvement 6 months after treatment, but, 4 patients (30%) presented a significant decrease of alveolar-arterial difference in oxygen after 1 year. One patient received lung transplantation and one patient was lost of follow-up within one year. Although a spontaneous improvement cannot be excluded in these 4 patients, improvement was more frequent in patients naïve to prior specific therapy and with higher level of anti-GM-CSF antibodies evaluated by ELISA. No serious adverse event was evidenced. Conclusions These data do not support rituximab as a second line therapy for patients with refractory aPAP.
Published: 2018
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4. Pulmonary complications of immune checkpoint inhibitors in patients with nonsmall cell lung cancer

Author: Jacques Cadranel, Anthony Canellas, Lise Matton, Marie Darrason, Antoine Parrot, Jean-Marc Naccache, Armelle Lavolé, Anne-Marie Ruppert, and Vincent Fallet
Subjects: Diseases of the respiratory system, RC705-779
Abstract: Immune checkpoint inhibitor-related pneumonitis (ICI-P) during cancer treatment is rarely observed (
Published: 2019
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5. French recommendations for the diagnosis and management of lymphangioleiomyomatosis

Author: Vincent Cottin, Elodie Blanchard, Mallorie Kerjouan, Romain Lazor, Martine Reynaud-Gaubert, Camille Taille, Yurdagül Uzunhan, Lidwine Wemeau, Claire Andrejak, Dany Baud, Philippe Bonniaud, Pierre-Yves Brillet, Alain Calender, Lara Chalabreysse, Isabelle Court-Fortune, Nicolas Pierre Desbaillets, Gilbert Ferretti, Anne Guillemot, Laurane Hardelin, Marianne Kambouchner, Violette Leclerc, Mathieu Lederlin, Marie-Claire Malinge, Alain Mancel, Sylvain Marchand-Adam, Jean-Michel Maury, Jean-Marc Naccache, Mouhamad Nasser, Hilario Nunes, Gaële Pagnoux, Grégoire Prévot, Christine Rousset-Jablonski, Olivier Rouviere, Salim Si-Mohamed, Renaud Touraine, Julie Traclet, Ségolène Turquier, Stéphane Vagnarelli, Kaïs Ahmad, 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), Centre de recherche Cardio-Thoracique de Bordeaux [Bordeaux] (CRCTB), Université Bordeaux Segalen - Bordeaux 2-CHU Bordeaux [Bordeaux]-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Pontchaillou [Rennes], Centre Hospitalier Universitaire Vaudois [Lausanne] (CHUV), Assistance Publique - Hôpitaux de Marseille (APHM), Microbes évolution phylogénie et infections (MEPHI), Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS), AP-HP - Hôpital Bichat - Claude Bernard [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Physiopathologie et Epidémiologie des Maladies Respiratoires (PHERE (UMR_S_1152 / U1152)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité), Plateforme F-CRIN, Université Toulouse III - Paul Sabatier (UT3), Université de Toulouse (UT)-Université de Toulouse (UT)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Institut National de la Santé et de la Recherche Médicale (INSERM), Hypoxie et Poumon : pneumopathologies fibrosantes, modulations ventilatoires et circulatoires (H&P), UFR SMBH-Université Sorbonne Paris Nord, Hôpital Avicenne [AP-HP], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), CHU Amiens-Picardie, Agents infectieux, résistance et chimiothérapie - UR UPJV 4294 (AGIR ), Université de Picardie Jules Verne (UPJV)-CHU Amiens-Picardie, EA3920 University of Burgundy Franche-Comté Besançon, Equipe CADIR (LNC - U1231), Lipides - Nutrition - Cancer [Dijon - U1231] (LNC), Université de Bourgogne (UB)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut Agro Dijon, Institut national d'enseignement supérieur pour l'agriculture, l'alimentation et l'environnement (Institut Agro)-Institut national d'enseignement supérieur pour l'agriculture, l'alimentation et l'environnement (Institut Agro)-Université de Bourgogne (UB)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut Agro Dijon, Institut national d'enseignement supérieur pour l'agriculture, l'alimentation et l'environnement (Institut Agro)-Institut national d'enseignement supérieur pour l'agriculture, l'alimentation et l'environnement (Institut Agro), Laboratoire de Biologie Tissulaire et d'ingénierie Thérapeutique UMR 5305 (LBTI), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Centre National de la Recherche Scientifique (CNRS), Hôpital Louis Pradel [CHU - HCL], Hospices Civils de Lyon (HCL), Centre pour l'innovation en cancérologie de Lyon (CICLY), Université de Lyon-Université de Lyon, Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E), Institute for Advanced Biosciences / Institut pour l'Avancée des Biosciences (Grenoble) (IAB), Centre Hospitalier Universitaire [Grenoble] (CHU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Etablissement français du sang - Auvergne-Rhône-Alpes (EFS)-Centre National de la Recherche Scientifique (CNRS)-Université Grenoble Alpes (UGA), and Centre Hospitalier Universitaire [Grenoble] (CHU)
Subjects: Pulmonary and Respiratory Medicine, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
Abstract: International audience; BackgroundThe present article is an English-language version of the French National Diagnostic and Care Protocol, a pragmatic tool to optimize and harmonize the diagnosis, care pathway, management and follow-up of lymphangioleiomyomatosis in France.MethodsPractical recommendations were developed in accordance with the method for developing a National Diagnosis and Care Protocol for rare diseases of the Haute Autorité de Santé and following international guidelines and literature on lymphangioleiomyomatosis. It was developed by a multidisciplinary group, with the help of patient representatives and of RespiFIL, the rare disease network on respiratory diseases.ResultsLymphangioleiomyomatosis is a rare lung disease characterised by a proliferation of smooth muscle cells that leads to the formation of multiple lung cysts. It occurs sporadically or as part of a genetic disease called tuberous sclerosis complex (TSC). The document addresses multiple aspects of the disease, to guide the clinicians regarding when to suspect a diagnosis of lymphangioleiomyomatosis, what to do in case of recurrent pneumothorax or angiomyolipomas, what investigations are needed to make the diagnosis of lymphangioleiomyomatosis, what the diagnostic criteria are for lymphangioleiomyomatosis, what the principles of management are, and how follow-up can be organised. Recommendations are made regarding the use of pharmaceutical specialties and treatment other than medications.ConclusionThese recommendations are intended to guide the diagnosis and practical management of pulmonary lymphangioleiomyomatosis.
Published: 2023
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6. Long-term safety of pirfenidone: results of the prospective, observational PASSPORT study

Author: Vincent Cottin, Dirk Koschel, Andreas Günther, Carlo Albera, Arata Azuma, C. Magnus Sköld, Sara Tomassetti, Philip Hormel, John L. Stauffer, Indiana Strombom, Klaus-Uwe Kirchgaessler, Toby M. Maher, PASSPORT study group, Peter Cerkl, Holger Flick, Christian Geltner, Judith Löffler-Ragg, Michael Studnicka, Elisabeth Bendstrup, Helle Dall Madsen, Saher B. Shaker, Maritta Kilpelainen, Marjukka Myllaerniemi, Minna Purokivi, Seppo Saarelainen, Emmanuel Bergot, Philippe Bonniaud, Raphael Borie, Pascal Chanez, Francis Couturaud, Claire Dromer, Frederic Gagnadoux, Anne-Sophie Gamez, Anne Gondouin, Dominique Israel-Biet, Stephane Jouneau, Romain Kessler, Francois Lebargy, Sylvain Marchand-Adam, Borsi Melloni, Jean-Marc Naccache, Christophe Pison, Gregoire Prevot, Martine Reynaud-Gaubert, Abdellatif Tazi, Dominique Valeyre, Benoit Wallaert, Jost Achenbach, Stefan Andreas, Burkhard Bewig, Reiner Bonnet, Stephan Budweiser, Ulrich Costabel, Martin Faehling, Joachim Ficker, Juergen Fisher, Sven Glaeser, Christian Grah, Christian Grohe, Martin Hetzel, Wolfgang Hohenforst-Schmidt, David Jungck, Peter Kardos, Claus Keller, Martin Kohlhaeufl, Claus Kroegel, Rainer Kruegel, Berthold Michels, Joachim Mueller-Quernheim, Claus Neurohr, Heinz-Theodor Pelzer, Michael Pfeifer, Michael Prediger, Klaus F. Rabe, Winifried Randerath, Nicolas Scheonfeld, Robert Scheubel, Jens Schreiber, Hartwig Schutte, Bernd Schoenhofer, Barbara Wagener, Tobias Welte, Michael Westhoff, Heinrike Wilkens, Hubert Wirtz, Michael Henry, Michael Keane, Anthony O'Regan, Katherine O'Reilly, Fabrizio Luppi, Sandra Nutini, Alberto Pesci, Venerino Poletti, Elisabetta Rosi, Paola Rottoli, Thomas Eagan, Durdica Kulosman, Tone Blorg Sjaheim, Karl Axel Karlsson, Magnus Sköld, Sanjay Agrawal, Kesavan Suresh Babu, Stephen Bianchi, Nazia Chaudhuri, Sophie Fletcher, Michael Gibbons, Simon Hart, Gisli Jenkins, Toby Maher, Ann Millar, Joanna Porter, Nicky Simler, Lisa Spencer, Monica Spiteri, and Melissa Wickremasinghe
Subjects: Medicine
Abstract: Real-world studies include a broader patient population for a longer duration than randomised controlled trials (RCTs) and can provide relevant insights for clinical practice. PASSPORT was a multicentre, prospective, post-authorisation study of patients who were newly prescribed pirfenidone and followed for 2 years after initiating treatment. Physicians collected data on adverse drug reactions (ADRs), serious ADRs (SADRs) and ADRs of special interest (ADRSI) at baseline and then every 3 months. Post hoc stepwise logistic regression models were used to identify baseline characteristics associated with discontinuing treatment due to an ADR. Patients (n=1009, 99.7% with idiopathic pulmonary fibrosis) had a median pirfenidone exposure of 442.0 days. Overall, 741 (73.4%) patients experienced ADRs, most commonly nausea (20.6%) and fatigue (18.5%). ADRs led to treatment discontinuation in 290 (28.7%) patients after a median of 99.5 days. Overall, 55 (5.5%) patients experienced SADRs, with a fatal outcome in six patients. ADRSI were reported in 693 patients, most commonly gastrointestinal symptoms (38.3%) and photosensitivity reactions/skin rashes (29.0%). Older age and female sex were associated with early treatment discontinuation due to an ADR. Findings were consistent with the known safety profile of pirfenidone, based on RCT data and other post-marketing experience, with no new safety signals observed.
Published: 2018
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7. Cyclophosphamide added to glucocorticoids in acute exacerbation of idiopathic pulmonary fibrosis (EXAFIP): a randomised, double-blind, placebo-controlled, phase 3 trial

Author: Abdellatif Tazi, Aurélie Le Borgne-Krams, Marine Cachanado, Tabassome Simon, Sylvain Marchand-Adam, Morgane Didier, Lidwine Wemeau-Stervinou, Sandrine Hirschi, Frédéric Rivière, Arnaud Bourdin, François Lebargy, Stéphane Dominique, Aude Gibelin, Alexandre Chabrol, Tristan Dégot, Jacques Cadranel, Martine Reynaud-Gaubert, Marie-Pierre Debray, Sylvie Leroy, Frédéric Gagnadoux, Emmanuel Bergot, François-Xavier Blanc, Alexandra Rousseau, Raphael Borie, Pierre Yves Brillet, Guillaume Beltramo, Mallorie Kerjouan, Hilario Nunes, Olivia Freynet, Julie Traclet, Bruno Crestani, Anne-Sophie Gamez, Grégoire Prévot, Jean Pastré, Dominique Israel-Biet, Marie-Christine Dombret, Laurent Plantier, Cécile Chenivesse, Laurence Berard, Ana Nieves, Emmanuel Gomez, Dominique Valeyre, Stéphane Jouneau, Anne Gondouin, Elodie Blanchard, C. Launois, Nathalie Bautin, Jean-Marc Naccache, Vincent Cottin, Antoine Parrot, Philippe Bonniaud, Centre de référence maladies rares des maladies pulmonaires rares de l’adulte (CHU Dijon) (CRMR des maladies pulmonaires rares de l’adulte), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Sorbonne Université (SU), Centre hospitalier Saint-Joseph [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Hôpital Foch [Suresnes], CHU Pontchaillou [Rennes], École des Hautes Études en Santé Publique [EHESP] (EHESP), 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 ), Hôpital Avicenne [AP-HP], Laboratoire d'Excellence INFLAMEX [Paris], Université Sorbonne Paris Cité (USPC), CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), AP-HP - Hôpital Bichat - Claude Bernard [Paris], CHU Saint-Antoine [AP-HP], Physiologie & médecine expérimentale du Cœur et des Muscles [U 1046] (PhyMedExp), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université de Montpellier (UM), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Centre régional de pharmacovigilance de Marseille Provence Corse [CHU de Marseille] (CRPV-Marseille), Assistance Publique - Hôpitaux de Marseille (APHM)-CHU Marseille, CHU Dijon, Université de Franche-Comté (UFC), Université Bourgogne Franche-Comté [COMUE] (UBFC), 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), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Les Hôpitaux Universitaires de Strasbourg (HUS), Nouvel Hôpital Civil de Strasbourg, Centre Hospitalier Universitaire de Reims (CHU Reims), CHU Trousseau [Tours], Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), CHU Lille, Hôpital Albert Calmette, Université de Lille, Droit et Santé-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), 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), Hospices Civils de Lyon (HCL), Université de Lyon, CIC CHU Lyon (inserm), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Université Côte d'Azur (UCA), Centre Hospitalier Universitaire de Nice (CHU Nice), Université d'Angers (UA), Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM), Hôpital d'instruction des Armées Percy, Service de Santé des Armées, Hôpital Côte de Nacre [CHU Caen], CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN), Hôpital JeanMinjoz, CHU Bordeaux [Bordeaux], Hôpital Haut-Lévêque [CHU Bordeaux], unité de recherche de l'institut du thorax UMR1087 UMR6291 (ITX), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Nantes Université - UFR de Médecine et des Techniques Médicales (Nantes Univ - UFR MEDECINE), Nantes Université - pôle Santé, Nantes Université (Nantes Univ)-Nantes Université (Nantes Univ)-Nantes Université - pôle Santé, Nantes Université (Nantes Univ)-Nantes Université (Nantes Univ), Centre hospitalier universitaire de Nantes (CHU Nantes), CHU Rouen, Normandie Université (NU), CHU Tenon [AP-HP], Immunologie humaine, physiopathologie & immunothérapie (HIPI (UMR_S_976 / U976)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité), Institut de Recherche Saint-Louis - Hématologie Immunologie Oncologie (Département de recherche de l’UFR de médecine, ex- Institut Universitaire Hématologie-IUH) (IRSL), Université Paris Cité (UPCité), Physiopathologie et Epidémiologie des Maladies Respiratoires (PHERE (UMR_S_1152 / U1152)), CHU Montpellier, Université de Lille, Institut Pasteur de Lille, Réseau International des Instituts Pasteur (RIIP), Centre d’Infection et d’Immunité de Lille - INSERM U 1019 - UMR 9017 - UMR 8204 (CIIL), 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), Roche, Ministere de la Sante et des Solidarites, 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), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM)-Centre National de la Recherche Scientifique (CNRS), CHU Marseille-Assistance Publique-Hôpitaux de Marseille (AP-HM), Hôpital Larrey [Toulouse], CHU Toulouse [Toulouse], Université de Nantes - UFR de Médecine et des Techniques Médicales (UFR MEDECINE), Université de Nantes (UN)-Université de Nantes (UN)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Paris (UP), Université de Paris (UP), Centre National de la Recherche Scientifique (CNRS)-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)-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), 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 ), and Université de Montpellier (UM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)
Subjects: Pulmonary and Respiratory Medicine, medicine.medical_specialty, education.field_of_study, Exacerbation, Cyclophosphamide, business.industry, [SDV]Life Sciences [q-bio], Population, medicine.disease, Placebo, 3. Good health, 03 medical and health sciences, Idiopathic pulmonary fibrosis, 0302 clinical medicine, 030228 respiratory system, Methylprednisolone, Internal medicine, Clinical endpoint, Medicine, 030212 general & internal medicine, business, education, Adverse effect, ComputingMilieux_MISCELLANEOUS, medicine.drug
Abstract: Summary Background The use of cyclophosphamide in patients with acute exacerbation of idiopathic pulmonary fibrosis (IPF) is unknown. Our study was designed to evaluate the efficacy and safety of four cyclophosphamide pulses in addition to high-dose methylprednisolone in this population. Methods In this double-blind, placebo-controlled trial done in 35 departments across 31 hospitals in France, adult patients (≥18 years) with acute exacerbation of IPF and those with suspected acute exacerbation of IPF were randomly assigned in a 1:1 ratio using a web-based system to receive either intravenous pulses of cyclophosphamide (600 mg/m2) plus uromitexan as haemorrhagic cystitis prophylaxis (200 mg/m2) at the time of cyclophosphamide administration and then again, 4 h later, or placebo at days 0, 15, 30, and 60. Random assignment was stratified according to the severity of IPF and was block-balanced with variable block sizes of four or six patients. Patients receiving mechanical ventilation, with active infection, with active cancer, or who were registered on the lung transplant waiting list were excluded. All patients received standardised high-dose glucocorticoids. The investigators, patients, and the sponsor were masked to the treatment assignments. The primary endpoint was 3-month all-cause mortality, analysed by a χ2 test adhering to an intention-to-treat principle. The trial is now complete and registered with ClinicalTrials.gov , NCT02460588 . Findings Between Jan 22, 2016, and July 19, 2018, 183 patients were assessed for eligibility, of whom 120 patients were randomly assigned and 119 patients (62 [52%] with severe IPF) received at least one dose of cyclophosphamide (n=60) or placebo (n=59), all of whom were included in the intention-to-treat analysis. The 3-month all-cause mortality was 45% (27/60) in patients given cyclophosphamide compared with 31% (18/59) in the placebo group (difference 14·5% [95% CI −3·1 to 31·6]; p=0·10). Similar results were found after adjustment by IPF severity (odds ratio [OR] 1·89 [95% CI 0·89–4·04]). The risk of death at 3 months, independent of the treatment received, was higher with severe than non-severe IPF (OR 2·62 [1·12–6·12]) and was lower with the use of antifibrotic therapy (OR 0·33 [0·13–0·82]). Adverse events were similar between groups by 6 months (25 [42%] in the cyclophosphamide group vs 30 [51%] in the placebo group) and their proportion, including infections, did not differ. Overall infection was the main adverse event and occurred in 20 (33%) of 60 patients in the cyclophosphamide group versus 21 (36%) of 59 patients in the placebo group. Interpretation In patients with acute exacerbation of IPF, adding intravenous cyclophosphamide pulses to glucocorticoids increased 3-month mortality. These findings provide evidence against the use of intravenous cyclophosphamide in such patients. Funding Programme Hospitalier de Recherche Clinique of the French Ministry of Health (PHRC 2014–502), Roche Pharmaceuticals.
Published: 2022
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8. Germline Mutations of Telomere-Related Genes are a Major Risk Factor for Liver Disease: A Multicentric Transversal Study

