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1. Lung function in Birt-Hogg-Dubé syndrome: a retrospective analysis of 96 patients

Author

C. Daccord, V. Cottin, G. Prévot, Y. Uzunhan, J. F. Mornex, P. Bonniaud, R. Borie, A. Briault, M. A. Collonge-Rame, B. Crestani, G. Devouassoux, O. Freynet, A. Gondouin, P. A. Hauss, C. Khouatra, S. Leroy, S. Marchand-Adam, C. Marquette, D. Montani, J. M. Naccache, G. Nadeau, N. Poulalhon, M. Reynaud-Gaubert, M. Salaun, B. Wallaert, J. F. Cordier, M. Faouzi, R. Lazor, and the OrphaLung network

Subjects
Birt-Hogg-Dube syndrome, FLCN protein, human, Respiratory function tests, Pleurodesis, Medicine
Abstract

Abstract Background Birt-Hogg-Dubé syndrome (BHD) is a rare autosomal dominant disorder caused by mutations in the FLCN gene coding for folliculin. Its clinical expression includes cutaneous fibrofolliculomas, renal tumors, multiple pulmonary cysts, and recurrent spontaneous pneumothoraces. Data on lung function in BHD are scarce and it is not known whether lung function declines over time. We retrospectively assessed lung function at baseline and during follow-up in 96 patients with BHD. Results Ninety-five percent of BHD patients had multiple pulmonary cysts on computed tomography and 59% had experienced at least one pneumothorax. Mean values of forced expiratory volume in 1 second (FEV1), forced vital capacity (FVC), FEV1/FVC ratio, and total lung capacity were normal at baseline. Mean (standard deviation) residual volume (RV) was moderately increased to 116 (36) %pred at baseline, and RV was elevated > 120%pred in 41% of cases. Mean (standard deviation) carbon monoxide transfer factor (DLco) was moderately decreased to 85 (18) %pred at baseline, and DLco was decreased

Published
2020
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3. Pulmonary alveolar proteinosis

Author

B. Crestani, R. Epaud, M. Aubier, M-C. Dombret, C. Taille, M-P. Debray, C. Danel, and R. Borie

Subjects
Granulocyte macrophage-colony stimulating factor, macrophage, pulmonary lavage, rituximab, surfactant, Diseases of the respiratory system, RC705-779
Abstract

Pulmonary alveolar proteinosis (PAP) is a rare pulmonary disease characterised by alveolar accumulation of surfactant. It may result from mutations in surfactant proteins or granulocyte macrophage-colony stimulating factor (GM-CSF) receptor genes, it may be secondary to toxic inhalation or haematological disorders, or it may be auto-immune, with anti-GM-CSF antibodies blocking activation of alveolar macrophages. Auto-immune alveolar proteinosis is the most frequent form of PAP, representing 90% of cases. Although not specific, high-resolution computed tomography shows a characteristic “crazy paving” pattern. In most cases, bronchoalveolar lavage findings establish the diagnosis. Whole lung lavage is the most effective therapy, especially for auto-immune disease. Novel therapies targeting alveolar macrophages (recombinant GM-CSF therapy) or anti-GM-CSF antibodies (rituximab and plasmapheresis) are being investigated. Our knowledge of the pathophysiology of PAP has improved in the past 20 yrs, but therapy for PAP still needs improvement.

Published
2011

6. Transplantation pulmonaire en France : actualisation des indications et contre-indications en 2022

Author

J. Le Pavec, C. Pison, S. Hirschi, V. Bunel, P. Mordant, O. Brugière, M. Le Guen, A. Olland, B. Coiffard, B. Renaud-Picard, A. Tissot, G. Brioude, R. Borie, B. Crestani, G. Deslée, S. Stelianides, H. Mal, A. Schuller, L. Falque, G. Lorillon, A. Tazi, P.R. Burgel, D. Grenet, S. De Miranda, A. Bergeron, D. Launay, V. Cottin, H. Nunes, D. Valeyre, Y. Uzunhan, G. Prévot, O. Sitbon, D. Montani, L. Savale, M. Humbert, E. Fadel, O. Mercier, J.F. Mornex, G. Dauriat, and M. Reynaud-Gaubert

