Author: "Sandrine Hirschi" - Searchworks@Jio Institute Digital Library Search Results

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108 results on '"Sandrine Hirschi"'

1. Phenotypes and outcome of diffuse pulmonary non-amyloid light chain deposition disease

Author: François Lestelle, Catherine Beigelman, David Rotzinger, Salim Si-Mohamed, Mouhamad Nasser, Lidwine Wemeau, Sandrine Hirschi, Grégoire Prevot, Antoine Roux, Vincent Bunel, Emmanuel Gomez, Laurent Sohier, Helene Morisse Pradier, Martine Reynaud Gaubert, Anne Gondouin, Romain Lazor, Jean-Charles Glerant, Françoise Thivolet Bejui, Magali Colombat, Vincent Cottin, and the OrphaLung network
Subjects: Light chain deposition disease, Lung cysts, Bronchiectasis, Lung transplantation, Diseases of the respiratory system, RC705-779
Abstract: Abstract Background Light chain deposition disease (LCDD) is a very rare entity. Clinical manifestations of LCDD vary according to the organs involved. Data on pulmonary LCDD are scarce and limited to small series or case reports. This study aimed to describe the characteristics and outcome of diffuse pulmonary non-amyloid LCDD localized to the lungs. Study design and methods A multicenter retrospective cohort study was conducted. Clinical characteristics were collected, and chest CTs were centrally reviewed. The diagnosis of pulmonary non-amyloid LCDD was confirmed by immunohistochemistry. Results Thirty-one cases were identified (68% female), with a median age at diagnosis of 50 years (IQR 20). Baseline FEV1/FVC was
Published: 2024
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2. Outcome of lung transplantation for adults with interstitial lung disease associated with genetic disorders of the surfactant system

Author: Julien Bermudez, Nadia Nathan, Benjamin Coiffard, Antoine Roux, Sandrine Hirschi, Tristan Degot, Vincent Bunel, Jérôme Le Pavec, Julie Macey, Aurélie Le Borgne, Marie Legendre, Vincent Cottin, Pascal-Alexandre Thomas, Raphaël Borie, and Martine Reynaud-Gaubert
Subjects: Medicine
Abstract: Background Interstitial lung disease associated with genetic disorders of the surfactant system is a rare entity in adults that can lead to lung transplantation. Our objective was to describe the outcome of these patients after lung transplantation. Methods We conducted a retrospective, multicentre study, on adults who underwent lung transplantation for such disease in the French lung transplant centres network, from 1997 to 2018. Results 20 patients carrying mutations in SFTPA1 (n=5), SFTPA2 (n=7) or SFTPC (n=8) were included. Median interquartile range (IQR) age at diagnosis was 45 (40–48) years, and median (IQR) age at lung transplantation was 51 (45–54) years. Median overall survival after transplantation was 8.6 years. Two patients had a pre-transplant history of lung cancer, and two developed post-transplant lung cancer. Female gender and a body mass index
Published: 2023
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3. Gender Differences in Idiopathic Pulmonary Fibrosis: Are Men and Women Equal?

Author: Lucile Sesé, Hilario Nunes, Vincent Cottin, Dominique Israel-Biet, Bruno Crestani, Stephanie Guillot-Dudoret, Jacques Cadranel, Benoit Wallaert, Abdellatif Tazi, Bernard Maître, Gregoire Prévot, Sylvain Marchand-Adam, Sandrine Hirschi, Sandra Dury, Violaine Giraud, Anne Gondouin, Philippe Bonniaud, Julie Traclet, Karine Juvin, Raphael Borie, Zohra Carton, Olivia Freynet, Thomas Gille, Carole Planès, Dominique Valeyre, and Yurdagül Uzunhan
Subjects: idiopathic pulmonary fibrosis, gender differences, occupational exposures, lung transplantation, women, Medicine (General), R5-920
Abstract: Background: Idiopathic pulmonary fibrosis (IPF) is characterized by a male predominance. The aim of the study was to explore gender differences in a well-designed French multicentre prospective IPF cohort (COhorte FIbrose, COFI) with a 5-year follow-up.Methods: Between 2007 and 2010, 236 patients with incident IPF were included in COFI. Gender characteristics were compared using a t-test, Chi-squared test and ANOVA, as appropriate. Survival analyses were performed.Results: Fifty-one (22%) females and 185 (78%) males with an average age at diagnosis of 70.1 ± 9.20 and 67.4 ± 10.9 years, respectively, were included in the cohort. Women were significantly less exposed to tobacco smoke [never n = 32 (62.7%) vs. n = 39 (21.1%), p < 0.001] and to occupational exposure [n = 7 (13.7%) vs. n = 63 (34.1%), p = 0.012]. Baseline forced vital capacity, % of predicted (FVC%) was significantly better in women compare to men (83.0% ± 25.0 v. 75.4% ± 18.7 p = 0.046). At presentation honeycombing and emphysema on CT scan were less common in women [n = 40 (78.4%) vs. n = 167 (90.3%) p = 0.041] and [n = 6 (11.8%) vs. n = 48 (25.9%) p = 0.029], respectively. During follow-up fewer women were transplanted compared to men [n = 1 (1.96%) vs. n = 20 (10.8%) p = 0.039]. Medians of survival were comparable by gender [31 months (CI 95%: 28–40) vs. 40 months (CI 95%: 33–72) p = 0.2]. After adjusting for age and FVC at inclusion, being a woman was not associated to a better survival.Conclusions: Women appear to have less advanced disease at diagnosis, maybe due to less exposure history compare to men. Disease progression and overall survival remains comparable regardless gender, but women have less access to lung transplantation.
Published: 2021
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4. Refining inflammatory myopathies incidence and characteristics: a quadruple source capture‐recapture survey using 2017 <scp>ACR</scp> / <scp>EULAR</scp> criteria

Author: Léa Debrut, Margherita Giannini, Delphine Klein, Lionel Spielmann, Philippe Mertz, Thierry Martin, Aleksandra Nadaj‐Pakleza, Sandrine Hirschi, Benoit Nespola, Béatrice Lannes, Joëlle Terzic, Olivier Hinschberger, Benjamin Dervieux, Dan Lipsker, Laurent Arnaud, Jacques‐Eric Gottenberg, Jean François Kleinmann, Bernard Geny, François Séverac, Michel Velten, Jean Sibilia, and Alain Meyer
Subjects: Rheumatology, Immunology, Immunology and Allergy
Published: 2023
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5. 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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6. Computed tomographic airway morphology after targeted lung denervation treatment in COPD