Author: Sabrina Sidali, Raphaël Borie, Flore Sicre de Fontbrune, Kinan El Husseini, Pierre-Emmanuel Rautou, Elodie Lainey, Odile Goria, Bruno Crestani, Jacques Cadranel, Vincent Cottin, Vincent Bunel, Jérôme Dumortier, Emmanuel Jacquemin, Noémi Reboux, Sandrine Hirschi, Arnaud Bourdin, Magdalena Meszaros, Sébastien Dharancy, Sophie Hilaire, Vincent Mallet, Martine Reynaud-Gaubert, Louis Terriou, Frédéric Gottrand, Wadih Abou Chahla, Jean-Emmanuel Kahn, Paul Carrier, Faouzi Saliba, Laura Rubbia-Brandt, John-David Aubert, Laure Elkrief, Victor de Ledinghen, Armand Abergel, Olivier Tournilhac, Pauline Houssel, Stéphane Jouneau, Ludivine Wemeau, Anne Bergeron, Thierry Leblanc, Isabelle Ollivier-Hourmand, Eric Nguyen-Khac, Hélène Morisse-Pradier, Ibrahima Ba, Catherine Boileau, Françoise Roulot-Thoraval, Valérie Vilgrain, Christophe Bureau, Hilario Nunes, Jean-Marc Naccache, François Durand, Claire Francoz, Dominique Roulot, Dominique-Charles Valla, Valérie Paradis, Caroline Kannengiesser, and Aurélie Plessier
Published: 2023
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9. French practical guidelines for the diagnosis and management of idiopathic pulmonary fibrosis – 2021 update. Full-length version

Author: Vincent Cottin, Philippe Bonniaud, Jacques Cadranel, Bruno Crestani, Stéphane Jouneau, Sylvain Marchand-Adam, Hilario Nunes, Lidwine Wémeau-Stervinou, Emmanuel Bergot, Elodie Blanchard, Raphaël Borie, Arnaud Bourdin, Cécile Chenivesse, Annick Clément, Emmanuel Gomez, Anne Gondouin, Sandrine Hirschi, François Lebargy, Charles-Hugo Marquette, David Montani, Grégoire Prévot, Sébastien Quetant, Martine Reynaud-Gaubert, Mathieu Salaun, Olivier Sanchez, Bruno Trumbic, Karim Berkani, Pierre-Yves Brillet, Marion Campana, Lara Chalabreysse, Gérard Chatté, Didier Debieuvre, Gilbert Ferretti, Jean-Michel Fourrier, Nicolas Just, Marianne Kambouchner, Bertrand Legrand, Frédéric Le Guillou, Jean-Pierre Lhuillier, Anas Mehdaoui, Jean-Marc Naccache, Catherine Paganon, Martine Rémy-Jardin, Salim Si-Mohamed, Philippe Terrioux, 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), Centre national de référence des maladies pulmonaires rares [Lyon] (CRMPM), Lipides - Nutrition - Cancer [Dijon - U1231] (LNC), Université de Bourgogne (UB)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut Agro Dijon, Institut national d'enseignement supérieur pour l'agriculture, l'alimentation et l'environnement (Institut Agro)-Institut national d'enseignement supérieur pour l'agriculture, l'alimentation et l'environnement (Institut Agro), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Service de Pneumologie Soins Intensifs, Appareillage Respiratoire [CHU de Dijon], CHU Tenon [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), AP-HP - Hôpital Bichat - Claude Bernard [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), 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 ), École des Hautes Études en Santé Publique [EHESP] (EHESP), Hôpital Bretonneau, Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), Hôpital Avicenne [AP-HP], Institut Coeur Poumon [CHU Lille], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN), CHU Pessac, Physiologie & médecine expérimentale du Cœur et des Muscles [U 1046] (PhyMedExp), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université de Montpellier (UM), Centre d’Infection et d’Immunité de Lille - INSERM U 1019 - UMR 9017 - UMR 8204 (CIIL), 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 Hospitalier Régional Universitaire [Lille] (CHRU Lille)-Centre National de la Recherche Scientifique (CNRS), Maladies génétiques d'expression pédiatrique (U933), Institut National de la Santé et de la Recherche Médicale (INSERM)-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 Hospitalier Régional Universitaire de Besançon (CHRU Besançon), Nouvel Hôpital Civil de Strasbourg, Centre Hospitalier Universitaire de Reims (CHU Reims), Hôpital Pasteur [Nice] (CHU), Institut de Recherche sur le Cancer et le Vieillissement (IRCAN), Université Nice Sophia Antipolis (1965 - 2019) (UNS), COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Côte d'Azur (UCA), Hypertension arterielle pulmonaire physiopathologie et innovation thérapeutique, Centre Chirurgical Marie Lannelongue (CCML)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Centre Hospitalier Universitaire [Grenoble] (CHU), CHU Marseille, Laboratoire d'Informatique, de Traitement de l'Information et des Systèmes (LITIS), Université Le Havre Normandie (ULH), Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Institut national des sciences appliquées Rouen Normandie (INSA Rouen Normandie), Institut National des Sciences Appliquées (INSA)-Normandie Université (NU)-Institut National des Sciences Appliquées (INSA), Centre d'Investigation Clinique [CHU Rouen] (CIC Rouen), Hôpital Charles Nicolle [Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-CHU Rouen, Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM), 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), Hypoxie et Poumon : pneumopathologies fibrosantes, modulations ventilatoires et circulatoires (H&P), UFR SMBH-Université Sorbonne Paris Nord, Centre Hospitalier Régional d'Orléans (CHRO), Hospices Civils de Lyon (HCL), Centre Hospitalier Emile Muller [Mulhouse] (CH E.Muller Mulhouse), Groupe Hospitalier de Territoire Haute Alsace (GHTHA), Evaluation des technologies de santé et des pratiques médicales - ULR 2694 (METRICS), Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Centre Hospitalier Eure-Seine - Hôpital d'Evreux - Vernon (Evreux), Groupe Hospitalier Paris Saint-Joseph (hpsj), Services de Pneumologie, Exploration Fonctionnelle Respiratoire et Cardiologie (Hôpital Louis Pradel), Centre de Recherche en Acquisition et Traitement de l'Image pour la Santé (CREATIS), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National des Sciences Appliquées de Lyon (INSA Lyon), and Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)
Subjects: Pulmonary and Respiratory Medicine, Biopsy, Biopsie, [SDV]Life Sciences [q-bio], Fibrose pulmonaire, Pneumopathie interstitielle commune, Interstitial lung disease, Common interstitial lung disease, Pneumopathie interstitielle diffuse, Pulmonary fibrosis
Abstract: National audience; BACKGROUND: Since the previous French guidelines were published in 2017, substantial additional knowledge about idiopathic pulmonary fibrosis has accumulated. METHODS: Under the auspices of the French-speaking Learned Society of Pulmonology and at the initiative of the coordinating reference center, practical guidelines for treatment of rare pulmonary diseases have been established. They were elaborated by groups of writers, reviewers and coordinators with the help of the OrphaLung network, as well as pulmonologists with varying practice modalities, radiologists, pathologists, a general practitioner, a head nurse, and a patients’ association. The method was developed according to rules entitled "Good clinical practice" in the overall framework of the "Guidelines for clinical practice" of the official French health authority (HAS), taking into account the results of an online vote using a Likert scale. RESULTS: After analysis of the literature, 54 recommendations were formulated, improved, and validated by the working groups. The recommendations covered a wide-ranging aspects of the disease and its treatment: epidemiology, diagnostic modalities, quality criteria and interpretation of chest CT, indication and modalities of lung biopsy, etiologic workup, approach to familial disease entailing indications and modalities of genetic testing, evaluation of possible functional impairments and prognosis, indications for and use of antifibrotic therapy, lung transplantation, symptom management, comorbidities and complications, treatment of chronic respiratory failure, diagnosis and management of acute exacerbations of fibrosis. CONCLUSION: These evidence-based guidelines are aimed at guiding the diagnosis and the management in clinical practice of idiopathic pulmonary fibrosis.
Published: 2022
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10. Effect of diagnosis level of certainty on adherence to antibiotics' guidelines in ED patients with pneumonia: a post-hoc analysis of an interventional trial

Author: Sarah, Tubiana, Loïc, Epelboin, Enrique, Casalino, Bérangère Gallet, De Saint Aurin, Jean-Marc, Naccache, Antoine, Feydy, Antoine, Khalil, Pierre, Hausfater, Xavier, Duval, and Yann-Erick, Claessens
Abstract: Clinical diagnosis of community-acquired pneumonia (CAP) is difficult to establish with certainty. Adherence to antibiotic guidelines independently affects the prognosis of CAP patients.We aimed to determine whether guidelines' adherence was related to CAP diagnosis level of certainty and could be reinforced accordingly to diagnosis improvement.Secondary analysis of a prospective, multicenter study, which evaluated the impact of early thoracic CT scan on diagnosis and therapeutic plan in patients with clinically suspected CAP visiting emergency departments.In total 319 patients with clinically suspected CAP were enrolled in four emergency departments, Paris, France, between Nov 2011 and Jan 2013.We evaluated guidelines' adherence before and after CT scan and its relationship with CAP diagnosis level of certainty. Antibiotics were categorized as adherent according to 2010 French guidelines. CAP diagnosis level of certainty was prospectively classified by the emergency physicians based on a Likert scale as excluded, possible, probable or definite before and immediately after the CT scan. These classifications and therapeutic plans were also completed by an independent adjudication committee. Determinants of adherence were assessed using Poisson regression with robust variance.Adherence to guidelines increased from 34.2% before CT scan to 51.3% after CT scan [difference 17.1% (95% CI, 9.5-24.7)], meanwhile CAP diagnosis with high level of certainty (definite and excluded CAP) increased from 46.1 to 79.6% [difference 33.5% (95% CI, 26.5-40.5)]. Diagnosis level of certainty before CT scan was the strongest determinant of adherence in multivariate analysis (RR, 2.63; 95% CI, 1.89-3.67).Antibiotic guidelines' adherence was poor and positively related to CAP diagnosis level of certainty. The results suggest that improvements in CAP diagnosis may increase adherence to antibiotic guidelines. Clinical trial registered with www.clinicaltrials.gov (NCT01574066).
Published: 2022

11. Difficultés diagnostiques de la bérylliose pulmonaire chronique en France

Author: B. Guillaud Segard, M. Ghanem, Romain Lazor, Dominique Israel-Biet, V. Bonneterre, Sylvain Marchand-Adam, J.P. L’huillier, Vincent Cottin, M.F. Marquignon, Abdellatif Tazi, Anne Gondouin, Jean-Marc Naccache, Dominique Valeyre, CHU Grenoble, Service de pneumologie, Hopital Saint-Louis [AP-HP] (AP-HP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de Pneumologie, oncologie thoracique et allergologie respiratoire [CHRU Besançon], Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon)-Université de Franche-Comté (UFC), Université Bourgogne Franche-Comté [COMUE] (UBFC)-Université Bourgogne Franche-Comté [COMUE] (UBFC), Université de Paris - UFR Médecine Paris Centre [Santé] (UP Médecine Paris Centre), Université de Paris (UP), 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), Laboratoire d'Anatomie Pathologique [CHU Caen], Université de Caen Normandie (UNICAEN), Normandie Université (NU)-Normandie Université (NU)-CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Tumorothèque de Caen Basse-Normandie (TCBN), Centre d’Etude des Pathologies Respiratoires (CEPR), UMR 1100 (CEPR), Université de Tours-Institut National de la Santé et de la Recherche Médicale (INSERM), and Université de Tours (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM)
Subjects: Pulmonary and Respiratory Medicine, Gynecology, 03 medical and health sciences, medicine.medical_specialty, 0302 clinical medicine, 030228 respiratory system, business.industry, Granulomatous disease, [SDV]Life Sciences [q-bio], Medicine, 030212 general & internal medicine, business, 3. Good health
Abstract: Resume Introduction Il existe peu de donnees concernant l’epidemiologie de la berylliose pulmonaire chronique (BPC) en France. L’objectif de cette etude etait de denombrer les cas de BPC prevalents en France pendant la periode allant de 2010 a 2014. Methodes Une enquete nationale a ete realisee en France a l’aide d’un questionnaire specifique diffuse aupres des services de pneumologie et de pathologie professionnelle. Resultats Nous avons identifie 33 cas de BPC en France qui avaient ete diagnostiques entre 1982 et 2014. L’exposition etait professionnelle dans 28/33 cas (85 %) et concernait majoritairement les metiers de la fonderie (39 % des cas). Un diagnostic de certitude etait pose chez 29/33 cas (88 %) avec l’association d’une hypersensibilite au Be et d’une granulomatose, les 4 cas restant etaient des BPC probables. Le diagnostic de granulomatose survenait en moyenne 4 ans apres la fin de l’exposition. Le delai median entre le diagnostic de granulomatose et le diagnostic de berylliose etait de 2 ans (0 a 38 ans). Nous retrouvions une predisposition genetique chez 14 des 17 patients testes (82 %). Conclusion Cette etude a permis de recenser 33 cas de BPC suivis en France entre 2010 et 2014. La meconnaissance de la BPC et des expositions favorisantes, l’apparition tardive apres l’arret des expositions, la complexite du diagnostic et les similitudes avec la sarcoidose pourraient expliquer le faible nombre de cas recenses.
Published: 2020
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12. Rituximab and mycophenolate mofetil combination in patients with interstitial lung disease (EVER-ILD): a double-blind, randomised, placebo-controlled trial