Subjects
Pulmonary and Respiratory Medicine
Published
2022

8. Télomères et poumon

Author

C. Guérin, B. Crestani, C. Dupin, L. Kawano-Dourado, I. Ba, C. Kannengiesser, and R. Borie

Subjects
Pulmonary and Respiratory Medicine
Published
2022

12. Updated indications and contraindications in 2022 for lung transplantation in France

Author

J, Le Pavec, C, Pison, S, Hirschi, V, Bunel, P, Mordant, O, Brugière, M, Le Guen, A, Olland, B, Coiffard, B, Renaud-Picard, A, Tissot, G, Brioude, R, Borie, B, Crestani, G, Deslée, S, Stelianides, H, Mal, A, Schuller, L, Falque, G, Lorillon, A, Tazi, P R, Burgel, D, Grenet, S, De Miranda, A, Bergeron, D, Launay, V, Cottin, H, Nunes, D, Valeyre, Y, Uzunhan, G, Prévot, O, Sitbon, D, Montani, L, Savale, M, Humbert, E, Fadel, O, Mercier, J F, Mornex, G, Dauriat, M, Reynaud-Gaubert, Pôle des Cardiopathies Congénitales du Nouveau-Né à L'adulte - Centre Constitutif Cardiopathies Congénitales Complexes M3C, Groupe Hospitalier Paris Saint-Joseph, Hôpital Marie-Lannelongue, Inserm U999, Université Paris-Saclay, Hôpital Marie-Lannelongue, Université Paris-Sud - Paris 11 (UP11), Université Grenoble Alpes (UGA), Service de pneumologie [Grenoble], Centre Hospitalier Universitaire [Grenoble] (CHU), Nouvel Hôpital Civil de Strasbourg, 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), Chirurgie thoracique et transplantation pulmonaire [Suresnes] (CT2P - Hôpital Foch), Hôpital Foch [Suresnes], ERAMET (ERAMET), Service de chirurgie thoracique, CHU Bordeaux [Bordeaux], Centre recherche en CardioVasculaire et Nutrition = Center for CardioVascular and Nutrition research (C2VN), Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE), Aix Marseille Université (AMU), Hôpital Nord [CHU - APHM], Nanomédecine Régénérative (NanoRegMed), Université de Strasbourg (UNISTRA)-Institut National de la Santé et de la Recherche Médicale (INSERM), Les Hôpitaux Universitaires de Strasbourg (HUS), Université de Strasbourg (UNISTRA), Centre de Recherche en Transplantation et Immunologie - Center for Research in Transplantation and Translational Immunology (U1064 Inserm - CR2TI), Institut National de la Santé et de la Recherche Médicale (INSERM)-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), Team 4 : Deciphering organ immune regulation in inflammation and transplantation (DORI-t) (U1064 Inserm - CR2TI), Nantes Université (Nantes Univ)-Nantes Université (Nantes Univ)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Nantes Université - UFR de Médecine et des Techniques Médicales (Nantes Univ - UFR MEDECINE), AP-HP - Hôpital Bichat - Claude Bernard [Paris], Pathologies Pulmonaires et Plasticité Cellulaire - UMR-S 1250 (P3CELL), Université de Reims Champagne-Ardenne (URCA)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre Hospitalier Universitaire de Reims (CHU Reims), Institut de réadaptation [Achères] (IR), CIC Hôpital Bichat, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-UFR de Médecine, Pôle Thorax et Vaisseaux [CHU Grenoble], Immunologie humaine, physiopathologie & immunothérapie (HIPI (UMR_S_976 / U976)), Service de pneumologie [Saint-Louis], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Cité (UPCité), Hopital Saint-Louis [AP-HP] (AP-HP), Institut Cochin (IC UM3 (UMR 8104 / U1016)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Paris Cité (UPCité), Hôpitaux Universitaires de Genève (HUG), Centre de Recherche Épidémiologie et Statistique Sorbonne Paris Cité (CRESS (U1153 / UMR_A_1125 / UMR_S_1153)), 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)-Université Sorbonne Paris Cité (USPC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE), Institute for Translational Research in Inflammation - U 1286 (INFINITE (Ex-Liric)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), CHU Lille, 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), Institut National de la Santé et de la Recherche Médicale (INSERM), 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), Physiologie de l'Insecte : Signalisation et Communication (PISC), Institut National de la Recherche Agronomique (INRA)-Université Pierre et Marie Curie - Paris 6 (UPMC)-AgroParisTech, Service de Pneumologie [AP-HP Hôpital Avicenne, Bobigny], Hypertension pulmonaire : physiopathologie et innovation thérapeutique (HPPIT), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris-Saclay, Hôpital Bicêtre, Centre Chirurgical Marie Lannelongue (CCML), Université Paris-Saclay, UMR INRA / ENV Lyon / Univ. Lyon 1 : Lentivirus des petits ruminants, Institut National de la Recherche Agronomique (INRA)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Ecole Nationale Vétérinaire de Lyon (ENVL), and Institut Hospitalier Universitaire Méditerranée Infection (IHU Marseille)