Author: Jorine E. Hartman, Felix J.F. Herth, Pallav Shah, Christophe Pison, Arschang Valipour, Dirk-Jan Slebos, Christine Abele, Irene Firlinger, Kiran Kothakuzhakal, Marina Duller, Bernd Lamprecht, Roland Kropfmueller, Kornelia Holzmann, Sandra Rathmeier, Ralf Hubner, Leonore Erdmann, Bettina Temmesfeld-Wollbrück, Christoph Ruwwe Glösenkamp, Wolfgang Gesierich, Frank Reichenberger, Christa Niehaus, Felix Herth, Ralf Eberhardt, Daniela Gompelmann, Brigitte Rump, Kaid Darwiche, Stephan Eisenmann, Ulrike Kaiser, Birte Schwarz, Ulrike Sampel, Christian Schumann, Robert Kaiser, Kathryn Schumann-Stoiber, Dirk Skowasch, Sabine Ring, Amandine Briault, Francois Arbib, Marie Jondot, Thierry Perez, Clement Fournier, Regis Matran, Michele Catto, Nathalie Bautin, Virginie De Broucker, Marie Willemin, Anne Prevotat, Ludivine Wemeau, Alice Gicquello, Morgane Foulon, Hasna Camara, Gaetan Deslee, Herve Vallerand, Sandra Dury, Delphine Gras, Margaux Bonnaire-Verdier, Romain Kessler, Sandrine Hirschi, Michele Porzio, Tristan Degot, Mathieu Canuet, Armelle Schuller, Julien Stauder, Sahra Ali Azouaou, Armelle Marceau, Hervé Mal, Yolande Costa, Pallav L. Shah, Justin Garner, Karthi Srikanthan, Cielito Caneja, John Thornton, Nick Ten Hacken, Jorine Hartman, Karin Klooster, Sonja Augustijn, Peter Bonta, Jouke Annema, Marianne van de Pol, Annika Goorsenberg, VU University medical center, Groningen Research Institute for Asthma and COPD (GRIAC), Pulmonology, ACS - Pulmonary hypertension & thrombosis, AII - Infectious diseases, AII - Inflammatory diseases, AII - Cancer immunology, CCA - Cancer biology and immunology, and CCA - Imaging and biomarkers
Subjects: Pulmonary and Respiratory Medicine
Abstract: This post-hoc analysis of the AIRFLOW-2 trial investigated the changes in airway CT-parameters after targeted lung denervation (TLD) and whether these changes are associated with treatment response. In the treatment group (n = 32), an improvement in air trapping was significantly associated with an improvement in residual volume (RV). Furthermore, improvements in Pi10 and airway lumen were significantly associated with an improvement in both RV and FEV1. Our results could suggest that when improving airway characteristics like decreasing airway wall thickness and increasing the airway lumen, this leads to less air trapping and an improvement in clinical outcomes.
Published: 2023
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7. 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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8. 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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9. Clinical impact of TERT somatic mutation in telomerase-related gene mutation carriers after lung transplantation

Author: Ibrahima Ba, Caroline Kannengiesser, Hervé Mal, Martine Reynaud-Gaubert, Vincent Cottin, Sandrine Hirschi, Clément Picard, Raphael Borie, 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), 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), 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), Les Hôpitaux Universitaires de Strasbourg (HUS), Laboratoire des Sciences du Numérique de Nantes (LS2N), Institut National de Recherche en Informatique et en Automatique (Inria)-Centre National de la Recherche Scientifique (CNRS)-IMT Atlantique (IMT Atlantique), Institut Mines-Télécom [Paris] (IMT)-Institut Mines-Télécom [Paris] (IMT)-École Centrale de Nantes (Nantes Univ - ECN), Nantes Université (Nantes Univ)-Nantes Université (Nantes Univ)-Nantes université - UFR des Sciences et des Techniques (Nantes univ - UFR ST), Nantes Université - pôle Sciences et technologie, Nantes Université (Nantes Univ)-Nantes Université (Nantes Univ)-Nantes Université - pôle Sciences et technologie, Nantes Université (Nantes Univ), Centre de Référence des Maladies Pulmonaires Rares [AP-HP Hôpital Bichat], and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)
Subjects: Pulmonary and Respiratory Medicine, Heterozygote, Transplantation, [SDV]Life Sciences [q-bio], telomerase-related gene, Telomere, idiopathic pulmonary fibrosis, Mutation, TERT somatic mutation, lung transplantation, Humans, Surgery, Cardiology and Cardiovascular Medicine, Telomerase, digital droplet PCR
Abstract: International audience; Almost 25% of patients with pulmonary fibrosis referred for lung transplantation have a germline rare variant of a telomere-related gene. Acquired TERT promoter mutations may counterbalanced the germline defect and reduce the risk of hematological complications in this population. In a series of 34 patients with a germline telomere-related gene mutation who underwent lung transplantation, 12 (35%) patients had at least 1 acquired TERT promoter mutation. Six patients presented myelodysplasia before lung transplantation, with no difference between patients with and without an acquired TERT promoter mutation. After lung transplantation, myelodysplasia developed in only 1 of 8 patients with an acquired TERT promoter mutation versus 7 of 18 patients without a mutation. Survival did not differ between patients with and without an acquired mutation. The presence of an acquired TERT promoter mutation could be associated with reduced hematological complications after transplantation and with better outcome in telomere-related gene mutation carriers but requires further study. (C) 2022 International Society for Heart and Lung Transplantation.
Published: 2022
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10. 2022 Update of indications and contraindications for lung transplantation in France