Author: Julie Mankikian, Agnès Caille, Martine Reynaud-Gaubert, Marie-Sara Agier, Julien Bermudez, Philippe Bonniaud, Raphael Borie, Pierre-Yves Brillet, Jacques Cadranel, Isabelle Court-Fortune, Bruno Crestani, Marie-Pierre Debray, Emmanuel Gomez, Anne Gondouin, Sandrine Hirschi-Santelmo, Dominique Israel-Biet, Stéphane Jouneau, Karine Juvin, Julie Leger, Mallorie Kerjouan, Charles-Hugo Marquette, Jean-Marc Naccache, Hilario Nunes, Laurent Plantier, Grégoire Prevot, Sébastien Quetant, Julie Traclet, Victor Valentin, Yurdagul Uzunhan, Lidwine Wémeau-Stervinou, Theodora Bejan-Angoulvant, Vincent Cottin, Sylvain Marchand-Adam, CHU Trousseau [Tours], Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), MethodS in Patients-centered outcomes and HEalth ResEarch (SPHERE), Université de Tours (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Nantes Université - UFR des Sciences Pharmaceutiques et Biologiques (Nantes Univ - UFR Pharmacie), Nantes Université - pôle Santé, Nantes Université (Nantes Univ)-Nantes Université (Nantes Univ)-Nantes Université - pôle Santé, Nantes Université (Nantes Univ)-Nantes Université (Nantes Univ), Centre d’Investigation Clinique [Tours] CIC 1415 (CIC ), Centre Hospitalier Régional Universitaire de Tours (CHRU Tours)-Hôpital Bretonneau-Université de Tours (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM), Hôpital Nord [CHU - APHM], Aix Marseille Université (AMU), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Lipides - Nutrition - Cancer [Dijon - U1231] (LNC), Université de Bourgogne (UB)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut Agro Dijon, Institut national d'enseignement supérieur pour l'agriculture, l'alimentation et l'environnement (Institut Agro)-Institut national d'enseignement supérieur pour l'agriculture, l'alimentation et l'environnement (Institut Agro), Physiopathologie et Epidémiologie des Maladies Respiratoires (PHERE (UMR_S_1152 / U1152)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité), Laboratoire d'Excellence INFLAMEX [Paris], Université Sorbonne Paris Cité (USPC), AP-HP - Hôpital Bichat - Claude Bernard [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Université Paris 13 (UP13), Hôpital Avicenne [AP-HP], CHU Tenon [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Sorbonne Université (SU), Biologie Intégrative du Tissu Osseux (LBTO), Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM), Santé Ingénierie Biologie Saint-Etienne (SAINBIOSE), Centre Ingénierie et Santé (CIS-ENSMSE), École des Mines de Saint-Étienne (Mines Saint-Étienne MSE), Institut Mines-Télécom [Paris] (IMT)-Institut Mines-Télécom [Paris] (IMT)-École des Mines de Saint-Étienne (Mines Saint-Étienne MSE), Institut Mines-Télécom [Paris] (IMT)-Institut Mines-Télécom [Paris] (IMT)-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM), Les Hôpitaux Universitaires de Strasbourg (HUS), Nouvel Hôpital Civil de Strasbourg, 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), Hôpital Pontchaillou, 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 ), Centre Hospitalier Universitaire de Nice (CHU Nice), Centre hospitalier Saint-Joseph [Paris], Service de pneumologie [Rennes] = Pneumology [Rennes], CHU Pontchaillou [Rennes], 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), CHU Grenoble, Hôpital Louis Pradel [CHU - HCL], Hospices Civils de Lyon (HCL), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon, CHU Lille, EA4245 - Transplantation, Immunologie, Inflammation [Tours] (T2i), Université de Tours (UT), PHRC national, and This study was supported by a grant from the French Ministry of Health (Programme Hospitalier de Recherche Clinique) 2015
Subjects: Pulmonary and Respiratory Medicine, [SDV.IMM.IMM]Life Sciences [q-bio]/Immunology/Immunotherapy, [SDV.MHEP.PSR]Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract
Abstract: BackgroundStandard of care for interstitial lung disease (ILD) with a nonspecific interstitial pneumonia (NSIP) pattern proposes mycophenolate mofetil (MMF) as one of the first step therapies while rituximab is used as rescue therapy.MethodsIn a randomised, double blind, two-parallel group, placebo-controlled trial (NCT02990286), patients with connective tissue disease-associated ILD or idiopathic interstitial pneumonia (with or without autoimmune feature) and a NSIP pattern (defined on NSIP pathological pattern or on integration of clinico-biological data and a NSIP-like HRCT pattern) were randomly assigned in a 1:1 ratio to receive rituximab (1000 mg) or placebo on day 1 and day 15 in addition to MMF (2 g daily) for six months. The primary endpoint was the change in percent of predicted forced vital capacity (FVC) from baseline to 6 months analysed by a linear mixed model for repeated measures analysis. Secondary endpoints included progression-free survival (PFS) up to 6 months and safety.FindingsBetween January 2017 and January 2019, 122 randomised patients received at least one dose of rituximab (n=63) or placebo (n=59). The least-squares mean change from baseline to 6 months in FVC (% predicted) was +1.60 (se1.13) in the rituximab+MMF group and −2.01 (se1.17) in the placebo+MMF group (between-group difference, 3.60 [95% CI 0.41 to 6.80]; p=0.0273). PFS was better in the rituximab+MMF group (crude HR 0.47 [95%CI 0.23 to 0.96]; p=0.03). Serious adverse events occurred in 26 patients of the rituximab+MMF group (41%) and in 23 of the placebo+MMF group (39%). Nine infections were reported in the rituximab+MMF group (five bacterial infections, 3 viral infections, 1 other) and four bacterial infections in the placebo+MMF group.InterpretationCombination of rituximab and MMF was superior to MMF alone in patients with ILD and a NSIP pattern. The use of this combination must consider the risk of viral infection.
Published: 2023
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13. Inhaled ciclesonide for outpatient treatment of COVID-19 in adults at risk of adverse outcomes: a randomised controlled trial (COVERAGE)

Author: Alexandre Duvignaud, Edouard Lhomme, Racha Onaisi, Rémi Sitta, Ambre Gelley, Julie Chastang, Lionel Piroth, Christine Binquet, Julie Dupouy, Alain Makinson, Benjamin Lefèvre, Jean-Marc Naccache, Caroline Roussillon, Roland Landman, Cédrick Wallet, Sophie Karcher, Valérie Journot, Duc Nguyen, Thierry Pistone, Stéphane Bouchet, Marie-Edith Lafon, Mathieu Molimard, Rodolphe Thiébaut, Xavier de Lamballerie, Jean-Philippe Joseph, Laura Richert, Olivier Saint-Lary, Sarah Djabarouti, Linda Wittkop, Xavier Anglaret, Denis Malvy, Bordeaux population health (BPH), Université de Bordeaux (UB)-Institut de Santé Publique, d'Épidémiologie et de Développement (ISPED)-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Bordeaux [Bordeaux], Statistics In System biology and Translational Medicine (SISTM), Inria Bordeaux - Sud-Ouest, Institut National de Recherche en Informatique et en Automatique (Inria)-Institut National de Recherche en Informatique et en Automatique (Inria)- Bordeaux population health (BPH), Université de Bordeaux (UB)-Institut de Santé Publique, d'Épidémiologie et de Développement (ISPED)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Bordeaux (UB)-Institut de Santé Publique, d'Épidémiologie et de Développement (ISPED)-Institut National de la Santé et de la Recherche Médicale (INSERM), CIC Bordeaux, Université Bordeaux Segalen - Bordeaux 2-Institut National de la Santé et de la Recherche Médicale (INSERM), Université de Bordeaux (UB), Institut Pierre Louis d'Epidémiologie et de Santé Publique (iPLESP), Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), Sorbonne Université - Département de médecine générale, Sorbonne Université (SU), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Centre d'Investigation Clinique 1432 (Dijon) - Epidemiologie Clinique/Essais Cliniques (CIC-EC), Université de Bourgogne (UB)-Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre d'Epidémiologie et de Recherche en santé des POPulations (CERPOP), Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Institut National de la Santé et de la Recherche Médicale (INSERM), Université Fédérale Toulouse Midi-Pyrénées, CIC Montpellier, Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Hôpital Saint Eloi (CHRU Montpellier), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Montpellier, Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Département Maladies Infectieuses et Tropicales, Hôpital Universitaire, Montpellier, France, Adaptation, mesure et évaluation en santé. Approches interdisciplinaires (APEMAC), Université de Lorraine (UL), Service des Maladies Infectieuses et Tropicales [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Groupe Hospitalier Paris Saint-Joseph (hpsj), Infection, Anti-microbiens, Modélisation, Evolution (IAME (UMR_S_1137 / U1137)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité)-Université Sorbonne Paris Nord, Institut de médecine et d'épidémiologie appliquée [AP-HP Hôpital Bichat-Claude Bernard] (IMEA), 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), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Microbiologie Fondamentale et Pathogénicité (MFP), Université Bordeaux Segalen - Bordeaux 2-Centre National de la Recherche Scientifique (CNRS), Unité des Virus Emergents (UVE), Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre de recherche en épidémiologie et santé des populations (CESP), Université de Versailles Saint-Quentin-en-Yvelines (UVSQ)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Paul Brousse-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris-Saclay, Université de Versailles Saint-Quentin-en-Yvelines - UFR Sciences de la santé Simone Veil (UVSQ Santé), Université de Versailles Saint-Quentin-en-Yvelines (UVSQ), Biologie des maladies cardiovasculaires = Biology of Cardiovascular Diseases, Université de Bordeaux (UB)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), ANR-20-COVI-0040,COVERAGE,Traitement à domicile des personnes infectées par le SRAS-CoV-2 sans signe de gravité mais à risque de complications: un essai randomisé à plusieurs bras et en plusieurs étapes (MAMS) pour évaluer l'efficacité de plusieurs antiviraux(2020), Richert, Laura, Traitement à domicile des personnes infectées par le SRAS-CoV-2 sans signe de gravité mais à risque de complications: un essai randomisé à plusieurs bras et en plusieurs étapes (MAMS) pour évaluer l'efficacité de plusieurs antiviraux - - COVERAGE2020 - ANR-20-COVI-0040 - COVID-19 - VALID, Université de Toulouse (UT)-Université de Toulouse (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM), and Université de Toulouse (UT)
Subjects: Microbiology (medical), Male, [SDV.SP.MED] Life Sciences [q-bio]/Pharmaceutical sciences/Medication, Ciclesonide, [SDV.SP.MED]Life Sciences [q-bio]/Pharmaceutical sciences/Medication, Pregnenediones, Outpatients, Adults, Humans, Aged, [SDV.MP.VIR] Life Sciences [q-bio]/Microbiology and Parasitology/Virology, Inhaled corticosteroids, SARS-CoV-2, COVID-19, General Medicine, Middle Aged, COVID-19 Drug Treatment, Treatment, Oxygen, Infectious Diseases, Treatment Outcome, Randomized controlled trial, [SDV.SPEE] Life Sciences [q-bio]/Santé publique et épidémiologie, [SDV.MP.VIR]Life Sciences [q-bio]/Microbiology and Parasitology/Virology, [SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie, Female
Abstract: International audience; Objectives: To assess the efficacy of inhaled ciclesonide in reducing the risk of adverse outcomes in COVID-19 outpatients at risk of developing severe illness.Methods: COVERAGE is an open-label, randomized controlled trial. Outpatients with documented COVID-19, risk factors for aggravation, symptoms for ≤7 days, and absence of criteria for hospitalization are randomly allocated to either a control arm or one of several experimental arms, including inhaled ciclesonide. The primary efficacy endpoint is COVID-19 worsening (hospitalization, oxygen therapy at home, or death) by Day 14. Other endpoints are adverse events, maximal follow-up score on the WHO Ordinal Scale for Clinical Improvement, sustained alleviation of symptoms, cure, and RT-PCR and blood parameter evolution at Day 7. The trial's Safety Monitoring Board reviewed the first interim analysis of the ciclesonide arm and recommended halting it for futility. The results of this analysis are reported here.Results: The analysis involved 217 participants (control 107, ciclesonide 110), including 111 women and 106 men. Their median age was 63 years (interquartile range 59-68), and 157 of 217 (72.4%) had at least one comorbidity. The median time since first symptom was 4 days (interquartile range 3-5). During the 28-day follow-up, 2 participants died (control 2/107 [1.9%], ciclesonide 0), 4 received oxygen therapy at home and were not hospitalized (control 2/107 [1.9%], ciclesonide 2/110 [1.8%]), and 24 were hospitalized (control 10/107 [9.3%], ciclesonide 14/110 [12.7%]). In intent-to-treat analysis of observed data, 26 participants reached the composite primary endpoint by Day 14, including 12 of 106 (11.3%, 95% CI: 6.0%-18.9%) in the control arm and 14 of 106 (13.2%; 95% CI: 7.4-21.2%) in the ciclesonide arm. Secondary outcomes were similar for both arms.Discussion: Our findings are consistent with the European Medicines Agency's COVID-19 task force statement that there is currently insufficient evidence that inhaled corticosteroids are beneficial for patients with COVID-19.
Published: 2021
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14. Authors' reply

Author: Jean-Marc Naccache, Marine Cachanado, Alexandra Rousseau, and Hilario Nunes
Subjects: Pulmonary and Respiratory Medicine
Published: 2021

15. Exposure to inorganic particles in paediatric sarcoidosis: the PEDIASARC study

Author: Véronique Houdouin, Catherine Cavalin, Antoine Deschildre, Jean-Marc Naccache, Catherine Chapelon-Abric, Céline Delestrain, Julien Haroche, Ralph Epaud, Ulrich Meinzer, Simon Chauveau, Fleur Cohen-Aubart, Mickaël Catinon, Florence Jeny, Odile Macchi, Sylvain Le Jeune, Hilario Nunes, Michel Vincent, Paul-André Rosental, Manon Brocvielle, Marie-Hélène Odièvre, Marie-Emeline Montagne, S. Mattioni, Dominique Valeyre, Karine Juvin, Annick Clement, Dominique Israël-Biet, A. Dossier, Nadia Nathan, Jacques Brouard, François Lionnet, Lucile Sesé, Rola Abou Taam, Christophe Delacourt, Marie Mittaine, CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Institut de Recherche Interdisciplinaire en Sciences Sociales (IRISSO), Université Paris Dauphine-PSL, Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE), Groupe de Recherche sur l'Adaptation Microbienne (GRAM 2.0), Université de Caen Normandie (UNICAEN), Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU), 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), Service de Département de médecine interne et immunologie clinique [CHU Pitié-Salpêtrière] (DMIIC), CHU Pitié-Salpêtrière [AP-HP], 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), Centre d'Immunologie et des Maladies Infectieuses (CIMI), Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS), Pneumologie et allergologie pédiatriques [CHU Jeanne de Flandre, Lille], Hôpital Jeanne de Flandre [Lille], Université de Lille, Service de Pneumologie et d'Allergologie Pédiatriques, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Biomécanique cellulaire et respiratoire (BCR), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12)-Centre National de la Recherche Scientifique (CNRS), Immunologie humaine, physiopathologie & immunothérapie (HIPI (UMR_S_976 / U976)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPC), Service de Pneumologie, soins intensifs (Pneumo - HEGP), 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)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), Service de Médecine Interne = Hôpital de jour de médecine [CHU Tenon], CHU Tenon [AP-HP], Centre d'études européennes et de politique comparée (Sciences Po, CNRS) (CEE), Sciences Po (Sciences Po)-Centre National de la Recherche Scientifique (CNRS), Centre de recherche sur l'Inflammation (CRI (UMR_S_1149 / ERL_8252 / U1149)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Paris Cité (UPC), Centre d'investigation clinique de Toulouse (CIC 1436), Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-CHU Toulouse [Toulouse]-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre de référence maladies rares des maladies pulmonaires rares de l’adulte (CHU Dijon) (CRMR des maladies pulmonaires rares de l’adulte), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Hypoxie et Poumon : pneumopathologies fibrosantes, modulations ventilatoires et circulatoires (H&P), UFR SMBH-Université Sorbonne Paris Nord, Biologie Intégrée du Globule Rouge (BIGR (UMR_S_1134 / U1134)), Institut National de la Transfusion Sanguine [Paris] (INTS)-Université de La Réunion (UR)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pointe-à-Pitre/Abymes [Guadeloupe] -Université des Antilles (UA)-Université Paris Cité (UPC), Centre d'histoire de Sciences Po (Sciences Po) (CHSP), Sciences Po (Sciences Po), Maladies génétiques d'expression pédiatrique (U933), Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Trousseau [APHP], Observatoire du Samu Social de Paris, CHU Necker - Enfants Malades [AP-HP], CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN), Centre des maladies respiratoires rares Respirare [CHI Créteil], Institut Mondor de Recherche Biomédicale (IMRB), Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR10-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), AP-HP - Hôpital Bichat - Claude Bernard [Paris], AP-HP Hôpital universitaire Robert-Debré [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), Service de médecine interne [Avicenne], Centre de Référence pour les Maladies Rhumatologiques Auto-Immunes et Systémiques [CHU Necker] (RAISE), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Service de pneumologie [Hôpital Foch], Hôpital Foch [Suresnes], Groupe Hospitalier Paris Saint-Joseph (hpsj), Laboratoire interdisciplinaire d'évaluation des politiques publiques (Sciences Po) (LIEPP), Centre d'études de l'emploi et du travail (CEET), Conservatoire National des Arts et Métiers [CNAM] (CNAM), HESAM Université - Communauté d'universités et d'établissements Hautes écoles Sorbonne Arts et métiers université (HESAM)-HESAM Université - Communauté d'universités et d'établissements Hautes écoles Sorbonne Arts et métiers université (HESAM)-Ministère de l'Education nationale, de l’Enseignement supérieur et de la Recherche (M.E.N.E.S.R.)-Ministère du Travail, de l'Emploi et de la Santé, contract grant sponsor: European Research Council (ERC) / SILICOSISproject / Principal investigator: Paul-André Rosental, Contract grant number: ERC2011-ADG_20110406ˆProject ID: 295817. Statistical analysis: RespiFIL funds, European Project: 295449,EC:FP7:ERC,ERC-2011-ADG_20110406,DARTCH(2012), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Paris (UP), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université de Paris (UP), Institut National de la Transfusion Sanguine [Paris] (INTS)-Université de La Réunion (UR)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pointe-à-Pitre/Abymes [Guadeloupe] -Université des Antilles (UA)-Université de Paris (UP), Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Normandie Université (NU)-Université de Caen Normandie (UNICAEN), Centre d'Immunologie et de Maladies Infectieuses (CIMI), Service de Médecine Interne [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Centre d'études européennes et de politique comparée (CEE), Centre d'histoire de Sciences Po (CHSP), Service de médecine interne et d'immunologie clinique [CHU Pitié-Salpêtrière], and Centre National de la Recherche Scientifique (CNRS)-Sciences Po (Sciences Po)
Subjects: Pulmonary and Respiratory Medicine, Adult, medicine.medical_specialty, paediatric interstitial lung disease, [SDV]Life Sciences [q-bio], [SHS]Humanities and Social Sciences, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, Occupational Exposure, medicine, Humans, sarcoidosis, Occupational lung disease, Occupations, Child, Inorganic particles, 030304 developmental biology, 0303 health sciences, Median score, rare lung diseases, business.industry, Dust, Environmental exposure, Environmental Exposure, medicine.disease, 3. Good health, 030228 respiratory system, Metal dust, Talc, Sarcoidosis, business, occupational lung disease
Abstract: International audience; Inorganic antigens may contribute to paediatric sarcoidosis. Thirty-six patients matched with 36 healthy controls as well as a group of 21 sickle-cell disease (SCD) controls answered an environmental questionnaire. Patients' indirect exposure to inorganic particles, through coresidents' occupations, was higher than in healthy and SCD controls (median score: 2.5 (0.5-7) vs 0.5 (0-2), p=0.003 and 1 (0-2), p=0.012, respectively), especially for construction, exposures to metal dust, talc, abrasive reagents and scouring products. Wood or fossil energies heating were also linked to paediatric sarcoidosis. This study supports a link between mineral environmental exposure due to adult coresident occupations and paediatric sarcoidosis.
Published: 2021
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16. Interstitial lung diseases associated with mutations of poly(A)-specific ribonuclease: A multicentre retrospective study