Subjects
Emergency registration, MESH: Respiratory Insufficiency* / etiology, MESH: France / epidemiology, MESH: Humans, Survival, Contraindications, [SDV]Life Sciences [q-bio], MESH: Quality of Life, Super urgence, MESH: Lung Transplantation* / methods, Contre-indication, Indication, List, Lung transplantation, Survie, Quality of Life, Humans, Transplantation pulmonaire, France, Inscription sur liste, Respiratory Insufficiency, MESH: Contraindications
Abstract

International audience; Lung transplantation (LTx) is the last-resort treatment for end-stage respiratory insufficiency, whatever its origin, and represents a steadily expanding field of endeavor. Major developments have been impelled over the years by painstaking efforts at LTx centers to improve donor and recipient selection, and multifaceted attempts have been made to meet the challenges raised by surgical management, perioperative care, and long-term medical complications. The number of procedures has increased, leading to improved post-LTx prognosis. One consequence of these multiple developments has been a pruning away of contraindications over time, which has, in some ways, complicated the patient selection process. With these considerations in mind, the Francophone Pulmonology Society (Société de Pneumology de Langue Française [SPLF]) has set up a task force to produce up-to-date working guidelines designed to assist pulmonologists in managing end-stage respiratory insufficiency, determining which patients may be eligible for LTx, and appropriately timing LTx-center referral. The task force has examined the most recent literature and evaluated the risk factors that continue to limit patient survival after LTx. Ideally, the objectives of LTx are to prolong life while improving quality of life. The guidelines developed by the task force apply to a limited resource and are consistent with the ethical principles described below.; La transplantation pulmonaire (TxP) constitue le traitement ultime de l’insuffisance respiratoire terminale quelle qu’en soit l’origine et voit son activité en permanente expansion. Le travail minutieux des centres de TxP portant sur la sélection des donneurs et des receveurs d’un greffon pulmonaire ainsi que de l’ensemble des efforts fournis pour relever les défis de la prise en charge chirurgicale, péri-opératoire et la gestion des complications médicales de la TxP à plus long terme ont permis une augmentation du nombre de procédures et l’amélioration du pronostic post-TxP. Les membres du groupe de travail de la Société de pneumologie de langue française (SPLF) ont réalisé une mise à jour des données connues de la littérature et analysé les facteurs de risque qui limitent les chances de survie après la TxP. L’objectif de ce travail a été de guider les pneumologues dans leur prise en charge de l’insuffisance respiratoire terminale, de les aider à identifier les patients potentiellement éligibles à la TxP et de déterminer selon quels délais les adresser à centre de TxP. Les objectifs de la TxP demeurent l’allongement de la vie et l’amélioration de la qualité de vie des malades. Les propositions faites dans ce document portent sur une ressource limitée et restent guidées par des principes éthiques décrits plus loin.