Author: Jérôme Le Pavec, Christophe Pison, Sandrine Hirschi, Vincent Bunel, Pierre Mordant, Olivier Brugière, Morgan Le Guen, Anne Olland, Benjamin Coiffard, Benjamin Renaud-Picard, Adrien Tissot, Geoffrey Brioude, Raphaël Borie, Bruno Crestani, Gaétan Deslée, Sandrine Stelianides, Hervé Mal, Armelle Schuller, Loïc Falque, Gwenaëlle Lorillon, Abdellatif Tazi, Pierre Regis Burgel, Dominique Grenet, Sandra De Miranda, Anne Bergeron, David Launay, Vincent Cottin, Hilario Nunes, Dominique Valeyre, Yurdagul Uzunhan, Grégoire Prévot, Olivier Sitbon, David Montani, Laurent Savale, Marc Humbert, Elie Fadel, Olaf Mercier, Jean François Mornex, Gaëlle Dauriat, Martine Reynaud-Gaubert, Université Paris-Sud - Paris 11 (UP11), Hôpital Marie-Lannelongue, Centre Hospitalier Universitaire [Grenoble] (CHU), Les Hôpitaux Universitaires de Strasbourg (HUS), CIC Hôpital Bichat, 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)-Institut National de la Santé et de la Recherche Médicale (INSERM)-UFR de Médecine, Université Paris Cité (UPCité), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de pneumologie [Hôpital Foch], Hôpital Foch [Suresnes], Université de Versailles Saint-Quentin-en-Yvelines - UFR Sciences de la santé Simone Veil (UVSQ Santé), Université de Versailles Saint-Quentin-en-Yvelines (UVSQ), Nanomédecine Régénérative (NanoRegMed), Université de Strasbourg (UNISTRA)-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Strasbourg, Hôpital Nord [CHU - APHM], 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), Hôpital Guillaume-et-René-Laennec [Saint-Herblain], Centre hospitalier universitaire de Nantes (CHU Nantes), 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), 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), 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 des maladies respiratoires et allergiques [CHU Reims], Centre Hospitalier Universitaire de Reims (CHU Reims), 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), Institut de réadaptation [Achères] (IdR), Service de Chirurgie Thoracique, CHU Strasbourg-Nouvel Hôpital Civil, 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), 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), Centre de référence des maladies auto-immunes systémiques rares du Nord et Nord Ouest [CHRU Lille], Hôpital Claude Huriez [Lille], CHU Lille-CHU Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU 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), 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), Physiologie de l'Insecte : Signalisation et Communication (PISC), Institut National de la Recherche Agronomique (INRA)-Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National Agronomique Paris-Grignon (INA P-G), Hôpital Avicenne [AP-HP], Centre hospitalier Saint-Joseph [Paris], Pôle Clinique des Voies respiratoires [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Service de pneumologie [CHU Bicêtre], 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, Centre d'Investigation Clinique [Bron] (CIC1407), Hospices Civils de Lyon (HCL)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Groupement Hospitalier Est [Bron], Groupe Hospitalier Paris Saint-Joseph (hpsj), and Aix Marseille Université (AMU)
Subjects: Pulmonary and Respiratory Medicine, High emergency allocation program, [SDV]Life Sciences [q-bio], Lung transplantation contraindications, Lung transplantation indications, Candidate selection
Abstract: International audience; Lung transplantation (LTx) is a steadily expanding field. The considerable developments have been driven over the years by indefatigable work conducted at LTx centers to improve donor and recipient selection, combined with multifaceted efforts to overcome challenges raised by the surgical procedure, perioperative care, and long-term medical complications. One consequence has been a pruning away of contraindications over time, which has, in some ways, complicated the patient selection process. The Francophone Pulmonology Society (Société de Pneumology de Langue Française, SPLF) 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 examined the most recent literature and evaluated the risk factors that limit patient survival after LTx. Ideally, the objectives of LTx are to prolong life while also 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.
Published: 2023
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11. 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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12. Sarcoïdose osseuse : étude cas-témoin multicentrique

Author: Marc Scherlinger, Patrice Cacoub, Hervé Devilliers, Christopher Rein, Christophe Richez, Catherine Chapelon-Abric, Matthieu Groh, Imen Ben Hassine, B. Meunier, Laurent Arnaud, Nathalie Saidenberg-Kermanac’h, Karim Sacre, Philip Bielefeld, Noémie Chanson, Camille Glanowski, Nicolas Schleinitz, Cloé Comarmond, David Saadoun, and Sandrine Hirschi
Subjects: 030203 arthritis & rheumatology, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, 030212 general & internal medicine
Abstract: Resume Objectif Decrire la presentation clinique, la distribution des lesions, le traitement et les resultats de la sarcoidose osseuse. Methodes Il s’agit d’une etude retrospective francaise multicentrique de patients atteints de sarcoidose confirmee par une biopsie a analyse des patients presentant un granulome confirme par une biopsie sans necrose caseeuse et, soit des manifestations cliniques d’atteintes osseuses, soit des anomalies osseuses a l’imagerie. Les patients presentant une sarcoidose avec atteinte osseuse (cas) ont ete compares a 264 patients atteints de sarcoidose sans manifestations osseuses apparies selon l’âge et le sexe (temoins). Resultats Dans le groupe sarcoidose osseuse (n = 88), 42 patients (48 %) presentaient des symptomes osseux touchant le squelette axial (69 %) et/ou appendiculaire (58 %). Les os les plus frequemment touches sur l’imagerie etaient le rachis (52 %), le pelvis (42 %), les mains (22 %) et le femur (19 %). Par rapport aux temoins, les cas avaient des taux plus eleves d’atteinte mediastinale (93 % contre 47 %), des ganglions lymphatiques extra-thoraciques (66 % contre 21 %), pulmonaire (90 % contre 65 %) et cutanee (44 % contre 23 %) (toutes les valeurs p Conclusion Le rachis et le pelvis etaient les os les plus frequemment touches chez les patients presentant une sarcoidose osseuse. Par rapport aux temoins, les patients atteints de sarcoidose osseuse avaient des taux plus eleves d’atteinte des ganglions lymphatiques thoraciques et extra-thoraciques, pulmonaire et cutanee et d’hypercalcemie. La reponse aux glucocorticoides combines avec le methotrexate ou l’hydroxychloroquine etait bonne chez la majorite des patients.
Published: 2020
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13. Renal involvement in eosinophilic granulomatosis with polyangiitis (EGPA): a multicentric retrospective study of 63 biopsy-proven cases