Author: Quentin, Philippot, Caroline, Kannengiesser, Marie Pierre, Debray, Clément, Gauvain, Ibrahima, Ba, Margherita, Vieri, Anne, Gondouin, Jean-Marc, Naccache, Martine, Reynaud-Gaubert, Yurdagul, Uzunhan, Benjamin, Bondue, Dominique, Israël-Biet, Philippe, Dieudé, Cécile, Fourrage, Elodie, Lainey, Effrosyne, Manali, Spyros, Papiris, Lidwine, Wemeau, Sandrine, Hirschi, Hervé, Mal, Hilario, Nunes, Frédéric, Schlemmer, Elodie, Blanchard, Fabian, Beier, Vincent, Cottin, Bruno, Crestani, and Raphaël, Borie
Subjects: Pulmonary and Respiratory Medicine, Exoribonucleases, Mutation, Humans, Lung Diseases, Interstitial, Idiopathic Pulmonary Fibrosis, Retrospective Studies
Abstract: Poly(A)-specific ribonuclease (PARN) mutations have been associated with familial pulmonary fibrosis. This study aims to describe the phenotype of patients with interstitial lung disease (ILD) and heterozygous PARN mutations.We performed a retrospective, observational, non-interventional study of patients with an ILD diagnosis and a pathogenic heterozygous PARN mutation followed up in a centre of the OrphaLung network.We included 31 patients (29 from 16 kindreds and two sporadic patients). The median age at ILD diagnosis was 59 years (range 54 to 63). In total, 23 (74%) patients had a smoking history and/or fibrogenic exposure. The pulmonary phenotypes were heterogenous, but the most frequent diagnosis was idiopathic pulmonary fibrosis (n = 12, 39%). Haematological abnormalities were identified in three patients and liver disease in two. In total, 21 patients received a specific treatment for ILD: steroids (n = 13), antifibrotic agents (n = 11), immunosuppressants (n = 5) and N-acetyl cysteine (n = 2). The median forced vital capacity decline for the whole sample was 256 ml/year (range -363 to -148). After a median follow-up of 32 months (range 18 to 66), 10 patients had died and six had undergone lung transplantation. The median transplantation-free survival was 54 months (95% CI 29 to ∞). Extra-pulmonary features were less frequent with PARN mutation than telomerase reverse transcriptase (TERT) or telomerase RNA component (TERC) mutation.IPF is common among individuals with PARN mutation, but other ILD subtypes may be observed.
Published: 2021

17. Mineral exposures in pediatric sarcoidosis

Author: Jean-Marc Naccache, François Lionnet, Lucile Sesé, Ulrich Meinzer, Fleur Cohen Aubart, Karine Juvin, Marie-Hélène Odièvre, Véronique Houdouin, Catherine Cavalin, Antoine Deschildre, Dominique Valeyre, Dominique Israel-Biet, Paul-André Rosental, Florence Jeny, Michel Vincent, Sylvain Le Jeune, Annick Clement, Jacques Brouard, Nadia Nathan, Ralph Epaud, Odile Macchi, Simon Chauveau, S. Mattioni, Mickaël Catinon, Céline Delestrain, Marie-Emeline Montagne, Julien Haroche, Hilario Nunes, Rola Abou Taam, Catherine Chapelon-Abric, Christophe Delacourt, Marie Mittaine, and A. Dossier
Subjects: medicine.medical_specialty, Mineral, business.industry, medicine, Sarcoidosis, medicine.disease, business, Dermatology
Published: 2021
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18. RCT Abstract - Cyclophosphamide added to glucocorticoids in acute exacerbation of idiopathic pulmonary fibrosis (EXAFIP): a randomized, double-blind, placebo-controlled, phase 3 trial

Author: Jean-Marc Naccache, Morgane Didier, Hilario Nunes, Julie Traclet, Olivia Freynet, Dominique Israel-Biet, Alexandre Chabrol, Arnaud Bourdin, Nathalie Bautin, Emmanuel Bergot, Grégoire Prévot, Jean Pastré, Sylvie Leroy, Philippe Bonniaud, Tristan Dégot, Frédéric Rivière, Raphael Borie, Cécile Chenivesse, Vincent Cottin, Lidwine Wemeau-Stervinou, Bruno Crestani, Martine Reynaud-Gaubert, Antoine Parrot, Dominique Valeyre, Marie-Christine Dombret, Malorie Kerjouan, Abdellatif Tazi, Anne Gondouin, Stéphane Jouneau, Elodie Blanchard, Sylvain Marchand-Adam, Sandrine Hirschi, C. Launois, Ana Nieves, Stéphane Dominique, François-Xavier Blanc, Jacques Cadranel, Marie-Pierre Debray, Anne-Sophie Gamez, Frédéric Gagnadoux, Marine Cachanado, Laurence Berard, Guillaume Beltramo, Tabassome Simon, Pierre-Yves Brillet, A. Gibelin, Emmanuel Gomez, Aurélie Le Borgne-Krams, François Lebargy, Alexandra Rousseau, and Laurent Plantier
Subjects: medicine.medical_specialty, Cyclophosphamide, Exacerbation, business.industry, medicine.disease, Placebo, Gastroenterology, law.invention, Double blind, Idiopathic pulmonary fibrosis, Randomized controlled trial, law, Internal medicine, Medicine, business, medicine.drug
Published: 2021
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19. Interstitial Lung Disease in Werner Syndrome: A Case Report of a 55-Year-Old Male Patient

Author: Tiphaine Goletto, Flora Crockett, Selim Aractingi, Cecile Toper, Patricia Senet, Jacques Cadranel, and Jean-Marc Naccache
Subjects: Diseases of the respiratory system, RC705-779
Abstract: Werner syndrome (WS) is a progeroid or premature aging syndrome characterized by early onset of age-related pathologies and cancer. The average life expectancy of affected people is 52.8 years and tends to increase. The major causes of death are malignancy and myocardial infarction. Increased telomere attrition and decay are thought to play a causative role in the clinical and pathological manifestations of the disease. Although telomere length, with or without germline mutation, is known to be associated with interstitial lung disease, the latter is not associated with WS. To the best of our knowledge, we report the first case describing a WS patient with fatal ILD. This case suggests that older patients with WS could develop ILD. Clinical outcome of WS patients may thus be improved by counselling them regarding smoking cessation or other exposure and by proposing antifibrotic therapy.
Published: 2015
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20. Cryobiopsies trans-bronchiques au cours des pneumopathies interstielles diffuses – expériences préliminaires

Author: M. Febvre, O. Ouennoure, J. Camuset, Jalal Assouad, Jean-Marc Naccache, F. Wallyn, Marie-Christine Copin, Martine Antoine, C. Fournier, Jacques Cadranel, Xavier Dhalluin, and CHU Lille
Subjects: Pulmonary and Respiratory Medicine, Gynecology, Rigid bronchoscopy, medicine.medical_specialty, Lung, business.industry, [SDV]Life Sciences [q-bio], 3. Good health, Interventional pulmonology, 03 medical and health sciences, 0302 clinical medicine, medicine.anatomical_structure, 030228 respiratory system, Medicine, 030212 general & internal medicine, business
Abstract: Resume Introduction Dans l’approche diagnostique des pneumopathies interstitielles diffuses (PID), les cryobiopsies trans-bronchiques (cryo-BTB) apparaissent etre une alternative a la biopsie pulmonaire chirurgicale (BPC). Nous rapportons la rentabilite des cryo-BTB et leur securite pour le diagnostic de PID avec l’experience preliminaire de 2 centres universitaires francais. Methodes Vingt-quatre patients ont beneficie de cryo-BTB pour le diagnostic de PID au bloc operatoire entre 2014 et 2017. Tous les diagnostics histologiques obtenus etaient ensuite valides lors de discussions multidisciplinaires (DMD). Resultats Les patients ont eu en moyenne 3 cryo-BTB. Les cryo-BTB etaient analysables chez 22/24 (91,7 %) patients, permettant un diagnostic histologique chez 14/22 (63,6 %) patients et un diagnostic de certitude dans 13/22 (59 %) cas apres DMD. La rentabilite diagnostique globale des cryo-BTB etait de 13/24 (54,2 %). En tenant compte de la courbe d’apprentissage pour les 15 patients ayant eu leurs cryo-BTB apres 2015, on note un diagnostic apres DMD pour 12 d’entre eux soit 80 %. Neuf (37,5 %) patients ont eu un pneumothorax. Cinq (20,8 %) patients ont eu un saignement. Il n’y a eu aucun deces. Conclusion Une etude prospective randomisee permettrait d’evaluer la technique en France afin de preciser son rendement diagnostique et son profil de securite en comparaison a la BPC.
Published: 2019
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21. Diagnosis Yield and Safety of Surgical Biopsy in Interstitial Lung Diseases: A Prospective Study

Author: Anne Charpentier, Denis Debrosse, Pierre Mordant, Olivia Freynet, Hilario Nunes, Yves Castier, Emmanuel Martinod, Jean Pierre L'Huillier, Marie-Pierre Debray, Françoise Le Pimpec-Barthes, Martine Antoine, Karine Juvin, Dana M. Radu, Pierre-Yves Brillet, Jean-Marc Naccache, Vincent Lévy, Sebastian Tavolaro, Mohammad Zaahid Noorah, Patrice Guiraudet, Dominique Valeyre, Sadek Beloucif, Clairelyne Dupin, Aurélie Cazes, Dominique Israël-Biet, Yurdagul Uzunhan, Marianne Kambouchner, I. Honoré, Marouane Boubaya, Jalal Assouad, Laure Gibault, Marco Alifano, Bruno Crestani, and Pauline Pradère
Subjects: Pulmonary and Respiratory Medicine, High-resolution computed tomography, medicine.medical_specialty, Vital capacity, medicine.diagnostic_test, business.industry, Thoracic Surgery, Video-Assisted, Biopsy, Interstitial lung disease, Lung biopsy, medicine.disease, Surgery, Pulmonary function testing, Idiopathic pulmonary fibrosis, FEV1/FVC ratio, medicine, Humans, Prospective Studies, Cardiology and Cardiovascular Medicine, Prospective cohort study, business, Lung Diseases, Interstitial, Lung, Retrospective Studies
Abstract: Background Surgical lung biopsy is essential in the diagnostic algorithm of interstitial lung disease (ILD) of unknown cause. Safety concerns have been recently reiterated. The aim of this study was to prospectively assess the yield of diagnosis and safety of video-assisted thoracoscopic surgical lung biopsy (VATS-LB) for ILD diagnosis. Methods This prospective study, conducted in 6 ILD-referral Paris hospitals, included 103 patients with ILD. After initial multidisciplinary discussion, VATS-LB was proposed. A final diagnosis was made after the procedure, during a second multidisciplinary discussion. The main outcome was to determine the final diagnoses and their proportion after VATS-LB. Other outcomes were the percentage of change in diagnosis and treatment propositions after VATS-LB and adverse events during 3 months after surgery, postoperative pulmonary function, quality of life and pain. Results A definite diagnosis was reached in 87 cases (84.4%), while 16 remained unclassifiable (15.6%). Hypothesized diagnosis and treatment changed after VATS-LB in 65 (63.1%) and in 41 patients (39.8%), respectively. There was one death due to acute exacerbation. In-hospital complications were predicted by a lower preoperative distance at 6-minute walking test and by forced vital capacity lower than 77%. Postoperative quality of life was not modified at 3 months while forced vital capacity decreased slightly. Postoperative neuropathic pain was revealed in 5% and 2% patients at 1 and 3 months, respectively. Conclusions VATS-LB dramatically changed preoperative hypothetical diagnoses and treatment in ILD of unknown cause with good patient survival in ILD-referral centers.
Published: 2021

22. Diffuse cystic lung disease in sickle cell anaemia: a series of 22 cases and a case-control study

Author: Laurent Savale, Anoosha Habibi, Bernard Maitre, Marie-France Carette, Jean-Marc Naccache, Antoine Parrot, Frédéric Schlemmer, Hilario Nunes, Fatma Kort, and François Lionnet
Subjects: Pulmonary and Respiratory Medicine, medicine.medical_specialty, Vital Capacity, Lung biopsy, Anemia, Sickle Cell, Gastroenterology, Pulmonary function testing, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, medicine, Humans, Pathological, Index case, Lung, 030304 developmental biology, 0303 health sciences, business.industry, Case-control study, respiratory system, medicine.disease, Constrictive Bronchiolitis, Acute chest syndrome, medicine.anatomical_structure, 030228 respiratory system, Case-Control Studies, business, Lung Diseases, Interstitial
Abstract: Chronic interstitial lung abnormalities have been described in sickle cell disease (SCD) and attributed to repetitive episode of acute chest syndrome. We report a series of 22 cases of diffuse cystic lung disease in SCD with a case–control study to hunt for mechanism. On pathological analysis of a surgical lung biopsy of the index case, the bronchioles had the appearance of constrictive bronchiolitis. Pulmonary function test results revealed lower forced expiratory flow from 25% to 75% of vital capacity in cases versus controls. These findings suggest a bronchiolar mechanism that was not associated with more acute chest syndrome.
Published: 2020

23. Interstitial lung diseases associated with mutations of poly(A)-specific ribonuclease: a multicentric retrospective cohort study