Published
2022

14. Pleuropulmonary manifestations of VEXAS syndrome

Author

R Borie, M P Debray, A Audemard, A Guedon, L Terriou, V Lacombe, E Lazaro, A Meyer, A Mathian, S Ardois, G Vial, T Moulinet, B Terrier, Y Jamilloux, M Heblig, J D Bouaziz, E Zakine, R Outh, S Groslerons, A Bigot, E Flamarion, M Kostine, P Henneton, S Humbert, A Constentin, M Samson, N Magy, C Dieval, P Biscay, H Lobbes, K Meghit, A Servettaz, L Adelaide, J Graveleau, B De Sainte Marie, V Guillotin, J Galland, O Kostminder, S Georgin Lavaille, and A Mekinian

Published
2022

15. COVID-19 in patients with Pulmonary Alveolar Proteinosis A European multicenter study

Author

S Papiris, I Campo, F Mariani, M Kallieri, L Kolilekas, A I Papaioannou, E G Chousein, E Cetinkaya, F Bonella, R Borie, M Kokosi, T Pickworth, M Molina-Molina, M Gasa, E Radzikowska, J Fijolek, S Jouneau, E Gomez, C Mccarthy, E Bendstrup, W J Piotrowski, R Pabary, A Hadchouel, N Coolen-Allou, T Alfaro, C R Cordeiro, E Antonogiannaki, I P Tomos, D Papakosta, T Kontakiotis, P Panagiotou, K Douros, A Schams, S Lettieri, V Papaevangelou, C Kanaka-Gantenbein, A Karakatsani, S Loukides, U Costabel, B Crestani, C Morgan, R Tazawa, A Bush, M Griese, and E D Manali

Published
2022

22. Caractérisation phénotypique des pneumopathies interstitielles diffuses liées à des mutations de SFTPC et d’ABCA3 chez l’adulte

Author

R. Diesler, N. Nathan, M. Legendre, S. Amsellem, R. Borie, A. Bourdin, H. Nunes, L. Wemeau, A. Gondouin, S. Dominique, H. Morisse Pradier, E. Blanchard, J. Macey, V. Bironneau, F. Gagnadoux, E. Manali, S. Papiris, S. Hirschi, S. Marchand-Adam, A. Justet, M. Reynaud-Gaubert, G. Lorillon, P.Y. Brillet, S. Si-Mohamed, and V. Cottin

Subjects
Pulmonary and Respiratory Medicine
Published
2023

23. Prévalence du cancer du poumon dans une cohorte de patients porteurs d’une mutation d’un gène lié au surfactant

Author

A. Brudon, M. Legendre, A. Mageau, N. Nathan, J. Bermudez, D. Bouvry, J. Cadranel, A. Cazes, B. Crestani, T. Dégot, C. Delestrain, R. Diesler, R. Epaud, M.P. Debray, P. Fanen, A. Gaubert, E. Manali, A. Gondouin, A. Guillaumot, S. Hirschi, S. Leroy, S. Marchand-Adam, H. Nunes, S. Amselem, C. Picard, G. Prevot, M. Reynaud, P. De Vuyst, L. Wémeau-Stervinou, G. Zalcman, V. Cottin, and R. Borie

Subjects
Pulmonary and Respiratory Medicine
Published
2023

28. [Pulmonary phenotypes of inborn errors of metabolism]

Author

W, Mauhin, A, Brassier, J, London, B, Subran, A, Zeggane, Q, Besset, C, Jammal, C, Montardi, C, Mellot, C, Strauss, R, Borie, and O, Lidove

Subjects
Phenotype, Metabolic Diseases, Humans, Amino Acid Metabolism, Inborn Errors, Metabolism, Inborn Errors
Abstract

Inborn metabolic diseases or inborn errors of metabolism comprise a large number of rare and heterogeneous genetic diseases categorized in several subgroups depending on their pathophysiologic mechanisms. In this review, we focus on different metabolic diseases with respiratory symptoms in adults: lysosomal glycosphingolipidoses such as acid sphingomyelinase deficiency (Niemann-Pick types A and B disease), Gaucher, Fabry, Pompe diseases and mucopolysaccharidoses in general. We also address classical homocystinuria, which is a monogenic vascular disease, Hermansky-Pudlak syndrome, which is associated with disorders in the lysosomal-related-organelles, and lysinuric protein intolerance, which is due to an amino-acid transporter defect. Presentation and prognosis of these diseases are highly heterogeneous, and respiratory impairment may be central and prognostic. Many are primarily pediatric, and diagnoses are often delivered during childhood. Improved pediatric management has enabled better prognosis and new phenotype of the diseases in the adulthood. Some others can be diagnosed during adulthood. While some diseases call for specific, specialized treatment, all necessitate systematic multidisciplinary management. It is of paramount importance that a pneumologist be familiar with these phenotypes, most of which can benefit from early diagnosis and early therapeutic management with dedicated innovative treatments.