Author: Marie Essig, Cécile-Audrey Durel, David Jayne, Christelle Barbet, Sandrine Hirschi-Santelmo, Thomas Le Gallou, Antoine Bardy, Jean-Jacques Boffa, Sylvain Marchand-Adam, Dimitri Titeca-Beauport, Grégory Pugnet, Xavier Belenfant, Camille Taillé, Xavier Puéchal, Cédric Rafat, Pascal Godmer, Vincent Cottin, Vítor Teixeira, Alexandre Karras, Julien Bouet, Renato Alberto Sinico, Jacques Gaultier, Philippe Guilpain, Daniel Engelbert Blockmans, Yoann Crabol, Christian Agard, Christophe Deligny, Durel, C, Sinico, R, Teixeira, V, Jayne, D, Belenfant, X, Marchand-Adam, S, Pugnet, G, Gaultier, J, Le Gallou, T, Titeca-Beauport, D, Agard, C, Barbet, C, Bardy, A, Blockmans, D, Boffa, J, Bouet, J, Cottin, V, Crabol, Y, Deligny, C, Essig, M, Godmer, P, Guilpain, P, Hirschi-Santelmo, S, Rafat, C, Puéchal, X, Taillé, C, and Karras, A
Subjects: Adult, Male, 0301 basic medicine, medicine.medical_specialty, Adolescent, 030232 urology & nephrology, Churg-Strauss Syndrome, Kidney, Gastroenterology, Nephropathy, Young Adult, 03 medical and health sciences, 0302 clinical medicine, renal biopsy, Rheumatology, Membranous nephropathy, Internal medicine, Eosinophilic, medicine, Humans, Pharmacology (medical), Kidney transplantation, Aged, Retrospective Studies, Aged, 80 and over, medicine.diagnostic_test, business.industry, Acute Kidney Injury, Middle Aged, medicine.disease, renal involvement, EGPA, 030104 developmental biology, medicine.anatomical_structure, vasculiti, glomerulonephriti, Female, Renal biopsy, Granulomatosis with polyangiitis, business, Vasculitis
Abstract: Objective Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic small-vessel vasculitis characterized by asthma, hypereosinophilia and ANCA positivity in 40% of patients. Renal involvement is rare and poorly described, leading to this renal biopsy-proven based study in a large EGPA cohort. Methods We conducted a retrospective multicentre study including patients fulfilling the 1990 ACR criteria and/or the 2012 revised Chapel Hill Consensus Conference criteria for EGPA and/or the modified criteria of the MIRRA trial, with biopsy-proven nephropathy. Results Sixty-three patients [27 women, median age 60 years (18–83)] were included. Renal disease was present at vasculitis diagnosis in 54 patients (86%). ANCA were positive in 53 cases (84%) with anti-MPO specificity in 44 (83%). All patients had late-onset asthma. Peripheral neuropathy was present in 29 cases (46%), alveolar haemorrhage in 10 (16%). The most common renal presentation was acute renal failure (75%). Renal biopsy revealed pauci-immune necrotizing GN in 49 cases (78%). Membranous nephropathy (10%) and membranoproliferative GN (3%) were mostly observed in ANCA-negative patients. Pure acute interstitial nephritis was found in six cases (10%); important interstitial inflammation was observed in 28 (44%). All patients received steroids with adjunctive immunosuppression in 54 cases (86%). After a median follow-up of 51 months (1–296), 58 patients (92%) were alive, nine (14%) were on chronic dialysis and two (3%) had undergone kidney transplantation. Conclusion Necrotizing pauci-immune GN is the most common renal presentation in ANCA-positive EGPA. ANCA-negative patients had frequent atypical renal presentation with other glomerulopathies such as membranous nephropathy. An important eosinophilic interstitial infiltration was observed in almost 50% of cases.
Published: 2020
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14. Impact of Gender on the Characteristics of Patients with Idiopathic Pulmonary Fibrosis Included in the RaDiCo-ILD Cohort