Author: Bruno Crestani, Matthieu Rigaud, Dominique Israel Biet, Quentin Philippot, Hervé Mal, Benjamin Bondue, Spyros Papiris, Anne Gondouin, Jean-Marc Naccache, Yurdagul Uzunhan, Martine Reynaud-Gobert, Vincent Cottin, Lidwine Wemeau, Caroline Kannengiesser, Raphael Borie, Frédéric Schlemmer, Sandrine Hirschi, Hilario Nunes, Effrosyne Manali, and Ibrahima Ba
Subjects: medicine.medical_specialty, education.field_of_study, Genetic heterogeneity, business.industry, medicine.medical_treatment, Population, Interstitial lung disease, Retrospective cohort study, respiratory system, medicine.disease, Gastroenterology, respiratory tract diseases, Idiopathic pulmonary fibrosis, FEV1/FVC ratio, Internal medicine, Pulmonary fibrosis, medicine, Lung transplantation, business, education
Abstract: Poly(A)-specific ribonuclease (PARN) mutations have been associated with familial pulmonary fibrosis. However, the phenotype of patients with interstitial lung disease (ILD) and PARN mutations is poorly described. We performed a retrospective, observational, non-interventional study. All the patients with an ILD diagnosis and a pathogenic heterozygous PARN mutation, followed in one of the OrphaLung center, were included. We included 30 patients (24 patients from 11 kindreds and 6 sporadic patients with early onset of ILD or extra-pulmonary phenotype). The median age at diagnosis of ILD was 59 years (range 54 - 64). Twenty-three patients (77 %) had a smoking history and/or a fibrogenic exposure. The pulmonary phenotypes were heterogenous but the most frequent diagnosis was idiopathic pulmonary fibrosis (IPF; n=15, 50%). Hematological abnormalities were identified in 3 patients and liver disease in 2 patients. Twenty-one patients received a specific treatment for ILD: steroids (n=13), antifibrotic (n= 11), immunosuppressants (n=5) and N-acetyl cysteine (n=2). The median decline of FVC for the whole population was 292 ml/year (range 146 - 657). After a median follow-up of 2.8 years 8 patients had died and 6 had undergone lung transplantation. The median transplantation-free survival was 4.5 years. PARN mutation carriers were older, and extra-pulmonary manifestations were less frequent as compared to TERT or TERC mutation carriers. PARN mutations are most frequently associated with IPF, but other ILD may be observed. Such phenotypic heterogeneity requires a thorough evaluation of PARN-associated ILD patients in order to define tailored therapeutic strategies.
Published: 2020
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24. Child–Adult Transition in Sarcoidosis: A Series of 52 Patients

Author: Simon Chauveau, Florence Jeny, Marie-Emeline Montagne, Rola Abou Taam, Véronique Houdouin, Ulrich Meinzer, Christophe Delacourt, Ralph Epaud, Fleur Cohen Aubart, Catherine Chapelon-Abric, Dominique Israël-Biet, Karine Juvin, Antoine Dossier, Bahram Bodaghi, Grégoire Prévot, Jean-Marc Naccache, Sarah Mattioni, Antoine Deschildre, Jacques Brouard, Abdellatif Tazi, Roderich Meckenstock, Morgane Didier, Julien Haroche, Annick Clement, Jean-François Bernaudin, Hilario Nunes, Dominique Valeyre, Nadia Nathan, and for the French Sarcoidosis Group (GSF)
Subjects: Pediatrics, medicine.medical_specialty, medicine.drug_class, Severe disease, lcsh:Medicine, Disease, 030204 cardiovascular system & hematology, Article, 03 medical and health sciences, 0302 clinical medicine, children, transition of care, 0502 economics and business, medicine, sarcoidosis, Adverse effect, interstitial lung disease, Series (stratigraphy), Cumulative dose, business.industry, 05 social sciences, lcsh:R, Interstitial lung disease, General Medicine, medicine.disease, Corticosteroid, 050211 marketing, Sarcoidosis, business
Abstract: (1) Background: Pediatric sarcoidosis is a rare and mostly severe disease. Very few pediatric series with a prolonged follow-up are reported. We aimed to evaluate the evolution of pediatric sarcoidosis in adulthood. (2) Material and methods: Patients over 18-years-old with a pediatric-onset sarcoidosis (&le, 15-year-old) who completed at least a three-year follow-up in French expert centers were included. Clinical information at presentation and outcome in adulthood were studied. (3) Results: A total of 52 patients were included (34 prospectively in childhood and 18 retrospectively in adulthood), with a mean age of 12 (&plusmn, 2.7) at diagnosis. The median duration time of follow-up was 11.5 years (range 3&ndash, 44.5). Relapses mostly occurred during treatment decrease (84.5%), others within the three years after treatment interruption (9.1%), and rarely when the disease was stable for more than three years (6.4%). Sarcoidosis was severe in 11 (21.2%) in adulthood. Patients received a high corticosteroid cumulative dose (median 17,900 mg) for a median duration of five years (range 0&ndash, 32), resulting in mostly mild (18, 35.3%) and rarely severe (2, 3.8%) adverse events. (4) Conclusions: Pediatric-onset sarcoidosis needed a long-term treatment in almost half of the patients. Around one fifth of pediatric-onset sarcoidosis patients had severe sarcoidosis consequences in adulthood.
Published: 2020

25. Anakinra for severe forms of COVID-19: a cohort study

Author: Gilles Hayem, Gilles Chatellier, Hélène Beaussier, Olivier Voisin, Yvonnick Bézie, Stéphane Jouveshomme, Sophie Laplanche, Joseph Emmerich, Gaëlle Dauriat, Thomas Huet, Emmanuelle Sacco, Jean-Jacques Mourad, Sergio Salmeron, Isabelle Lazareth, Alice Le Berre, Jean Marc Naccache, and Jérôme Le Pavec
Subjects: Mechanical ventilation, medicine.medical_specialty, Anakinra, business.industry, medicine.medical_treatment, Hazard ratio, Immunology, Intensive care unit, Article, law.invention, Rheumatology, law, Internal medicine, Cohort, medicine, Immunology and Allergy, Respiratory function, Prospective cohort study, business, medicine.drug, Cohort study
Abstract: Summary Background Coronaviruses can induce the production of interleukin (IL)-1β, IL-6, tumour necrosis factor, and other cytokines implicated in autoinflammatory disorders. It has been postulated that anakinra, a recombinant IL-1 receptor antagonist, might help to neutralise the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)-related hyperinflammatory state, which is considered to be one cause of acute respiratory distress among patients with COVID-19. We aimed to assess the off-label use of anakinra in patients who were admitted to hospital for severe forms of COVID-19 with symptoms indicative of worsening respiratory function. Methods The Ana-COVID study included a prospective cohort from Groupe Hospitalier Paris Saint-Joseph (Paris, France) and a historical control cohort retrospectively selected from the Groupe Hospitalier Paris Saint-Joseph COVID cohort, which began on March 18, 2020. Patients were included in the prospective cohort if they were aged 18 years or older and admitted to Groupe Hospitalier Paris Saint-Joseph with severe COVID-19-related bilateral pneumonia on chest x-ray or lung CT scan. The other inclusion criteria were either laboratory-confirmed SARS-CoV-2 or typical lung infiltrates on a lung CT scan, and either an oxygen saturation of 93% or less under oxygen 6 L/min or more, or aggravation (saturation ≤93% under oxygen 3 L/min) with a loss of 3% of oxygen saturation in ambient air over the previous 24 h. The historical control group of patients had the same inclusion criteria. Patients in the anakinra group were treated with subcutaneous anakinra (100 mg twice a day for 72 h, then 100 mg daily for 7 days) as well as the standard treatments at the institution at the time. Patients in the historical group received standard treatments and supportive care. The main outcome was a composite of either admission to the intensive care unit (ICU) for invasive mechanical ventilation or death. The main analysis was done on an intention-to-treat basis (including all patients in the anakinra group who received at least one injection of anakinra). Findings From March 24 to April 6, 2020, 52 consecutive patients were included in the anakinra group and 44 historical patients were identified in the Groupe Hospitalier Paris Saint-Joseph COVID cohort study. Admission to the ICU for invasive mechanical ventilation or death occurred in 13 (25%) patients in the anakinra group and 32 (73%) patients in the historical group (hazard ratio [HR] 0·22 [95% CI 0·11–0·41; p
Published: 2020
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26. Interstitial Lung Disease Associated with Lung Cancer: A Case–Control Study

Author: Pierre Levy, Christos Chouaid, Quentin Gibiot, Jean-Marc Naccache, Anne-Laure Brun, Martine Antoine, Isabelle Monnet, Jacques Cadranel, Service de Pneumologie [CHI Créteil], CHI Créteil, Institut Pierre Louis d'Epidémiologie et de Santé Publique (iPLESP), Sorbonne Université (SU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Radiologie [CHU Cochin], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service d’Anatomie et cytologie pathologiques [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), 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), Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), Service d'Anatomie et cytologie pathologiques [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), Service de Pneumologie = Pneumologie - Oncologie Thoracique - Maladies Pulmonaires Rares [CHU Tenon], and Gestionnaire, Hal Sorbonne Université
Subjects: medicine.medical_specialty, Population, retrospective, lcsh:Medicine, [SDV.CAN]Life Sciences [q-bio]/Cancer, outcomes, Gastroenterology, [SDV.MHEP.PSR]Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract, behavioral disciplines and activities, Article, 03 medical and health sciences, 0302 clinical medicine, [SDV.CAN] Life Sciences [q-bio]/Cancer, Internal medicine, medicine, education, Lung cancer, interstitial lung disease, education.field_of_study, business.industry, Hazard ratio, lcsh:R, Interstitial lung disease, Case-control study, General Medicine, respiratory system, medicine.disease, Confidence interval, 3. Good health, respiratory tract diseases, body regions, lung cancer, 030228 respiratory system, [SDV.SPEE] Life Sciences [q-bio]/Santé publique et épidémiologie, 030220 oncology & carcinogenesis, Cohort, [SDV.MHEP.PSR] Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract, [SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie, prognosis, business, Cohort study
Abstract: Interstitial lung disease (ILD) seems to be associated with an increased risk of lung cancer (LC) and to have a poorer prognosis than LC without ILD. The frequency of ILD in an LC cohort and its prognosis implication need to be better elucidated. This retrospective, observational, cohort study evaluated the frequency of ILD among LC patients (LC&ndash, ILD) diagnosed over a 2-year period. LC&ndash, ILD patients&rsquo, characteristics were compared to those with LC without ILD (LC&ndash, noILD). Lastly, we conducted a case&ndash, control study within this cohort, matching three LC&ndash, noILDs to each LC&ndash, ILD patient, to evaluate the ILD impact on LC patients&rsquo, prognoses. Among 906 LC patients, 49 (5.4%) also had ILD. Comparing LC&ndash, ILD to LC&ndash, noILD patients, respectively, more were men (85.7% vs. 66.2%, p = 0.02), adenocarcinomas were less frequent (47.1% vs. 58.7%, p = 0.08), median [range] and overall survival was shorter: (9 [range: 0.1&ndash, 39.4] vs. 17.5 [range: 0.8&ndash, 50.4] months, p = 0.01). Multivariate analysis (hazard ratio [95% confidence interval]) retained two factors independently associated with LC risk of death: ILD (1.79 [1.22&ndash, 2.62], p = 0.003) and standard-of-care management (0.49 [0.33&ndash, 0.72], p &lt, 0.001). Approximately 5% of patients with a new LC diagnosis had associated ILD. ILD was a major prognosis factor for LC and should be taken into consideration for LC management. Further studies are needed to determine the best therapeutic strategy for the LC&ndash, ILD population.
Published: 2020
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27. Hémorragies intra-alvéolaires auto-immune en rhumatologie

Author: Jacques Cadranel, Jean-Marc Naccache, A. Canellas, and Antoine Parrot
Subjects: 030203 arthritis & rheumatology, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, 030212 general & internal medicine
Abstract: Resume L’hemorragie intra-alveolaire (HIA) est une maladie rare et grave. Le diagnostic est evoque devant la triade hemoptysie, anemie et infiltrat radiologique. Il est confirme par la realisation d’une tomodensitometrie thoracique et d’une fibroscopie bronchique avec lavage broncho-alveolaire revelant des secretions uniformement hemorragiques. Les etiologies en sont nombreuses. Elles sont classiquement differenciees en cause « immune » (vascularites liees aux anticorps anti-cytoplasme des polynucleaires neutrophiles (ANCA), les connectivites/lupus erythemateux systemique (LES) et le syndrome de Goodpasture) et « non immune » (cardiopathie, toxique, infection…). Un bilan etiologique avec la recherche de signes cliniques extrathoraciques, d’auto-anticorps (anticorps anti-nucleaires et anti-membrane basale glomerulaire [ANCA]), la realisation d’une echographie cardiaque et la recherche d’une imputabilite iatrogenique ou d’un contexte septique evident est urgent. Le LES et le syndrome des antiphopholipides sont les causes immunes les plus frequentes en rhumatologie avec les vascularites a ANCA. Les autres connectivites sont rarement directement impliquees dans le mecanisme d’HIA, hormis en cas de formes associees a une vascularite et la presence d’ANCA. Une telle situation doit donc faire rechercher une cause « non immune » en priorite.
Published: 2018
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28. Lung cancer and interstitial lung disease: a literature review

Author: Martine Antoine, Jacques Cadranel, Isabelle Monnet, Christos Chouaid, Quentin Gibiot, Marie Wislez, and Jean-Marc Naccache
Subjects: Pulmonary and Respiratory Medicine, medicine.medical_specialty, Exacerbation, medicine.medical_treatment, Review Article, behavioral disciplines and activities, Gastroenterology, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, Pulmonary fibrosis, medicine, Lung cancer, Pneumonitis, business.industry, Incidence (epidemiology), Interstitial lung disease, Cancer, respiratory system, medicine.disease, respiratory tract diseases, body regions, Radiation therapy, 030228 respiratory system, 030220 oncology & carcinogenesis, business
Abstract: The association between lung cancer (LC) and interstitial lung disease (ILD) can be explained by the shared risk factors like smoking and physiopathology of fibrogenesis and cancerogenesis. The relative LC risk is shown to be 3.5- to 7.3-times higher in ILD, with LC occurrence estimated at 10–20% in ILD, with >15% of ILD patients likely to die from LC. ILD incidence upon LC diagnosis varied from 2.4–10.9%. Primary radiological presentations consist of peripheral lesions, mostly in the inferior pulmonary lobes, either close to or within the ILD areas. There is a trend towards inverted proportion of adenocarcinomas and squamous-cell carcinomas, with EGFR mutations very rarely found. ILD negatively impacted LC prognosis, with surgery associated with increased morbidity-mortality, particularly due to acute exacerbation (AE) of ILD. Limited resection reduced this risk, whilst increasing that of cancer mortality. Studies on radiotherapy that can induce AE-ILD are scarce. Chemotherapy was associated with similar response rates to those in LC patients without ILD, yet worse survival. This difference may be accounted for by ILD patients’ poorer health and higher risk of drug-induced pneumonitis. Further studies are warranted to better understand cancer physiopathology within the fibrotic areas, along with the therapeutic strategies required.
Published: 2018
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29. Cas clinique n° 3 : Une hémorragie intra-alvéolaire atypique

Author: A. Canellas, Antoine Parrot, Jean-Marc Naccache, and Jacques Cadranel
Subjects: Pulmonary and Respiratory Medicine
Published: 2018
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30. Sarco-IO : Étude des facteurs de risque associés à la survenue d’une infection opportuniste chez les patients porteurs d’une sarcoïdose

Author: Pascal Sève, Jean-Marc Naccache, N. Costedoat-Chalumeau, Vincent Cottin, Arnaud Hot, C. Bernard, Yves Pacheco, Hilario Nunes, Aurélien Guffroy, Dominique Valeyre, Florence Jeny, A.S. Korganow, T. El Jammal, Mathieu Gerfaud-Valentin, Alain Calender, and Y. Jamilloux
Subjects: Gastroenterology, Internal Medicine
Abstract: Introduction La sarcoidose est une granulomatose systemique d’etiologie indeterminee [1] . De nombreux cas et des series d’infections opportunistes (IO) chez des patients atteints de sarcoidose sont rapportes dans la litterature medicale [2] , [3] . Sont generalement exclues les aspergilloses cavitaires et les tuberculoses, associees respectivement a la fibrose et a des elements demographiques communs. Les agents opportunistes rapportes sont varies (Cryptococcus neoformans, JC virus, mycobacteries atypiques, Nocardia spp.) mais actuellement aucun facteur de risque d’infection opportuniste n’est decrit au cours de la sarcoidose. Patients et methodes Nous avons effectue un recueil de la litterature medicale via le moteur de recherche Medline et realise un appel a observations via le groupe sarcoidose francophone a la recherche d’associations « infection opportuniste » et « sarcoidose ». Les caracteristiques cliniques des IO ont ete comparees a une population temoin constituee de patients porteurs d’une sarcoidose sans IO issus d’une cohorte de notre service. Resultats La revue de la litterature a permis de recenser 164 cas d’infections qualifiees d’opportunistes chez des patients porteurs d’une sarcoidose. Parmi ces 164 infections, 83 etaient des cryptococcoses (51 %) et 39 etaient des leuco-encephalopathies multifocales progressives (LEMP) (24 %). Notre appel a observations a permis de recenser 50 IO chez 49 patients, incluant : 24 cryptococcoses (49 %), 12 LEMP (24 %), 7 pneumocystoses (14 %) et 7 autres infections (14 %) dont 3 mycobacterioses atypiques, 2 nocardioses et 2 infections a cytomegalovirus. Ces 49 patients avec IO ont ete compares a 135 controles porteurs d’une sarcoidose sans IO. Dans le groupe infection opportuniste, le compte lymphocytaire moyen etait de 1000/mm3 (100-2680/mm3) et 14 patients ne presentaient pas de lymphopenie (29 %). Les facteurs de risques identifies en analyse univariee etaient : le sexe masculin (OR = 4,76; IC95 % [2,12 ; 10,00]), la presence d’adenopathies peripheriques (OR = 3,45 ; [1,49 ; 8,04]), d’une hepatomegalie (OR = 8,10 ; [1,91 ; 40,41]) ou d’une splenomegalie (OR = 9,00, [1,80 ; 58,86]), la presence d’une atteinte pulmonaire de stade ≥2 (OR = 6,66 ; [2,80 ; 17,74]), d’une atteinte neurologique centrale (OR = 8,73 ; [1,98 ; 53,52]) ou d’une insuffisance renale (OR = 5,65 ; [1,33 ; 25,24]), la corticotherapie (OR = 10,26 ; [4,15 ; 29,22]) et l’utilisation du cyclophosphamide (OR = 9,42 ; [1,61 ; 98,92]) ou d’un anti-TNFα (OR = 7,68 ; [1,21 ; 83,62]). En analyse multivariee (ajustement sur l’âge et le sexe), les facteurs de risque identifies etaient l’utilisation de corticoides (OR = 10,60 ; [4,31 ; 29,57]) ou d’un anti-TNFα (OR = 13,47 ; [2,05 ; 129,43]), la presence d’une hepatomegalie (OR = 9,91 ; [2,23 ; 55,85]), une atteinte pulmonaire de stade 2 ou superieur (OR = 5,65 ; [2,34 ; 15,36]) et l’insuffisance renale (OR = 11,76 ; [2,32 ; 68,35]). La lymphopenie Conclusion Les facteurs de risque d’IO dans notre etude sont associes a la gravite de la sarcoidose et/ou a sa diffusion systemique. Il existe un facteur confondant lie a l’utilisation des immunosuppresseurs chez des patients avec une sarcoidose plus severe. Dans notre serie, 6 % des patients ont developpe une infection opportuniste sans aucun facteur de risque, suggerant neanmoins une immunosuppression propre a la sarcoidose dans ces cas.
Published: 2021
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31. Toxicité pulmonaire des médicaments : ce que le réanimateur doit connaître ?