Published
2022

30. Les échanges plasmatiques ne semblent pas être associés à un meilleur pronostic chez les patients avec une atteinte pulmonaire rapidement progressive d’une dermatomyosite anti-MDA5

Author

P. Bay, M. Pineton De Chambrun, V. Rothstein, M. Mahevas, A. Roux, B. Zuber, D. Israël-Biet, B. Hervier, A. Tazi, L. Mouthon, A. Mekinian, C. Deligny, R. Borie, A. Meyer, P. Priou, L. Savale, P.Y. Brillet, H. Nunes, Y. Allenbach, Y. Uzunhan, Institut E3M [CHU Pitié-Salpêtrière], CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Institut de cardiologie [CHU Pitié-Salpêtrière], IMRB - 'Transfusion et Maladies du Globule Rouge' [Créteil] (U955 Inserm - UPEC), 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)-Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR10-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Institut de Planétologie et d'Astrophysique de Grenoble (IPAG), Centre National d'Études Spatiales [Toulouse] (CNES)-Observatoire des Sciences de l'Univers de Grenoble (OSUG ), Institut national des sciences de l'Univers (INSU - CNRS)-Université Savoie Mont Blanc (USMB [Université de Savoie] [Université de Chambéry])-Centre National de la Recherche Scientifique (CNRS)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE)-Université Grenoble Alpes (UGA)-Météo-France -Institut national des sciences de l'Univers (INSU - CNRS)-Université Savoie Mont Blanc (USMB [Université de Savoie] [Université de Chambéry])-Centre National de la Recherche Scientifique (CNRS)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE)-Université Grenoble Alpes (UGA)-Météo-France, Université Paris Cité - UFR Médecine [Santé] (UPCité UFR Médecine), Université Paris Cité (UPCité), 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), Service de Département de médecine interne et immunologie clinique [CHU Pitié-Salpêtrière] (DMIIC), Hopital Saint-Louis [AP-HP] (AP-HP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Institut Cochin (IC UM3 (UMR 8104 / U1016)), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Departement Hospitalo- Universitaire - Inflammation, Immunopathologie, Biothérapie [Paris] (DHU - I2B), Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS)-CHU Saint-Antoine [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), CHU de la Martinique [Fort de France], AP-HP - Hôpital Bichat - Claude Bernard [Paris], Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM), Hypertension pulmonaire : physiopathologie et innovation thérapeutique (HPPIT), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris-Saclay, Hôpital Marie-Lannelongue, Université Paris Nord (Paris 13), Institut National de la Santé et de la Recherche Médicale (INSERM), 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 de recherche en Myologie – U974 SU-INSERM, Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), and Service de Pneumologie [AP-HP Hôpital Avicenne, Bobigny]

Subjects
[SDV]Life Sciences [q-bio], Gastroenterology, Internal Medicine
Abstract

International audience

Published
2022

34. Recommandations pratiques pour le diagnostic et la prise en charge de la fibrose pulmonaire idiopathique – Actualisation 2021. Version courte