Author: Sandrine Hirschi, Sonia Gueguen, M. Chevereau, Serge Amselem, Anne-Sophie Gamez, Martine Reynaud-Gaubert, Philippe Bonniaud, Dominique Israel-Biet, Annick Clement, I. Dufaure-Garé, Sylvain Marchand-Adam, Lidwine Wemeau-Stervinou, David Montani, Vincent Cottin, Jacques Cadranel, Hilario Nunes, Sébastien Quétant, Bruno Crestani, Stéphane Jouneau, 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), Hospices Civils de Lyon (HCL), 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), 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 Pontchaillou [Rennes], Hypoxie et Poumon : pneumopathologies fibrosantes, modulations ventilatoires et circulatoires (H&P), UFR SMBH-Université Sorbonne Paris Nord, 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), 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), Hôpital Claude Huriez [Lille], CHU Lille, Hôpital Sainte-Marguerite [CHU - APHM] (Hôpitaux Sud ), 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 Européen Georges Pompidou [APHP] (HEGP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), CHU Tenon [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Pathologies Respiratoires : Protéolyse et Aérosolthérapie, Université de Tours (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM), Pôle Thorax et Vaisseaux [CHU Grenoble], Centre Hospitalier Universitaire [Grenoble] (CHU), CHU Strasbourg, Hypertension pulmonaire : physiopathologie et innovation thérapeutique (HPPIT), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris-Saclay, Hôpital Lapeyronie [Montpellier] (CHU), CHU Trousseau [APHP], French National Research Agency under the 'cohorts' programme of the Investissements d'Avenir (Investments for the Future) French National Research Agency (ANR) [ANR-10-COHO-0003], ANR-10-COHO-0003,RADICO,Cohorte nationale maladies rares(2010), École pratique des hautes études (EPHE), Physiopathologie des maladies génétiques d'expression pédiatrique (UMRS_933), Sorbonne Université (SU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique )-Institut National de la Santé et de la Recherche Médicale (INSERM)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Université d'Angers (UA), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Paris (UP), Université de Bourgogne (UB)-Institut National de la Santé et de la Recherche Médicale (INSERM)-AgroSup Dijon - Institut National Supérieur des Sciences Agronomiques, de l'Alimentation et de l'Environnement, Centre Constitutif de Référence des Maladies Pulmonaires Rares [AP-HP Tenon], Centre national de référence des maladies pulmonaires rares [Lyon] (CRMPM)-Service de Pneumologie - Oncologie Thoracique - Maladies Pulmonaires Rares [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)-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), Université de Tours-Institut National de la Santé et de la Recherche Médicale (INSERM), Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), Université de Bourgogne (UB)-AgroSup Dijon - Institut National Supérieur des Sciences Agronomiques, de l'Alimentation et de l'Environnement-Institut National de la Santé et de la Recherche Médicale (INSERM), Maladies génétiques d'expression pédiatrique [CHU Trousseau] (Inserm U933), Centre national de référence des maladies pulmonaires rares [Lyon] (CRMPM)-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)-CHU Tenon [AP-HP], UF de Génétique moléculaire [CHU Trousseau], Centre de référence national pour les maladies respiratoires rares de l’enfant RespiRare [CHU Trousseau], Service de Pneumologie pédiatrique [CHU Trousseau], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-CHU Trousseau [APHP], Chard-Hutchinson, Xavier, and Cohortes - Cohorte nationale maladies rares - - RADICO2010 - ANR-10-COHO-0003 - COHO - VALID
Subjects: Pulmonary and Respiratory Medicine, Male, medicine.medical_specialty, Vital capacity, Registry, [SDV]Life Sciences [q-bio], Idiopathic pulmonary fibrosis, [SDV.MHEP.PSR]Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract, Pulmonary function testing, Cohort Studies, 03 medical and health sciences, 0302 clinical medicine, Rare Diseases, Internal medicine, medicine, Humans, 030212 general & internal medicine, Honeycombing, Idiopathic interstitial pneumonia, 10. No inequality, Emphysema, Patient characteristics, business.industry, Interstitial lung disease, Gender, respiratory system, medicine.disease, Confidence interval, 3. Good health, respiratory tract diseases, [SDV] Life Sciences [q-bio], 030228 respiratory system, Cohort, Quality of Life, [SDV.MHEP.PSR] Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract, Female, France, business, Lung Diseases, Interstitial
Abstract: Background: There is growing evidence of gender-specific phenotypic differences among patients with idiopathic pulmonary fibrosis (IPF), which may affect patient outcomes. Objectives: We present the characteristics of patients with IPF at inclusion in the French Rare Disease Cohort – Interstitial Lung Disease (RaDiCo-ILD) with the aim of characterizing gender-specific phenotypic differences. Methods: Patients with IPF who were enrolled in the national, multicentre RaDiCo-ILD cohort were included. Demographic characteristics, comorbidities, health-related quality of life (HRQoL) scores, pulmonary function, chest imaging, and IPF treatment were collected at inclusion and described by gender. Results: The cohort included 724 patients with IPF (54% of RaDiCo-ILD cohort), of whom 82.9% were male. The proportion of male and female patients with a prior history of smoking was 75.0% and 26.8%, respectively. Emphysema was present in 17.0% (95% confidence interval [CI]: 10.0, 24.0) of men and 5.4% (95% CI: 1.2, 9.6) of women. At inclusion, females had poorer HRQoL than males based on St. George’s Respiratory Questionnaire scores (48.5 [95% CI: 43.9, 53.0] and 41.5 [39.4, 43.6], respectively). The mean forced vital capacity per cent predicted was 77.7% (95% CI: 76.2, 79.3) and 87.4% (83.4, 91.4) for males and females, respectively. Honeycombing on high-resolution computed tomography (HRCT) was present in 70.8% (95% CI: 61.0, 80.6) of males and 45.8% (95% CI: 35.1, 56.5) of females. Conclusions: This analysis of patients with IPF at inclusion in the RaDiCo-ILD cohort provides evidence that comorbid emphysema, lung volume reduction, and honeycombing on HRCT are more common characteristics of males than females.
Published: 2022
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15. Clinical spectrum time course in non-Asian patients positive for anti-MDA5 antibodies