Author: Muriel Fartoukh, Antoine Parrot, Jacques Cadranel, Aude Gibelin, Guillaume Voiriot, Michel Djibré, Jean-Marc Naccache, and T. Issoufaly
Subjects: Emergency Medicine, Emergency Nursing
Abstract: Le diagnostic d’insuffisance respiratoire aigue d’origine medicamenteuse est un veritable defi. Les traitements a l’origine d’une toxicite pulmonaire sont tres nombreux. Les nouveaux traitements, notamment en oncohematologie (therapie ciblee, immunotherapie), ne sont pas en reste. L’expression clinique tres heterogene est sous-tendue par des mecanismes lesionnels tres differents. Apres avoir ecarte une infection ou un œdeme pulmonaire, il faut savoir evoquer une pneumopathie medicamenteuse. La realisation d’un scanner thoracique et d’une fibroscopie bronchique avec lavage bronchoalveolaire peut etre utile pour exclure une pathologie infectieuse et suggerer une pathologie medicamenteuse. Le traitement est l’arret du medicament et repose frequemment dans ces formes graves sur une corticotherapie.
Published: 2018
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32. Cas clinique n° 5 : Trouble ventilatoire obstructif au cours d’une sarcoïdose

Author: T. Issoufaly, L. Mourtada, Jean-Marc Naccache, A. Souidi, Antoine Parrot, and Jacques Cadranel
Subjects: Pulmonary and Respiratory Medicine, 03 medical and health sciences, 0302 clinical medicine, 030228 respiratory system, business.industry, Medicine, 030212 general & internal medicine, business
Published: 2017
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33. Liver involvement in patients with telomere-related genes mutations: prevalence, clinical, radiological, pathological features, outcome and risk factors

Author: Sabrina Sidali, Raphaël Borie, Sicre Flore, Elodie Lainey, Pierre-Emmanuel Rautou, Jacques Cadranel, Jean-marc Naccache, Vincent Cottin, Jérôme Dumortier, Emmanuel Jacquemin, Nousbaum Jean Baptiste, Sandrine Hirschi, Arnaud Bourdin, Magdalena Meszaros, Sebastien Dharancy, Sophie Hilaire, Vincent Mallet, Reynaud-Gaubert Martine, Louis Terriou, Frédéric Gottrand, Wadih Abou Chahla, Jean Emmanuel Khan, Paul Carrier, Faouzi Saliba, Laura Rubbia-Brandt, Laure Elkrief, Victor de Lédinghen, Armand Abergel, Tournilhac Olivier, Yasmina Chouik, Antoine Coupier, Thierry Leblanc, Isabelle Ollivier-Hourmand, Eric Nguyen Khac, Hélène Morisse-Pradier, Kinan El Husseini, Odile Goria, Ba Ibrahima, Dominique Roulot, Christophe Bureau, Hilario Nunes, Dominique Valla, Valérie Paradis, Kannengiesser Caroline, and Aurélie Plessier
Subjects: Hepatology
Published: 2020
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34. Clinical outcomes after lung transplantation for fibrosis in telomerase related genes mutation carriers

Author: Stéphane Jouneau, Sylvain Marchand Adam, Hilario Nunes, Jean-Marc Naccache, Jérôme Le Pavec, Martine Reynaud-Gaubert, Antoine Froidure, Aurélie Le Borgne, Christophe Pison, Bruno Crestani, Lidwine Wemeau-Stervinou, Raphael Borie, François Philit, Antoine Roux, Sandrine Hirschi, Hervé Mal, Vincent Cottin, Romain Lazor, Caroline Kannengiesser, Mathilde Phillips Houlbracq, 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), 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), Service de pneumologie (Strasbourg), CHU Strasbourg-Nouvel Hôpital Civil, Service de pneumologie. Centre de compétence des maladies pulmonaires rares, Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Service de chirurgie thoracique et transplantation pulmonaire, Centre chirurgical Marie Lannelongue, 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 (UGA), Cliniques Universitaires Saint-Luc [Bruxelles], Centre Hospitalier Lyon Sud [CHU - HCL] (CHLS), 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), Contaminants Chimiques, immunité et Inflammation, 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 )-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 ), Hypoxie et Poumon : pneumopathologies fibrosantes, modulations ventilatoires et circulatoires (H&P), Université Paris 13 (UP13)-UFR SMBH, Hôpital Nord [CHU - APHM], Microbes évolution phylogénie et infections (MEPHI), Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS), Physiopathologie et Epidemiologie de l'Insuffisance Respiratoire, Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre de recherche sur l'Inflammation (CRI (UMR_S_1149 / ERL_8252 / U1149)), Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Université Paris Diderot, Sorbonne Paris Cité, Paris, France, Université Paris Diderot - Paris 7 (UPD7), Centre Chirurgical Marie Lannelongue (CCML), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Grenoble Alpes [2016-2019] (UGA [2016-2019]), 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 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 Paris Diderot - Paris 7 (UPD7)-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), 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)-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), and Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Université d'Angers (UA)
Subjects: medicine.medical_specialty, Cirrhosis, medicine.medical_treatment, Idiopathic pulmonary fibrosis, Neutropenia, Gastroenterology, Liver disease, [SDV.MHEP.CSC]Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, [SDV.MHEP.MI]Life Sciences [q-bio]/Human health and pathology/Infectious diseases, Median follow-up, Internal medicine, Pulmonary fibrosis, medicine, Transplantation pulmonaire, Lung transplantation, [SDV.MP.PAR]Life Sciences [q-bio]/Microbiology and Parasitology/Parasitology, [SDV.MHEP.ME]Life Sciences [q-bio]/Human health and pathology/Emerging diseases, Lung, business.industry, Fibrose pulmonaire, medicine.disease, [SDV.MP.BAC]Life Sciences [q-bio]/Microbiology and Parasitology/Bacteriology, 3. Good health, medicine.anatomical_structure, [SDV.MP.VIR]Life Sciences [q-bio]/Microbiology and Parasitology/Virology, business
Abstract: International audience; Carriers of telomerase related genes (TRG) mutation seem to present a worst prognosis with more common hematological complications after lung transplantation (LT) for pulmonary fibrosis. The aim of this study was to describe the outcomes after LT and identify pre-LT prognosis factors in a multicenter cohort of lung transplant recipients with TRG mutation.We retrospectively reviewed all identified patients with pathogenic TRG mutation (n=38; TERT, n=22, TERC, n=10, RTEL1, n=6) who received LT in France, Switzerland and Belgium between 2009 and 2018. The median age at LT was 54 years (46-59), 70% were male, and 60% had idiopathic pulmonary fibrosis (IPF). At diagnosis of pulmonary fibrosis, 84% had a hematological disease, including 8 with myelodysplasia, and 45% had a liver disease. After a median follow up of 2.2 years (1,2-3,7), 16 received a single LT, 22 a double LT and 2 a combined liver-LT.The overall post-LT median survival was 3.75 years (1.8-NA). Patients with myelodysplasia before LT had an increased risk of death after LT (HR= 4.12 (1.47-11.53) p=0.007). After LT, all patients showed anemia, 70% thrombocytopenia, and 60% neutropenia. Four patients showed severe liver disease: portal hypertension, cirrhosis. Sixteen patients (42%) experienced acute renal failure and 18 (47%) developed chronic renal insufficiency during follow up. Four developed chronic lung allograft dysfunction.Overall survival after LT supports LT in TRG mutation carriers. Careful evaluation at diagnosis, might limit LT indication for patients with established myelodysplasia.
Published: 2019
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35. Outcome and prognostic factors in a French cohort of patients with myositis-associated interstitial lung disease

Author: Julie Obert, Jean-Marc Naccache, Hilario Nunes, Olivia Freynet, Dominique Valeyre, Robin Dhote, Makoto Miyara, and Pierre-Yves Brillet
Subjects: Adult, Male, medicine.medical_specialty, Adolescent, medicine.medical_treatment, Immunology, Polymyositis, Young Adult, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, DLCO, Internal medicine, medicine, Humans, Immunology and Allergy, Lung transplantation, Lung, Aged, Retrospective Studies, 030203 arthritis & rheumatology, Myositis, business.industry, Mortality rate, Interstitial lung disease, Retrospective cohort study, Middle Aged, respiratory system, Dermatomyositis, Prognosis, medicine.disease, Respiratory Function Tests, respiratory tract diseases, Surgery, Pulmonology, 030228 respiratory system, Female, Lung Diseases, Interstitial, Tomography, X-Ray Computed, business
Abstract: Interstitial lung disease (ILD) is a common form of extramuscular involvement in patients with polymyositis/dermatomyositis and is associated with poor prognosis. This study was designed to describe the long-term outcome of myositis-associated ILD. This retrospective observational study was conducted in 48 consecutive patients. Two groups defined according to outcome were compared to determine prognostic factors: a “severe” group (vital capacity [VC]
Published: 2016
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36. Anomalie respiratorie nel soggetto affetto da virus dell’immunodeficienza umana

Author: A Parrot, Jacques Cadranel, H Ferrand, P Héloret, P Créquit, and Jean-Marc Naccache
Subjects: 03 medical and health sciences, 0302 clinical medicine, 030228 respiratory system, Philosophy, 030212 general & internal medicine, Humanities
Abstract: Le anomalie respiratorie osservate nelle persone affette da virus dell’immunodeficienza umana (HIV) sono varie e possono trovarsi in rapporto o meno con questo contesto particolare. Si sono evolute nel tempo grazie a una migliore conoscenza dell’infezione da HIV, all’introduzione di trattamenti antiretrovirali e alla prescrizione di trattamenti profilattici che hanno permesso di rendere questa infezione, mortale all’inizio degli anni ’80, una malattia cronica. La diagnosi di ogni danno polmonare dipende, in particolare, dalla conta di linfociti CD4, dall’assunzione di una terapia antiretrovirale e da un’eventuale profilassi. Inoltre, l’anamnesi, l’esame obiettivo e l’aspetto radiologico aiutano l’orientamento diagnostico e la gestione. Un stessa patologia puo assumere aspetti diversi in funzione dello stato immunitario del paziente. La polmonite batterica e la principale causa di malattia polmonare nei pazienti affetti da HIV. La tubercolosi e la pneumocistosi sono le due principali infezioni opportunistiche polmonari. Dopo gli antiretrovirali e apparsa una nuova patologia: la sindrome infiammatoria da immunoricostituzione. L’incidenza dei linfomi e dei cancri del polmone aumenta a causa dell’allungamento dell’aspettativa di vita. La broncopneumopatia cronica ostruttiva e una causa importante di morbilita e mortalita in questa popolazione, in cui il tasso di fumatori e elevato. In questo articolo si propone un aggiornamento sul processo diagnostico e sulle principali eziologie di fronte a un’anomalia respiratoria in un paziente affetto da HIV.
Published: 2016
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37. Endobronchial ultrasound-guided transbronchial needle aspiration is feasible, safe, and reaches a 90 % diagnostic yield in patients with hypoxemic acute respiratory failure

Author: Jean-Marc Naccache, Michel Febvre, Antoine Parrot, Jocelyne Fleury Feith, Maxens Decavèle, Valérie Gounant, Muriel Fartoukh, Université Pierre et Marie Curie - Paris 6 (UPMC), CHU Tenon [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Université Paris Diderot - Paris 7 (UPD7), Service d’oncologie thoracique et essais précoces [CHU Bichat], 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), Service de Réanimation et USC Médico-Chirurgicale [CHU Tenon], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de Pneumologie - Oncologie Thoracique - Maladies Pulmonaires Rares [CHU Tenon], Service d’Anatomie et cytologie pathologiques [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Tenon [AP-HP], and Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)
Subjects: medicine.medical_specialty, business.industry, Pain medicine, Yield (finance), Biopsy, Fine-Needle, Bronchi, 030208 emergency & critical care medicine, 030204 cardiovascular system & hematology, Critical Care and Intensive Care Medicine, [SDV.MHEP.PSR]Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract, 3. Good health, 03 medical and health sciences, 0302 clinical medicine, Anesthesia, Anesthesiology, Bronchoscopy, Humans, Medicine, Acute respiratory failure, In patient, Endobronchial ultrasound, Hypoxia, Respiratory Insufficiency, business, Ultrasonography
Abstract: International audience; Adverse events are common during conventional invasive lung sampling procedures [1] and may be particularly detrimental in critically ill patients [2]. We sought to describe the feasibility, safety, and diagnostic accuracy of endobronchial ultrasound transbronchial needle aspiration (EBUS-TBNA) in the intensive care unit (ICU).Between May 2011 and May 2015, nine consecutive patients with acute respiratory failure (ARF) and mediastinal or hilar involvement were selected from a prospective cohort [3] that comprehensively describes all EBUS-TBNA procedures in Tenon hospital, a tertiary university teaching hospital in Paris, France. EBUS-TBNA was performed using a 6.7-mm-outer-diameter, real-time, bronchoscope with a 7.5-MHz linear ultrasound transducer.
Published: 2016
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38. Place du cyclophosphamide (CYC) intraveineux dans le traitement des pneumopathies interstitielles diffuses (PID)

Author: A. Canellas, R. Hindré, Muriel Fartoukh, A. Milon, Jean-Marc Naccache, Antoine Parrot, P. Rigaud, and Jacques Cadranel
Subjects: Pulmonary and Respiratory Medicine
Abstract: Introduction Les PID conduisent a un declin fonctionnel respiratoire plus ou moins rapide. Deux essais therapeutiques ont montre une efficacite moderee du CYC sur le declin respiratoire de patients atteints de PID de sclerodermie systemique. Peu de donnees sont disponibles pour les PID d’autres etiologies, en dehors de cohortes retrospectives de faible effectif. L’objectif de l’etude est de decrire l’effet du CYC sur le pronostic et les fonctions respiratoires d’une cohorte de patients atteints de PID de causes variees. Methodes Cette etude retrospective monocentrique exhaustive a inclus 134 patients entre 01/2005 et 12/2017 ayant une PID idiopathique (PII) (n = 53), de connectivite (n = 49), une IPAF (n = 17) ou une PHS (n = 15). Les patients etaient traites de facon homogene par CYC: 600 mg/m2 IV a J1, J15, J28, M2. Les donnees epidemiologiques, cliniques, fonctionnelles et scannographiques etaient recueillies avant traitement, puis a M3, M7, M13, M25 et a la derniere evaluation disponible. Le critere de jugement principal etait le taux de patients morts ou transplantes a la date du 31/12/2018 en fonction du type de PID et des caracteristiques initiales. Resultats Les patients ont recu en moyenne 5 bolus de CYC avec une bonne tolerance. Apres un suivi moyen de 28 mois, 75 patients etaient decedes ou transplantes. La mediane de survie de la population globale etait de 33 mois et de 9, 15, 45, 65 mois pour les FPI, les PII hors FPI, les IPAF et les connectivites. Une PII, la presence de rayon de miel et d’une fievre initiale etaient des facteurs independants de mauvais pronostic. Le taux eleve de lymphocytes au LBA etait associe a un meilleur pronostic. Le CYC permettait une baisse des besoins en oxygene, un gain significatif de CVF et DLCO principalement pour les PID de connectivites comparees aux PII. Les condensations alveolaires, l’absence de bronchectasies et un pattern non evocateur de PIC au scanner etaient associes a un gain plus important de CVF. Conclusion Le CYC semble bien tolere et efficace dans certaines PID autres que la sclerodermie systemique avec des disparites selon l’etiologie, les donnees du scanner et du LBA.
Published: 2021
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39. 2010 French SPILF-AFSSAPS guiding criteria for Streptococcuspneumoniae acute community-acquired pneumonia: Evaluation in patients of the PACSCAN-ESCAPED cohort