Author

V. Cottin, P. Bonniaud, J. Cadranel, B. Crestani, S. Jouneau, S. Marchand-Adam, H. Nunes, L. Wémeau-Stervinou, E. Bergot, E. Blanchard, R. Borie, A. Bourdin, C. Chenivesse, A. Clément, E. Gomez, A. Gondouin, S. Hirschi, F. Lebargy, C.-H. Marquette, D. Montani, G. Prévot, S. Quetant, M. Reynaud-Gaubert, M. Salaun, O. Sanchez, B. Trumbic, K. Berkani, P.-Y. Brillet, M. Campana, L. Chalabreysse, G. Chatté, D. Debieuvre, G. Ferretti, J.-M. Fourrier, N. Just, M. Kambouchner, B. Legrand, F. Le Guillou, J.-P. Lhuillier, A. Mehdaoui, J.-M. Naccache, C. Paganon, M. Rémy-Jardin, S. Si-Mohamed, P. Terrioux, Physiologie & médecine expérimentale du Cœur et des Muscles [U 1046] (PhyMedExp), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM)-Centre National de la Recherche Scientifique (CNRS), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Microbes évolution phylogénie et infections (MEPHI), Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS), Service de Pneumologie et Allergie - Hôpital Nord [Marseille], Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Equipe Quantification en Imagerie Fonctionnelle (QuantIF-LITIS), Laboratoire d'Informatique, du 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)-Université Le Havre Normandie (ULH), and Institut National des Sciences Appliquées (INSA)-Normandie Université (NU)-Institut National des Sciences Appliquées (INSA)

Subjects
Pulmonary and Respiratory Medicine, [SDV]Life Sciences [q-bio], ComputingMilieux_MISCELLANEOUS
Abstract

International audience

Published
2022

36. Atteintes respiratoires basses de la papillomatose respiratoire récurrente

Author

R. Ratsihorimanana, T. Maitre, G. Mangiapan, M.T. Ngo, N. Guibert, C. Fournier, R. Kessler, S. Jouneau, A. Bourdin, T. Egenod, D. Wermert, N. Farvrot, H. Nunes, V. Gounant, R. Borie, R. Caliandro, J.F. Boitiaux, J. Mankikian, J. Camuset, V. Cottin, J.M. Vergnon, and J. Cadranel

Subjects
Pulmonary and Respiratory Medicine
Published
2023

37. Les pneumopathies interstitielles diffuses de la polyarthrite rhumatoïde sont associées à une taille plus courte des télomères et un excès de variants rares au sein de gènes impliqués dans les téloméropathies

Author

L. Kawano, P.A. Juge, T. Doyle, A. Walts, B. Granger, R. Borie, C. Kannengiesser, M. Soubrier, V. Dominique, C. Boileau, L. Wemeau-Stervinou, M.P. Debray, S. Marchand-Adam, C. Richez, H. Nunes, R.M. Flipo, T. Schaeverbeke, N. Saidenberg-Kermanac’h, L. Joyce, B. Crestani, and P. Dieudé

Subjects
Rheumatology
Published
2022

39. Excès de variants rares délétères au sein de gènes appartenant à la voir JAK-STAT chez des patients atteints de pneumopathie interstitielle diffuse associée à la polyarthrite rhumatoïde

Author

P.A. Juge, S. Gazal, R. Borie, L. Wemeau-Stervinou, M.P. Debray, S. Ottaviani, S. Marchand-Adam, C. Richez, H. Nunes, P. Richette, C. Kannengiesser, P. Froguel, J. Avouac, J. Sibilia, R.M. Flipo, V. Cottin, T. Schaeverbeke, M. Soubrier, N. Saidenberg-Kermanac’h, V. Dominique, C. Boileau, B. Crestani, and P. Dieudé

Subjects
Rheumatology
Published
2022

48. [Telomeres and lung]

Author

C, Guérin, B, Crestani, C, Dupin, L, Kawano-Dourado, I, Ba, C, Kannengiesser, and R, Borie

Subjects
Vital Capacity, Humans, Nuclear Proteins, Cell Cycle Proteins, Telomere, Lung Diseases, Interstitial, Lung, Idiopathic Pulmonary Fibrosis
Abstract

Genetic studies of familial forms of interstitial lung disease (ILD) have led to the discovery of telomere-related gene (TRG) mutations (TERT, TERC, RTEL1, PARN, DKC1, TINF2, NAF1, NOP10, NHP2, ACD, ZCCH8) in approximately 30% of familial ILD forms. ILD patients with TRG mutation are also subject to extra-pulmonary (immune-hematological, hepatic and/or mucosal-cutaneous) manifestations. TRG mutations may be associated not only with idiopathic pulmonary fibrosis (IPF), but also with non-IPF ILDs, including idiopathic and secondary ILDs, such as hypersensitivity pneumonitis (HP). The presence of TRG mutation may also be associated with an accelerated decline of forced vital capacity (FVC) or poorer prognosis after lung transplantation, notwithstanding which, usual ILD treatments may be proposed. Lastly, patients and their relatives are called upon to reduce their exposure to environmental lung toxicity, and are likely to derive benefit from specific genetic counseling and pre-symptomatic genetic testing.