Author: Lorenzo, Cavagna, Federica, Meloni, Alain, Meyer, Gianluca, Sambataro, Mirko, Belliato, Ellen De Langhe, Cavazzana, Ilaria, Nicolò, Pipitone, Konstantinos, Triantafyllias, Marta, Mosca, Simone, Barsotti, Giuseppe, Zampogna, Alessandro, Biglia, Giacomo, Emmi, Marianne De Visser, Anneke Van Der Kooi, Paola, Parronchi, Sandrine, Hirschi, Jose Antonio Pereira da Silva, Carlo Alberto Scirè, Federica, Furini, Margherita, Giannini, Olga Martinez Gonzalez, Laura, Damian, Yves, Piette, Vanessa, Smith, Antonio, Mera-Valera, Javier, Bachiller-Corral, Ivan Cabezas Rodriguez, Anahy, M Brandy-Garcia, François, Maurier, Julie, Perrin, Juan, Gonzalez-Moreno, Ulrich, Drott, Christiane, Delbruck, Andreas, Schwarting, Eugenio, Arrigoni, Gian Domenico Sebastiani, Annamaria, Iuliano, Carlotta, Nannini, Luca, Quartuccio, Ana, B Rodriguez Cambron, Maria, Á Blázquez Cañamero, Ignacio Villa Blanco, Giovanni, Cagnotto, Alberto, Pesci, Francesco, Luppi, Giulia, Dei, Fredeswinda Isabel Romero Bueno, Franceschini, Franco, Ilaria, Chiapparoli, Giovanni, Zanframundo, Sara, Lettieri, Ludovico De Stefano, Maurizio, Cutolo, Alessandro, Mathieu, Matteo, Piga, Sergio, Prieto-González, Maria Francisca Moraes-Fontes, Joao Eurico Fonseca, Vega, Jovani, Valeria, Riccieri, Alessandro, Santaniello, Stephen, Montfort, David, Bilocca, Gian Luca Erre, Elena, Bartoloni, Roberto, Gerli, M Cristina Monti, Hanns, M Lorenz, Domenico, Sambataro, Silvia Bellando Randone, Udo, Schneider, Claudia, Valenzuela, Raquel, Lopez-Mejias, Jose, Cifrian, Mayra, Mejia, Monserrat-Ixchel Gonzalez Perez, Sarah, Wendel, Marco, Fornaro, Giacomo De Luca, Giovanni, Orsolini, Maurizio, Rossini, Philippe, Dieude, Johannes, Knitza, Santos, Castañeda, Reinhard, E Voll, Jorge, Rojas-Serrano, Adele, Valentini, Carlo, Vancheri, Marco, Matucci-Cerinic, Eugen, Feist, Veronica, Codullo, Florenzo, Iannone, Jorg, H Distler, Carlomaurizio, Montecucco, Miguel, A Gonzalez-Gay, AENEAS collaborative group, Neurology, ANS - Neuroinfection & -inflammation, AII - Inflammatory diseases, EURO-NMD, Cavagna, L, Meloni, F, Meyer, A, Sambataro, G, Belliato, M, De Langhe, E, Cavazzana, I, Pipitone, N, Triantafyllias, K, Mosca, M, Barsotti, S, Zampogna, G, Biglia, A, Emmi, G, De Visser, M, Van Der Kooi, A, Parronchi, P, Hirschi, S, da Silva, J, Scire, C, Furini, F, Giannini, M, Martinez Gonzalez, O, Damian, L, Piette, Y, Smith, V, Mera-Valera, A, Bachiller-Corral, J, Cabezas Rodriguez, I, Brandy-Garcia, A, Maurier, F, Perrin, J, Gonzalez-Moreno, J, Drott, U, Delbruck, C, Schwarting, A, Arrigoni, E, Sebastiani, G, Iuliano, A, Nannini, C, Quartuccio, L, Rodriguez Cambron, A, Blazquez Canamero, M, Villa Blanco, I, Cagnotto, G, Pesci, A, Luppi, F, Dei, G, Romero Bueno, F, Franceschini, F, Chiapparoli, I, Zanframundo, G, Lettieri, S, De Stefano, L, Cutolo, M, Mathieu, A, Piga, M, Prieto-Gonzalez, S, Moraes-Fontes, M, Fonseca, J, Jovani, V, Riccieri, V, Santaniello, A, Montfort, S, Bilocca, D, Erre, G, Bartoloni, E, Gerli, R, Monti, M, Lorenz, H, Sambataro, D, Bellando Randone, S, Schneider, U, Valenzuela, C, Lopez-Mejias, R, Cifrian, J, Mejia, M, Gonzalez Perez, M, Wendel, S, Fornaro, M, De Luca, G, Orsolini, G, Rossini, M, Dieude, P, Knitza, J, Castaneda, S, Voll, R, Rojas-Serrano, J, Valentini, A, Vancheri, C, Matucci-Cerinic, M, Feist, E, Codullo, V, Iannone, F, Distler, J, Montecucco, C, Gonzalez-Gay, M, Repositório da Universidade de Lisboa, Cavagna, Lorenzo, Meloni, Federica, Meyer, Alain, Sambataro, Gianluca, Belliato, Mirko, De Langhe, Ellen, Cavazzana, Ilaria, Pipitone, Nicolò, Triantafyllias, Konstantino, Mosca, Marta, Barsotti, Simone, Zampogna, Giuseppe, Biglia, Alessandro, Emmi, Giacomo, De Visser, Marianne, Van Der Kooi, Anneke, Parronchi, Paola, Hirschi, Sandrine, da Silva, Jose Antonio Pereira, Scirè, Carlo Alberto, Furini, Federica, Giannini, Margherita, Martinez Gonzalez, Olga, Damian, Laura, Piette, Yve, Smith, Vanessa, Mera-Valera, Antonio, Bachiller-Corral, Javier, Cabezas Rodriguez, Ivan, Brandy-Garcia, Anahy M, Maurier, Françoi, Perrin, Julie, Gonzalez-Moreno, Juan, Drott, Ulrich, Delbruck, Christiane, Schwarting, Andrea, Arrigoni, Eugenio, Sebastiani, Gian Domenico, Iuliano, Annamaria, Nannini, Carlotta, Quartuccio, Luca, Rodriguez Cambron, Ana B, Blázquez Cañamero, Maria Á, Villa Blanco, Ignacio, Cagnotto, Giovanni, Pesci, Alberto, Luppi, Francesco, Dei, Giulia, Romero Bueno, Fredeswinda Isabel, Franceschini, Franco, Chiapparoli, Ilaria, Zanframundo, Giovanni, Lettieri, Sara, De Stefano, Ludovico, Cutolo, Maurizio, Mathieu, Alessandro, Piga, Matteo, Prieto-González, Sergio, Moraes-Fontes, Maria Francisca, Fonseca, Joao Eurico, Jovani, Vega, Riccieri, Valeria, Santaniello, Alessandro, Montfort, Stephen, Bilocca, David, Erre, Gian Luca, Bartoloni, Elena, Gerli, Roberto, Monti, M Cristina, Lorenz, Hanns M, Sambataro, Domenico, Bellando Randone, Silvia, Schneider, Udo, Valenzuela, Claudia, Lopez-Mejias, Raquel, Cifrian, Jose, Mejia, Mayra, Gonzalez Perez, Monserrat-Ixchel, Wendel, Sarah, Fornaro, Marco, De Luca, Giacomo, Orsolini, Giovanni, Rossini, Maurizio, Dieude, Philippe, Knitza, Johanne, Castañeda, Santo, Voll, Reinhard E, Rojas-Serrano, Jorge, Valentini, Adele, Vancheri, Carlo, Matucci-Cerinic, Marco, Feist, Eugen, Codullo, Veronica, Iannone, Florenzo, Distler, Jorg H, Montecucco, Carlomaurizio, and Gonzalez-Gay, Miguel A
Subjects: Lung Diseases, Interferon-Induced Helicase, IFIH1, rapidly progressive interstitial lung diseases, idiopathic inflammatory myopathies, idiopathic inflammatory myopathie, Immunology, Middle Aged, Prognosis, Dermatomyositis, rapidly progressive interstitial lung disease, Rheumatology, melanoma differentiation-associated protein 5 antibody, rapidly progressive interstitial lung diseases, idiopathic inflammatory myopathies, Humans, Immunology and Allergy, Female, Lung Diseases, Interstitial, Interferon-Induced Helicase, Interstitial, melanoma differentiation-associated protein 5 antibody, Autoantibodies, Retrospective Studies, IFIH1
Abstract: © Copyright Clinical and Experimental Rheumatology 2022., Objectives: To define the clinical spectrum time-course and prognosis of non-Asian patients positive for anti-MDA5 antibodies. Methods: We conducted a multicentre, international, retrospective cohort study. Results: 149 anti-MDA5 positive patients (median onset age 53 years, median disease duration 18 months), mainly females (100, 67%), were included. Dermatomyositis (64, 43%) and amyopathic dermatomyositis (47, 31%), were the main diagnosis; 15 patients (10%) were classified as interstitial pneumonia with autoimmune features (IPAF) and 7 (5%) as rheumatoid arthritis. The main clinical findings observed were myositis (84, 56%), interstitial lung disease (ILD) (108, 78%), skin lesions (111, 74%), and arthritis (76, 51%). The onset of these manifestations was not concomitant in 74 cases (50%). Of note, 32 (21.5%) patients were admitted to the intensive care unit for rapidly progressive-ILD, which occurred in median 2 months from lung involvement detection, in the majority of cases (28, 19%) despite previous immunosuppressive treatment. One-third of patients (47, 32% each) was ANA and anti-ENA antibodies negative and a similar percentage was anti-Ro52 kDa antibodies positive. Non-specific interstitial pneumonia (65, 60%), organising pneumonia (23, 21%), and usual interstitial pneumonia-like pattern (14, 13%) were the main ILD patterns observed. Twenty-six patients died (17%), 19 (13%) had a rapidly progressive-ILD. Conclusions: The clinical spectrum of the anti-MDA5 antibodies-related disease is heterogeneous. Rapidly-progressive ILD deeply impacts the prognosis also in non-Asian patients, occurring early during the disease course. Anti-MDA5 antibody positivity should be considered even when baseline autoimmune screening is negative, anti-Ro52 kDa antibodies are positive, and radiology findings show a NSIP pattern.
Published: 2022