Author: Yann-Erick Claessens, M. Ben Hayoun, Xavier Duval, Catherine Leport, Jean-Marc Naccache, H. Le Floch, Sarah Tubiana, and Emmanuelle Varon
Subjects: Adult, Male, medicine.medical_specialty, Chest pain, Polymerase Chain Reaction, Cohort Studies, 03 medical and health sciences, Community-acquired pneumonia, Internal medicine, Nasopharynx, medicine, Humans, Blood culture, Medical history, Prospective Studies, Aged, Aged, 80 and over, 0303 health sciences, medicine.diagnostic_test, 030306 microbiology, business.industry, Sputum, Middle Aged, Pneumonia, Pneumococcal, medicine.disease, Community-Acquired Infections, Radiography, Pneumonia, Infectious Diseases, Streptococcus pneumoniae, Cohort, Practice Guidelines as Topic, Etiology, Female, medicine.symptom, business, Emergency Service, Hospital, Tomography, X-Ray Computed
Abstract: Objective To assess the proportion of patients meeting the 2010 SPILF-AFSSAPS guiding criteria for Streptococcus pneumoniae in patients consulting at the emergency departments of four French university hospitals for acute community-acquired pneumonia (CAP) suspicion. Patients and methods The PACSCAN study prospectively included 319 patients. Medical history, clinical, biological, and radiological presentations were collected. An adjudication committee retrospectively classified the diagnostic certainty based on the initial chest CT scan data and the follow-up data up to Day 28. S. pneumoniae was looked for according to the clinician's choice of blood culture, pneumococcal urinary antigen test, nasopharyngeal PCR, and/or sputum microbiological examination. Results All patients (100%) met at least one criterion for S. pneumoniae CAP and six (2%) met all criteria. The distribution of criteria ranged from 32% (chest pain criterion) to 86% (age ≥ 40 years criterion). These figures were respectively 100%, 3%, 38%, and 82% when the study population was restricted to the 139 patients with definite or probable CAP, according to the adjudication committee. Taking into account the microbiological results, the criteria taken one by one or combined did not make it possible to differentiate the 19 S. pneumoniae CAP from the other CAPs. Conclusion The 2010 SPILF-AFSSAPS guiding criteria for S. pneumoniae CAP are found in very variable proportions and do not, in their current form, make it possible to accurately guide towards a pneumococcal etiology in patients included in the PACSCAN study.
Published: 2019

40. Tolerance and efficacy of antifibrotic treatments in IPF patients carriying telomere related gene mutations

Author: Martine Reynaud-Gaubert, Antoine Froidure, Anne Gondouin, Stéphane Jouneau, Claire Andrejak, Benjamin Bondue, Anne-Sophie Gamez, Effrosyni D. Manali, Jean-Marc Naccache, Aurélien Justet, Bruno Crestani, Manuela Funke-Chambour, Maria Molina Molina, Sandrine Hirschi, Caroline Kanengiesser, Dymph Klay, Hilario Nunes, Gregoire Prevost, Raphael Borie, Coline H.M. van Moorsel, Cécile Tromeur, Liwine Wemeau, Philippe Bonniaud, Vincent Cottin, Sylvain Marchand-Adam, Physiopathologie et Epidémiologie des Maladies Respiratoires (PHERE (UMR_S_1152 / U1152)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité), Service de pneumologie [CHU Caen], Université de Caen Normandie (UNICAEN), Normandie Université (NU)-Normandie Université (NU)-CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Tumorothèque de Caen Basse-Normandie (TCBN), Imagerie et Stratégies Thérapeutiques des pathologies Cérébrales et Tumorales (ISTCT), Normandie Université (NU)-Normandie Université (NU)-Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Centre National de la Recherche Scientifique (CNRS), St. Antonius Hospital [Nieuwegein], Infections Virales et Pathologie Comparée - UMR 754 (IVPC), 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, Institut d'Investigació Biomèdica de Bellvitge [Barcelone] (IDIBELL), Hôpital Avicenne [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Microbes évolution phylogénie et infections (MEPHI), Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS), Hôpital Nord [CHU - APHM], Sorbonne Université (SU), CHU Tenon [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Groupe de recherche clinique Biomarqueurs Théranostiques des Cancers Bronchiques Non à Petites Cellules (GRC 4 - Theranoscan), University of Athens Medical School [Athens], Cliniques Universitaires Saint-Luc [Bruxelles], École des Hautes Études en Santé Publique [EHESP] (EHESP), 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 ), CHU Amiens-Picardie, Agents infectieux, résistance et chimiothérapie - UR UPJV 4294 (AGIR ), Université de Picardie Jules Verne (UPJV)-CHU Amiens-Picardie, Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), Les Hôpitaux Universitaires de Strasbourg (HUS), Hôpital Erasme [Bruxelles] (ULB), Faculté de Médecine [Bruxelles] (ULB), Université libre de Bruxelles (ULB)-Université libre de Bruxelles (ULB), CHU Dijon, Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Institut National de la Santé et de la Recherche Médicale (INSERM), CIC Brest, Université de Brest (UBO)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Hôpital de la Cavale Blanche, Institut Henri Beaufour (IPSEN), IPSEN-BEAUFOUR, Hôpital Bretonneau, Bern University Hospital [Berne] (Inselspital), and Hôpital Lapeyronie [Montpellier] (CHU)
Subjects: medicine.medical_specialty, business.industry, [SDV]Life Sciences [q-bio], Pirfenidone, medicine.disease, Gastroenterology, Telomere, 03 medical and health sciences, Idiopathic pulmonary fibrosis, FEV1/FVC ratio, chemistry.chemical_compound, 0302 clinical medicine, 030228 respiratory system, chemistry, DLCO, Internal medicine, Pulmonary fibrosis, medicine, Nintedanib, 030212 general & internal medicine, Related gene, business, medicine.drug
Abstract: Telomere related gene (TRG) mutations are found in approximately 20% of patients with familial pulmonary fibrosis and are associated with hepatic, cutaneous and hematologic manifestations. Pirfenidone and nintedanib have been shown to slow the decline of FVC of patients with idiopathic pulmonary fibrosis (IPF). There is limited evidence of efficacy and safety of these treatments in IPF patients carrying a TRG mutation. The objective of this retrospective multicenter study was to analyze the efficacy and safety of antifibrotic drugs in IPF patients carrying a TRG mutation. We identified 103 IPF patients carrying a mutation in TERT (n=74), TERC (n=17), RTEL1 (n=10) or PARN (n=3). from 6 countries (France, Netherlands, Belgium, Spain, Greece and Switzerland) Forty-four patients received nintedanib, 59 received pirfenidone. The mean age at diagnosis was 57.9 ± 13.6 years and the median delay between diagnosis and treatment initiation was 6.0 months [3.0-15.1]. At initiation of treatment, the FVC was 80.8% ± 20.2% and the DLCO was 44.0% ± 13.7%. The median duration of treatment was 10.8 months [6.5-18.1]. Antifibrotic treatment was terminated in 18 patients because of progression of the disease and in 15 patients due to intolerable side effects, mainly digestive (nintedanib, n = 9, pirfenidone, n = 6). The median decline in FVC prior to initiation of treatment was 24.5 mL [10.5-30.1] per month. After initiation of treatment, the median decline in FVC was 18.0 mL [9.0-27.0] per month in patients treated with nintedanib and 25.4 mL per month [14.7-36.7] in patients treated with pirfenidone. This retrospective series shows that antifibrotic therapies are well tolerated in IPF patients with TRG mutations. Further analyses are needed to evaluate the efficacy.
Published: 2019
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41. Caractéristiques et pronostic des malades avec prélèvements respiratoires positifs pour mycobactéries non tuberculeuses (MNT)–étude de cohorte rétrospective de 171 cas consécutifs

Author: Antoine Parrot, N. Veziris, Charlotte Verdet, L. Deneuville, Jean-Marc Naccache, Pierre Levy, Gilles Pialoux, and Jacques Cadranel
Subjects: Pulmonary and Respiratory Medicine
Abstract: Introduction L’isolement de MNT dans les secretions respiratoires n’est pas toujours associe a une infection. Le diagnostic d’infection a MNT selon les criteres 2007 de l’American Thoracic Society (ATS) ne conduit pas necessairement a la mise en place d’un traitement dont l’impact pronostique est incertain. L’objectif de l’etude est de decrire et comparer les caracteristiques et la survie d’une cohorte de patients ayant au moins un prelevement respiratoire positif a MNT (MNT+). Methodes Cette etude retrospective a inclus tous les patients MNT+ identifies dans un Hopital Universitaire Parisien entre 01/2012 et 06/2016. Les patients ayant un antecedent de VIH et de mucoviscidose ont ete exclus. La mediane de suivi etait de 31 mois. Les patients ont ete classes en 3 categories : infectes (IF), indetermines (ID) ou colonises (CO) en tenant compte des criteres ATS. Resultats Cent soixante et onze patients MNT+ ont ete inclus et repartis en : 75 IF (44 %), 41 ID (24 %) et 55 CO (32 %). Il n’y avait pas de difference selon l’âge, le sex-ratio, le tabagisme, les comorbidites, la presence d’une coinfection bacterienne ou aspergillaire et la frequence des symptomes respiratoires et generaux entre les 3 groupes. L’IMC etait inferieur chez les IF (IF : 20, ID : 24, C0 : 23, p = 0,0002). La positivite de l’examen direct (IF : 51 %, ID : 7 %, CO : 0 %, p Conclusion Dans notre etude, 44 % des patients avec MNT+ sont classes comme des infections et 24 % restent avec une incertitude diagnostique du fait d’un TDM non compatible ou d’un autre diagnostic associe. Les infections se caracterisent par un IMC plus bas et avec plus souvent un aspect TDM cavitaire et des examens directs positifs. Ces caracteristiques semblent egalement associees a la mise en route d’un traitement, avec dans 1/3 des cas un arret pour toxicite.
Published: 2020
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42. Pneumopathies interstitielles diffuses associées aux mutations de Poly(A)-specific ribonuclease (PARN) : une étude de cohorte rétrospective multicentrique

Author: Martine Reynaud-Gaubert, Hilario Nunes, Raphael Borie, Bruno Crestani, Q. Philippot, D. Israel Biet, Frédéric Schlemmer, Effrosyni D. Manali, Hervé Mal, Vincent Cottin, Benjamin Bondue, Spyros Papiris, Anne Gondouin, Caroline Kannengiesser, Jean-Marc Naccache, and Lidwine Wemeau
Subjects: Pulmonary and Respiratory Medicine
Abstract: Introduction Des mutations de Poly(A)-specific ribonuclease (PARN) ont ete associees a des cas familiaux de fibrose pulmonaire. Le phenotype des patients presentant une pneumopathie interstitielle diffuse (PID) et une mutation de PARN est cependant mal decrit. Methodes Nous avons realise une etude retrospective, observationnelle, non interventionnelle. Tous les patients avec une PID et une mutation de PARN, suivis dans les centres du reseau OrphaLung, ont ete inclus. Resultats Vingt patients, issus de 12 familles, ont ete inclus. L’âge median lors du diagnostic de PID etait de 58,8 ans (ecart interquartile [IQR] de 52,8 a 64,5 ans). Les diagnostics les plus frequents etaient : fibrose pulmonaire idiopathique (FPI ; n = 8 ; 40 %) et fibrose inclassable (n = 5 ; 12,5 %). L’hemogramme, disponible pour 17 patients, et le bilan hepatique, disponible pour 16 patients, etaient normaux. Treize patients ont recu un traitement specifique pour leur PID comme suit : corticoides (n = 8), anti-fibrosant (n = 6), immunosuppresseur (n = 2) et N-acetyl-cysteine (n = 1). Sept patients n’ont pas recu de traitement specifique. Aucun patient n’a presente une amelioration respiratoire (objectivee par des epreuves fonctionnelles respiratoires ou une diminution des besoins en oxygene). Le declin median de capacite vitale forcee, pour l’ensemble de la population, etait de 219 mL par an (IQR de −73 a −511). Apres un suivi median de 2,9 ans (IQR de 0,93 a 7,1), sept patients sont decedes et cinq patients ont ete transplantes. Conclusion Cette etude objective que les mutations de PARN sont le plus souvent associees avec une FPI, d’autres phenotypes peuvent neanmoins etre observes. Une telle heterogeneite phenotypique implique une evaluation complete des PID associees aux mutations de PARN afin de definir une strategie therapeutique adaptee.
Published: 2020
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43. Données démographiques et caractéristiques au moment du diagnostic des 847 patients atteints de fibrose pulmonaire idiopathique inclus dans la cohorte Radico-PID

Author: Sandrine Hirschi, I. Dufaure-Garé, Lidwine Wemeau, Stéphane Jouneau, Jean-Marc Naccache, M. Chevereau, Bruno Crestani, Martine Reynaud-Gaubert, David Montani, Vincent Cottin, J.-C. Dalphin, Sylvain Marchand-Adam, Sébastien Quétant, Philippe Bonniaud, A. Clément, Dominique Israel-Biet, and Hilario Nunes
Subjects: Pulmonary and Respiratory Medicine
Abstract: Introduction La fibrose pulmonaire idiopathique (FPI) est une affection rare et peu de donnees epidemiologiques sont disponibles en France. Ce projet vise a decrire les caracteristiques en vie reelle des patients atteints de FPI recevant un traitement anti-fibrosant, ainsi que leurs comorbidites et la mortalite. Methodes RaDiCo-PID est une cohorte longitudinale a long terme incluant des patients enfants et adultes atteints de pneumopathies interstitielles diffuses (PID) idiopathiques suivis dans des centres de reference et de competence de la filiere Respifil et des reseaux OrphaLung et Respirare. RaDiCo-PID comporte un sous-groupe de patients atteints de FPI. Nous presentons ici les caracteristiques de base des patients atteints de FPI apres 2 ans d’inclusion. Resultats Entre le 15 juin 2017 et le 4 septembre 2019, 1246 patients atteints de pneumopathie interstitielle diffuse ont ete inclus dans le registre Radico-PID dans 18 centres, dont 847 atteints de FPI (68 %). Les patients atteints de FPI etaient en majorite des hommes (82,7 %), avec un âge moyen de 72,5 ± 9 ans a l’inclusion et un index de masse corporelle moyen de 26,8 ± 4,3. Parmi les patients avec FPI, 44,6 % etaient des cas incidents avec une duree mediane entre le diagnostic et l’inclusion de 8,9 mois (Q1 = 0,9 et Q3 = 26,4) ; 25,3 % de ces patients avaient eu une biopsie. La capacite vitale forcee moyenne au diagnostic etait de 73,4 ± 25,0 % de la valeur theorique (n = 561), et la capacite de transfert du monoxyde de carbone moyenne au diagnostic etait de 39,6 ± 18,2 % (n = 498). Selon les donnees disponibles sur les traitements anti-fibrosants (n = 661), 347 patients ont ete traites, au moins une fois, par du nintedanib et 312 patients par de la pirfenidone ; 113 patients ont recu sequentiellement ces 2 molecules. Conclusion La cohorte Radico-PID fournit des donnees concretes precieuses sur les caracteristiques demographiques des patients, notamment au moment du diagnostic de FPI. Les effectifs actuels permettent d’envisager des analyses plus approfondies.
Published: 2020
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44. Rituximab for auto-immune alveolar proteinosis, a real life cohort study