Published
2021

49. POS0064 EPIDEMIOLOGY OF INTERSTITIAL LUNG DISEASE IN SYSTEMIC LUPUS ERYTHEMATOSUS IN FRANCE: A NATION-WIDE POPULATION-BASED STUDY OVER 10 YEARS

Author

A. Mageau, R. Borie, B. Crestani, J. F. Timsit, T. Papo, and K. Sacre

Subjects
Rheumatology, Immunology, Immunology and Allergy, General Biochemistry, Genetics and Molecular Biology
Abstract

BackgroundData regarding interstitial lung disease (ILD) in the setting of systemic lupus erythematosus (SLE) are limitedObjectivesWe used a nation-wide database to determine the incidence and the prevalence of ILD in SLE patients.MethodsCharacteristics of all SLE inpatients admitted between 2011 and 2012 in France were analyzed through the French medico-administrative database. Features associated with the presence of ILD were studied. Cox proportional hazard model was used to look for the impact of ILD on survival from the first stay to 2020. The incidence rate of ILD in SLE was estimated by analysing the onset of ILD from 2013 to 2020 in SLE patients who had no evidence of ILD in 2013.ResultsBetween 2011 and 2012, 10 460 SLE patients had at least one hospital stay and could be traced until 2020. Among them, 134 (1.2%) had an ILD diagnosed at baseline. The frequency of ILD in SLE was higher in patients who had an associated autoimmune disease – such as Sjögren’s syndrome or systemic sclerosis (29.9% vs 5.9%; p< 0.0001). ILD was associated with an increased risk of death in SLE after adjustment on comorbidities in the multivariable analysis (HR [CI95] 1.992 [1.420-2.794]; p ConclusionIn SLE, ILD is exceedingly rare, often associated with another systemic autoimmune disorder and appears as a major risk factor for death.Disclosure of InterestsArthur Mageau: None declared, Raphael Borie Grant/research support from: RocheBoerhniger Ingelheim, Bruno Crestani: None declared, Jean-Francois Timsit: None declared, Thomas Papo: None declared, karim sacre: None declared

Published
2022

50. POS0062 A RISK SCORE TO DETECT SUBCLINICAL RHEUMATOID ARTHRITIS-ASSOCIATED INTERSTITIAL LUNG DISEASE

Author

P. A. Juge, B. Granger, M. P. Debray, E. Ebstein, F. Louis-Sidney, J. Kedra, T. Doyle, R. Borie, A. Constantin, B. Combe, R. M. Flipo, X. Mariette, O. Vittecoq, A. Saraux, G. Carvajal Alegria, J. Sibilia, F. Berenbaum, C. Kannengiesser, C. Boileau, J. Sparks, B. Crestani, B. Fautrel, and P. Dieudé

Subjects
Rheumatology, Immunology, Immunology and Allergy, General Biochemistry, Genetics and Molecular Biology
Abstract