16. Initiation of extracorporeal photopheresis in lung transplant patients with mild to moderate refractory BOS: A single-center real-life experience

Author: Justine Leroux, Sandrine Hirschi, Arnaud Essaydi, Alain Bohbot, Tristan Degot, Armelle Schuller, Anne Olland, Romain Kessler, and Benjamin Renaud-Picard
Subjects: Pulmonary and Respiratory Medicine, Photopheresis, Humans, Syndrome, Bronchiolitis Obliterans, Lung Transplantation, Retrospective Studies
Abstract: Bronchiolitis obliterans syndrome (BOS) is the main limitation to long-term survival following lung transplantation. Several studies generated promising results regarding the efficacy of extracorporeal photopheresis (ECP) in BOS management. We aimed to compare FEV1 evolution in ECP-treated versus non-ECP treated patients among BOS recipients.Overall, 25 BOS patients were included after receiving optimized treatment. Data were collected retrospectively. Twelve patients with moderate and refractory BOS received ECP treatment.Among non-ECP treated control patients (n = 13), six experienced persistent decline without undergoing ECP for various reasons. ECP stabilized pre-ECP lung function during the subsequent 6 to 24 months (repeated measures one-way Anova, p = 0.002), without any significant impact observed by either FEV1 decline speed prior to ECP or time between BOS diagnosis and ECP onset. ECP-treated patients displayed a similar risk of an additional permanent 20% or higher drop in FEV1 after BOS onset compared to controls, but a lower risk compared to control decliners (p = 0.05). ECP quickly stabilized FEV1 decline in refractory BOS patients compared to non-treated decliners.We confirmed that this therapeutic option against refractory BOS can be managed in a medium-size LTx center, with a satisfactory efficacy and an acceptable tolerance.
Published: 2021