Author: Martine Reynaud-Gaubert, Jean-Marc Naccache, Leonidas Chavez, Lidwine Wemeau-Stervinou, Sylvain Marchand-Adam, Laurent Savale, Abdellatif Tazi, Marie-Pierre Debray, Bruno Crestani, Jacques Cadranel, Hilario Nunes, Sylvie Leroy, Raphael Borie, Berenice Soyez, Vincent Cottin, Cedric Menard, Emmanuel Gomez, Service de Pneumologie A [Paris], 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), 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), LabEx Inflamex, Université Sorbonne Paris Cité (USPC), CHU Pontchaillou [Rennes], 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 Universitaire [Grenoble] (CHU), Department of Pneumology [Lyon], Hospices Civils de Lyon (HCL), Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Pathologies Respiratoires : Protéolyse et Aérosolthérapie, Université de Tours-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre Hospitalier Régional Universitaire de Tours (CHRU TOURS), COMUE Université Côte d'Azur (2015-2019) (COMUE UCA), Hôpital Pasteur [Nice] (CHU), Université Pierre et Marie Curie - Paris 6 (UPMC), Hypoxie et Poumon : pneumopathologies fibrosantes, modulations ventilatoires et circulatoires (H&P), Université Paris 13 (UP13)-UFR SMBH, Microbes évolution phylogénie et infections (MEPHI), Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS), Université Paris-Sud - Paris 11 - Faculté de médecine (UP11 UFR Médecine), Université Paris-Sud - Paris 11 (UP11), Centre de référence des Histiocytoses, Service de pneumologie. Centre de compétence des maladies pulmonaires rares, Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Département de Radiologie [Bichat] (DR- Bichat), Fondation du Souffle (FR), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Université de Tours (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), 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), COMBE, Isabelle, 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), and Centre National de la Recherche Scientifique (CNRS)-Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)
Subjects: Adult, Male, medicine.medical_specialty, Alveolar proteinosis, medicine.medical_treatment, Interstitial lung disease, Pulmonary Alveolar Proteinosis, Bronchoalveolar Lavage, Gastroenterology, [SDV.MHEP.PSR]Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract, Cohort Studies, 03 medical and health sciences, 0302 clinical medicine, Refractory, Internal medicine, Surfactant, medicine, Humans, Immunologic Factors, Lung transplantation, Whole lung lavage, 030212 general & internal medicine, Adverse effect, Autoantibodies, Retrospective Studies, lcsh:RC705-779, business.industry, Research, GM-CSF, lcsh:Diseases of the respiratory system, [SDV.IMM.IMM]Life Sciences [q-bio]/Immunology/Immunotherapy, Middle Aged, medicine.disease, 3. Good health, 030228 respiratory system, [SDV.MHEP.PSR] Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract, Female, Rituximab, France, Therapy, [SDV.IMM.IMM] Life Sciences [q-bio]/Immunology/Immunotherapy, business, Pulmonary alveolar proteinosis, Follow-Up Studies, medicine.drug, Cohort study
Abstract: International audience; Background: Whole lung lavage is the current standard therapy for pulmonary alveolar proteinosis (PAP) that is characterized by the alveolar accumulation of surfactant. Rituximab showed promising results in auto-immune PAP (aPAP) related to anti-GM-CSF antibody.Methods: We aimed to assess efficacy of rituximab in aPAP in real life and all patients with aPAP in France that received rituximab were retrospectively analyzed.Results: Thirteen patients were included. No patients showed improvement 6 months after treatment, but, 4 patients (30%) presented a significant decrease of alveolar-arterial difference in oxygen after 1 year. One patient received lung transplantation and one patient was lost of follow-up within one year. Although a spontaneous improvement cannot be excluded in these 4 patients, improvement was more frequent in patients naive to prior specific therapy and with higher level of anti-GM-CSF antibodies evaluated by ELISA. No serious adverse event was evidenced. Conclusions: These data do not support rituximab as a second line therapy for patients with refractory aPAP.
Published: 2018
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45. Early Chest Computed Tomography Scan to Assist Diagnosis and Guide Treatment Decision for Suspected Community-acquired Pneumonia

Author: Marie-France Carette, Blandine Rammaert, Patrick Ray, Pierre Hausfater, Florence Tubach, Christophe Choquet, Catherine Leport, Marie-Pierre Debray, Anne-Laure Brun, Xavier Duval, Yann-Erick Claessens, Jean-Marc Naccache, and Charles Mayaud
Subjects: Adult, Male, Pulmonary and Respiratory Medicine, medicine.medical_specialty, Clinical Decision-Making, Computed tomography, Critical Care and Intensive Care Medicine, Cohort Studies, Community-acquired pneumonia, Multidetector Computed Tomography, Ambulatory Care, medicine, Humans, In patient, Prospective Studies, Lung, Aged, Aged, 80 and over, medicine.diagnostic_test, business.industry, Disease Management, Pneumonia, Emergency department, Middle Aged, medicine.disease, Anti-Bacterial Agents, Discontinuation, Community-Acquired Infections, Hospitalization, Early Diagnosis, Female, Radiography, Thoracic, Radiology, Treatment decision making, Emergency Service, Hospital, Chest radiograph, business
Abstract: Clinical decision making relative to community-acquired pneumonia (CAP) diagnosis is difficult. Chest radiograph is key in establishing parenchymal lung involvement. However, radiologic performance may lead to misdiagnosis, rendering questionable the use of chest computed tomography (CT) scan in patients with clinically suspected CAP.To assess whether early multidetector chest CT scan affects diagnosis and management of patients visiting the emergency department with suspected CAP.A total of 319 prospectively enrolled patients with clinically suspected CAP underwent multidetector chest CT scan within 4 hours. CAP diagnosis probability (definite, probable, possible, or excluded) and therapeutic plans (antibiotic initiation/discontinuation, hospitalization/discharge) were established by emergency physicians before and after CT scan results. The adjudication committee established the final CAP classification on Day 28.Chest radiograph revealed a parenchymal infiltrate in 188 patients. CAP was initially classified as definite in 143 patients (44.8%), probable or possible in 172 (53.8%), and excluded in 4 (1.2%). CT scan revealed a parenchymal infiltrate in 40 (33%) of the patients without infiltrate on chest radiograph and excluded CAP in 56 (29.8%) of the 188 with parenchymal infiltrate on radiograph. CT scan modified classification in 187 (58.6%; 95% confidence interval, 53.2-64.0), leading to 50.8% definite CAP and 28.8% excluded CAP, and 80% of modifications were in accordance with adjudication committee classification. Because of CT scan, antibiotics were initiated in 51 (16%) and discontinued in 29 (9%), and hospitalization was decided in 22 and discharge in 23.In CAP-suspected patients visiting the emergency unit, early CT scan findings complementary to chest radiograph markedly affect both diagnosis and clinical management. Clinical trial registered with www.clinicaltrials.gov (NCT 01574066).
Published: 2015
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46. Interstitial lung disease in anti-synthetase syndrome: Initial and follow-up CT findings

Author: Marie-Pierre Debray, Raphael Borie, Jean-Marc Naccache, Marie-Pierre Revel, Antoine Khalil, Candice Estellat, Cécile Toper, Pierre-Yves Brillet, and Dominique Israel-Biet
Subjects: Adult, Male, medicine.medical_specialty, Diagnosis, Differential, Fibrosis, medicine, Humans, Radiology, Nuclear Medicine and imaging, Interstitial pneumonia, Ct findings, Honeycombing, Traction bronchiectasis, Lung, Myositis, Aged, Retrospective Studies, business.industry, Interstitial lung disease, General Medicine, Middle Aged, medicine.disease, Bronchiectasis, respiratory tract diseases, Treatment Outcome, Female, Organizing pneumonia, Radiology, Lung Diseases, Interstitial, Tomography, X-Ray Computed, business, Follow-Up Studies
Abstract: Purpose To describe the initial and follow-up CT features of interstitial lung disease associated with anti-synthetase syndrome (AS-ILD). Materials and methods Two independent thoracic radiologists retrospectively analysed thin-section CT images obtained at diagnosis of AS-ILD in 33 patients (17 positive for anti-Jo1, 13 for anti-PL12, and three for anti-PL7 antibodies). They evaluated the pattern, distribution and extent of the CT abnormalities. They also evaluated the change in findings during follow-up (median 27 months; range 13–167 months) in 26 patients. Results At diagnosis, ground-glass opacities (100%), reticulations (87%) and traction bronchiectasis (76%) were the most common CT findings. Consolidations were present in 45% of patients. A non-specific interstitial pneumonia (NSIP), organizing pneumonia (OP) or mixed NSIP-OP CT pattern were observed in 15 out of 33 (45%), seven out of 33 (21%) and eight out of 33 (24%) patients, respectively, whereas the CT pattern was indeterminate in three patients. During follow-up, consolidations decreased or disappeared in 11 out of 12 patients (92%), among which seven within the first 6 months, but honeycombing progressed or appeared in ten out of 26 patients (38%) and overall disease extent increased in nine out of 26 patients (35%). Conclusion CT features at diagnosis of AS-ILD mainly suggest NSIP and OP, isolated or in combination. Consolidations decrease or disappear in most cases but the disease may progress to fibrosis in more than one third of patients.
Published: 2015
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47. Cystic Pulmonary Myxoid Liposarcoma Mimicking Endobronchial Blood Clot

Author: Martine Antoine, Frédéric Schlemmer, Jean-Marc Naccache, J. Assouad, Flora Crockett, Matthias Papo, Valérie Gounant, and Anne Bergeron
Subjects: Male, Pulmonary and Respiratory Medicine, medicine.medical_specialty, medicine.medical_treatment, Liposarcoma, Bronchoscopies, Diagnosis, Differential, 03 medical and health sciences, 0302 clinical medicine, Bronchoscopy, medicine.artery, medicine, Humans, Cyst, Embolization, Aged, Myxoid liposarcoma, Bronchus, medicine.diagnostic_test, business.industry, Bronchial Neoplasms, Thrombosis, medicine.disease, Liposarcoma, Myxoid, medicine.anatomical_structure, 030228 respiratory system, 030220 oncology & carcinogenesis, Radiology, Bronchial artery, business
Abstract: A 71-year-old-man was transferred to our hospital in November 2012 for a bronchial artery embolization in the context of recurrent blood clots obstructing the left lower bronchus. Since June 2012, he had been explored for a cystic hypermetabolic lesion of the entire left lobe, and underwent 3 bronchoscopies and 2 computed tomography scan-guided biopsies, with no success. A fourth bronchoscopy enabled the extraction of a large blood clot (8×1.5 cm) that obstructed the left main bronchus. The pathologic examination of the mucosal biopsy samples was inconclusive, whereas the cytologic examination of the blood clot revealed myxoid liposarcoma. Liposarcomas are the most common histologic types of soft-tissue sarcomas. They preferentially metastasize to the lungs and can appear as cystic mass. Bronchial obstruction by blood clots is not a rare finding on bronchoscopy, their main problem is their removal which could require rigid bronchoscope and large forceps. However, bronchial blood clot containing tumoral process had never been reported before. In conclusion, this case conveys 2 messages. First, pulmonary metastasis of myxoid liposarcoma can appear as cyst secondary to endobronchial tumoral growth. Second, endobronchial blood clots should always be sent for pathologic analysis.
Published: 2016
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48. Évolution après transplantation pulmonaire pour fibrose chez les patients porteurs d’une mutation du complexe télomérase

Author: G. Prévot, A. Roux, Romain Lazor, Hervé Mal, Sylvain Marchand-Adam, J. Le Pavec, Raphael Borie, Caroline Kannengiesser, Jean-Marc Naccache, Johanna Claustre, Sandrine Hirschi, Vincent Cottin, Antoine Froidure, Martine Reynaud-Gaubert, Stéphane Jouneau, M. Phillips Houlbracq, Lidwine Wemeau-Stervinou, S. Park, Bruno Crestani, and Hilario Nunes
Subjects: Pulmonary and Respiratory Medicine
Abstract: Introduction Les patients atteints d’une pneumopathie interstitielle diffuse associee a une mutation d’un gene regulateur du complexe telomerase (TRG) semblent presenter une moins bonne survie et des complications hematologiques plus frequentes apres transplantation pulmonaire (TxP). L’objectif de cette etude etait de determiner l’evolution apres TxP de ces patients dans une cohorte francaise. Methodes Tous les patients porteurs d’une mutation d’un TRG ayant eu une TxP en France entre 2009 et 2017 ont ete identifies par les centres de transplantation et le reseau francais OrphaLung. Resultats Vingt-sept patients ont ete inclus, dont 67 % etaient des hommes, avec des mutations de TERT (n = 17), TERC (n = 6), et RTEL1 (n = 4). Avant la greffe, 19 (70 %) presentaient une atteinte hematologique, dont 6 un syndrome myelodysplasique et 14 une hepatopathie. L’âge median lors de la TxP etait de 54 (± 9) ans. Apres la TxP, tous les patients presentaient une anemie, 74 % une thrombopenie, et 54 % une neutropenie. Quatre patients ont presente des complications hepatiques severes : une hemochromatose post transfusionnelle, une fibrose hepatique et deux patients une hypertension portale. 26 % ont presente une dysfonction chronique du greffon dans un delai median de 2,1 (± 1,3) ans. Quinze receveurs sont decedes (56 %) au cours du suivi. La mediane de survie apres la TP etait de 4,4 ans. La presence d’une myelodysplasie avant la TxP etait associee a une diminution de la survie (p Conclusion La survie apres TxP des patients porteurs d’une mutation d’un TRG est comparable a celle des patients sans mutation. Une TxP est possible apres un bilan exhaustif des comorbidites extra-respiratoires et une prise en charge post-greffe adaptee. La presence d’un syndrome myelodysplasique avant la greffe doit faire rediscuter le projet de greffe.
Published: 2019
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49. Caractéristiques et pronostiques d’une cohorte rétrospective de 134 patients (pts) traités par cyclophosphamide (CYC) IV pour une pneumopathie interstitielle diffuse (PID)

Author: Jean-Marc Naccache, J. Fillon, Muriel Fartoukh, P. Rigaud, R. Hindré, D. Sroussi, Antoine Parrot, Jacques Cadranel, and A. Canellas
Subjects: Pulmonary and Respiratory Medicine
Abstract: Introduction Les PID sont d’etiologies variees et conduisent a un declin fonctionnel progressif ou rapide quel que soit le traitement. Le CYC permet une stabilisation de la fonction respiratoire chez les pts traites pour des PID de sclerodermie systemique. Peu de donnees sont disponibles pour les PID d’autres etiologies. Le but de cette etude est de decrire les caracteristiques d’une cohorte de pts traites par CYC et de determiner d’eventuels facteurs pronostics. Methodes Cohorte retrospective monocentrique de 134 pts traites entre le 01/01/2005 et le 31/12/2017 dans un service de pneumologie universitaire pour PID idiopathique (n = 53) (22 FPI, 20 PINS idiopathiques, 7 PIA, 1 POC, 1 PPFE, 2 PID inclassables), secondaire a une connectivite (n = 49) (myosites/anti-synthetase, 17 ; myosites associe au Gougerot Sjogren [GJ], 3 ; GJ, 8 ; connectivites mixtes, 6 ; polyarthrite rhumatoide, 6 ; sclerodermie systemique [SS], 7 ; SS associe au GJ, 1 ; scleromyosite, 1), IPAF ou PHS. L’interrogation du logiciel hospitalier « CHIMIO », a permis un recueil exhaustif des pts traites de facon homogene par CYC : 600 mg/m2 IV a j1, j15, j28, M2. Tous les pts (hormis 4) recevaient une dose mediane de 48,4 mg (range : 5–150) d’equivalent prednisone. Les donnees epidemiologiques, cliniques, fonctionnelles et scanographiques ont ete recueillies avant traitement, apres CYC, puis a M6, M12, M24 et a la derniere evaluation disponible. Le critere principal de jugement etait la proportion de pts decedes ou transplantes a la date du 31/12/2018. Resultats Caracteristiques des 134 pts a la date du traitement par CYC : cf. Tableau 1 . Conclusion Nous rapportons l’experience d’une large cohorte de PID de causes variees mais traitees de maniere homogene par CYC. L’effet du CYC sur la CVF, la DLCO et le besoin en O2, le taux de mortalite/transplantation a la date de point, ainsi que les facteurs pronostiques seront communiques lors du CPLF.
Published: 2020
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50. Manifestations pulmonaires au cours de l’infection par le VIH : démarche diagnostique

Author: F. Crockett, H Ferrand, Jacques Cadranel, Charles Mayaud, C. Rioux, Jean-Marc Naccache, and Yazdan Yazdanpanah
Subjects: Pulmonary and Respiratory Medicine, Pediatrics, medicine.medical_specialty, medicine.diagnostic_test, business.industry, Human immunodeficiency virus (HIV), Physical examination, medicine.disease_cause, Antiretroviral therapy, Hiv infected, medicine, CD4 Lymphocyte, Chest radiograph, business
Abstract: The spectrum of pulmonary diseases that can affect human immunodeficiency virus (HIV)-infected patients is wide and includes both HIV and non-HIV-related conditions. Opportunistic infections and neoplasms remain a major concern even in the current era of combination antiretroviral therapy. Although these diseases have characteristic clinical and radiological features, there can be considerable variation in these depending on the patient's CD4 lymphocyte count. The patient's history, physical examination, CD4 count and chest radiograph features must be considered in establishing an appropriate diagnostic algorithm. In this article, we propose different diagnostic approaches HIV infected to patients with respiratory symptoms depending on their clinico-radiological pattern.
Published: 2014
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