BackgroundDespite a high morbi-mortality rate, there are no definite strategy for subclinical interstitial lung disease (ILD) screening in patients with rheumatoid arthritis (RA).ObjectivesOur objectives were: 1. to identify risk factors for subclinical RA-ILD in a prospective discovery cohort (ESPOIR) 2.to develop a risk score for subclinical RA-ILD and 3. to validate the risk score in an independent replication cohort (TRANSLATE 2).MethodsPatients without pulmonary symptoms from 2 prospective RA cohorts who underwent chest HRCT scans were included. All patients were genotyped for MUC5B rs35705950. A risk score based on independent risk factors for subclinical RA-ILD was developed using multiple logistic regression in the discovery cohort. The risk score was tested for validation in the replication cohort.ResultsDiscovery and replication cohorts included 163 and 89 patients, respectively. Subclinical ILD was detected in 19.0% and 16.9% of the patients, respectively. In the discovery cohort, independent risk factors for subclinical RA-ILD were the MUC5B rs35705950 T risk allele (odds ratio [OR]=3.74; 95% confidence interval [CI] [1.37–10.39], male sex (OR=3.93; 95%CI [1.40–11.39]), older age at RA onset (for each year, OR=1.10; 95%CI [1.04–1.16]) and increased mean DAS28-ESR (for each unit, OR=2.03; 95%CI [1.24–3.42]). We developed a risk score for subclinical RA-ILD with AUC=0.82; 95%CI [0.70–0.94] (sensitivity (Se)=71.0%) and specificity (Sp)=79.6%). The risk score was validated in the replication cohort with AUC=0.78; 95%CI [0.65–0.92] (Se=86.7%, Sp=62.2%).ConclusionOur risk score could help identifying patients at high-risk for subclinical RA-ILD before the onset of pulmonary symptoms.Disclosure of InterestsPierre-Antoine Juge Speakers bureau: AstraZeneca, Boehringer Ingelheim, Bristol Myers Squibb, Consultant of: Bristol Myers Squibb, Benjamin Granger: None declared, Marie-Pierre Debray: None declared, Esther Ebstein: None declared, Fabienne Louis-Sidney: None declared, Joanna KEDRA: None declared, Tracy Doyle: None declared, Raphael Borie: None declared, Arnaud Constantin Consultant of: Abbvie, Amgen, Biogen, BMS, Boehringer Ingelheim, Fresenius Kabi, Galapagos, Janssen, Lilly, Medac, MSD, Mylan, Novartis, Pfizer, Procter & Gamble, Roche, Sanofi, UCB, Viatris, Bernard Combe Consultant of: AbbVie, BMS, Eli-Lilly, Gilead, Janssen, Merck, Novartis, Pfizer, Roche-Chugai, Sanofi, UCB, René-Marc Flipo Consultant of: Abbvie, Janssen, MSD and Pfizer. He reports research grants from Abbvie, Janssen, Novartis and Pfizer, Xavier Mariette Consultant of: BMS, Gilead, Janssen, Pfizer, Samsung, UCB, Olivier VITTECOQ: None declared, Alain Saraux: None declared, Guillermo CARVAJAL ALEGRIA: None declared, Jean Sibilia Consultant of: AbbVie, Lilly, MSD, Amgen, Pfizer, BMS, Janssen, Roche, Sandoz, Sanofi-Genzyme, SOBI, UCB, Novartis, Grant/research support from: AbbVie, Lilly, MSD, Amgen, Pfizer, BMS, Janssen, Roche, Sandoz, Sanofi-Genzyme, SOBI, UCB, Novartis, Francis Berenbaum: None declared, Caroline Kannengiesser: None declared, Catherine Boileau: None declared, Jeffrey Sparks Consultant of: AbbVie, Boehringer Ingelheim, Bristol Myers Squibb, Gilead, Inova Diagnostics, Janssen, Optum, and Pfizer, Grant/research support from: National Institute of Arthritis and Musculoskeletal and Skin Diseases (grant numbers R01 AR077607, P30 AR070253, and P30 AR072577), The R. Bruce and Joan M. Mickey Research Scholar Fund, Bristol Myers Squibb,Bruno Crestani Speakers bureau: Boehringer Ingelheim, AstraZeneca, Roche, Sanofi, Grant/research support from: MedImmune, Roche, Boehringer Ingelheim, Bruno Fautrel Consultant of: AbbVie, Amgen, Biogen, BMS, Celgene, Fresenius Kabi, Janssen, Lilly, Medac, MSD, NORDIC Pharma, Novartis, Pfizer, Roche, SOBI, UCB, Grant/research support from: AbbVie, Lilly, MSD, Pfizer, Philippe Dieudé Speakers bureau: Roche – Chugai, Bristol Myers Squibb, Consultant of: Pfizer, Roche – Chugai, Bristol Myers Squibb, Abbvie, MSD, Grant/research support from: Novartis

Published
2022

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