17. Gain-of-Function Mutations in RPA1 Cause a Syndrome with Short Telomeres and Somatic Genetic Rescue

Author: Masayoshi Honda, Melchior Lauten, Marcus B. Valentine, Patrick Revy, Stéphane Coulon, Richa Sharma, Charnise Goodings, Caroline Kannengiesser, Fabian Beier, Melanie Boerries, Shondra M. Pruett-Miller, Sophie L Granger, Miriam Erlacher, Maria Spies, Axel Künstner, Megan A. Cooper, Jill A. Rosenfeld, Vincent Géli, Carole Saintomé, Victor B Pastor, Charlotte M. Niemeyer, Dmitri Churikov, Marcin W. Wlodarski, Sophia Polychronopoulou, Hauke Busch, Ti-Cheng Chang, Sandrine Hirschi, Louis Sanchez, Charikleia Kelaidi, Sushree S. Sahoo, Marc S. Wold, Alfonso G Fernandez, Sarah K. Nicholas, Indian Institute of Technology Delhi (IIT Delhi), Uppsala University, Freiburg Institute for Advanced Studies-LifeNet, Albert-Ludwigs-Universität Freiburg, St Jude Children's Research Hospital, University of Iowa [Iowa City], Sorbonne Université (SU), Universität zu Lübeck = University of Lübeck [Lübeck], Universitätsklinikum RWTH Aachen - University Hospital Aachen [Aachen, Germany] (UKA), Rheinisch-Westfälische Technische Hochschule Aachen University (RWTH), Centre de Recherche en Cancérologie de Marseille (CRCM), Aix Marseille Université (AMU)-Institut Paoli-Calmettes, Fédération nationale des Centres de lutte contre le Cancer (FNCLCC)-Fédération nationale des Centres de lutte contre le Cancer (FNCLCC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Institut Paoli-Calmettes, Fédération nationale des Centres de lutte contre le Cancer (FNCLCC), CHU Strasbourg, Université de Strasbourg (UNISTRA), German Cancer Consortium [Heidelberg] (DKTK), University Hospital Schleswig-Holstein-Campus Luebeck, 'Aghia Sophia' Children's Hospital, University of Freiburg [Freiburg], Washington University in Saint Louis (WUSTL), Baylor College of Medicine (BCM), Baylor University, AP-HP - Hôpital Bichat - Claude Bernard [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Muséum national d'Histoire naturelle (MNHN), German Cancer Research Center - Deutsches Krebsforschungszentrum [Heidelberg] (DKFZ), Imagine - Institut des maladies génétiques (IHU) (Imagine - U1163), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité), Structure et Instabilité des Génomes (STRING), Muséum national d'Histoire naturelle (MNHN)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut de Chimie du CNRS (INC)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS), University Medical Center of Schleswig–Holstein = Universitätsklinikum Schleswig-Holstein (UKSH), Kiel University, Molecular Genetics of Breast Cancer, German Cancer Research Center (DKFZ), Washington University School of Medicine in St. Louis, Physiopathologie et Epidémiologie des Maladies Respiratoires (PHERE (UMR_S_1152 / U1152)), Sorbonne Université - UFR Sciences de la vie (UFR 927 ), Universität zu Lübeck [Lübeck], RWTH Aachen University, Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut Paoli-Calmettes, Fédération nationale des Centres de lutte contre le Cancer (FNCLCC)-Fédération nationale des Centres de lutte contre le Cancer (FNCLCC)-Aix Marseille Université (AMU), and Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Paris (UP)
Subjects: Adult, Male, Heterozygote, Adolescent, Somatic cell, [SDV]Life Sciences [q-bio], Immunology, [SDV.BC.BC]Life Sciences [q-bio]/Cellular Biology/Subcellular Processes [q-bio.SC], Biology, medicine.disease_cause, Biochemistry, Germline, 03 medical and health sciences, Young Adult, 0302 clinical medicine, Replication Protein A, medicine, Missense mutation, Humans, Child, Gene, Telomere Shortening, 030304 developmental biology, Genetics, 0303 health sciences, Mutation, Infant, Newborn, [SDV.BBM.BM]Life Sciences [q-bio]/Biochemistry, Molecular Biology/Molecular biology, [SDV.MHEP.HEM]Life Sciences [q-bio]/Human health and pathology/Hematology, Cell Differentiation, Cell Biology, Hematology, DNA-binding domain, Bone Marrow Failure Disorders, Middle Aged, Telomere, 3. Good health, [SDV.GEN.GH]Life Sciences [q-bio]/Genetics/Human genetics, [SDV.IMM.IA]Life Sciences [q-bio]/Immunology/Adaptive immunology, Gain of Function Mutation, Myelodysplastic Syndromes, Female, Blood Commentary, Stem cell, 030215 immunology
Abstract: Human telomere biology disorders (TBD)/short telomere syndromes (STS) are heterogeneous disorders caused by inherited loss-of-function mutations in telomere-associated genes. Here, we identify 3 germline heterozygous missense variants in the RPA1 gene in 4 unrelated probands presenting with short telomeres and varying clinical features of TBD/STS, including bone marrow failure, myelodysplastic syndrome, T- and B-cell lymphopenia, pulmonary fibrosis, or skin manifestations. All variants cluster to DNA-binding domain A of RPA1 protein. RPA1 is a single-strand DNA-binding protein required for DNA replication and repair and involved in telomere maintenance. We showed that RPA1E240K and RPA1V227A proteins exhibit increased binding to single-strand and telomeric DNA, implying a gain in DNA-binding function, whereas RPA1T270A has binding properties similar to wild-type protein. To study the mutational effect in a cellular system, CRISPR/Cas9 was used to knock-in the RPA1E240K mutation into healthy inducible pluripotent stem cells. This resulted in severe telomere shortening and impaired hematopoietic differentiation. Furthermore, in patients with RPA1E240K, we discovered somatic genetic rescue in hematopoietic cells due to an acquired truncating cis RPA1 mutation or a uniparental isodisomy 17p with loss of mutant allele, coinciding with stabilized blood counts. Using single-cell sequencing, the 2 somatic genetic rescue events were proven to be independently acquired in hematopoietic stem cells. In summary, we describe the first human disease caused by germline RPA1 variants in individuals with TBD/STS.
Published: 2021
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18. 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

19. Idiopathic chronic obliterative bronchiolitis: a multicentric retrospective cohort

Author: Mouhamad Nasser, Yurdagül Uzunhan, Sandrine Hirschi, Stéphane Jouneau, François Lebargy, Aurélien Justet, Camille Taillé, Lidwine Wemeau, Victor Valentin, Raphael Borie, Benoit Godbert, Frédéric Gagnadoux, Laurent Guilleminault, Hilario Nunes, Sylvain Marchand-Adam, and Vincent Cottin
Subjects: Pediatrics, medicine.medical_specialty, business.industry, medicine, Retrospective cohort study, Chronic obliterative bronchiolitis, business
Published: 2021
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20. Comparison of extracorporeal photopheresis efficacy in treated versus non-treated patients with refractory BOS

Author: A. Schuller, Alain Bohbot, Romain Kessler, Anne Olland, Tristan Dégot, Justine Leroux, Sandrine Hirschi, Benjamin Renaud-Picard, and Arnaud Essaydi
Subjects: medicine.medical_specialty, Refractory, business.industry, Extracorporeal Photopheresis, Medicine, business, Surgery
Published: 2021
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21. Diffuse Pulmonary Non-Amyloid Light-Chain Deposition Disease: Phenotypes and Outcome

Author: Romain Lazor, Laurent Sohier, Vincent Cottin, Magali Colombat, Antoine Roux, Lidwine Wemeau, Anne Gondouin, François Lestelle, Salim Si-Mohamed, Pierre Rigaud, Yurdagul Uzunhan, Helene Morisse Pradier, David C. Rotzinger, Catherine Beigelman, Vincent Bunel, Grégoire Prévot, Martine Reynaud Gaubert, Mouham Nasser, Emmanuel Gomez, and Sandrine Hirschi
Subjects: Pathology, medicine.medical_specialty, Amyloid, business.industry, medicine, medicine.disease, business, Phenotype, Light chain deposition disease
Published: 2021
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22. 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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23. Late Breaking Abstract - Good outcome for lung transplantation for adults with interstitial lung disease associated with genetic disorders of surfactant metabolism

Author: Raphael Borie, Julien Bermudez, Sandrine Hirschi, Vincent Cottin, Julie Macey, Antoine Roux, Aurélie Leborgne-Krams, Vincent Bunel, Benjamin Coiffard, Tristan Dégot, Martine Reynaud-Gaubert, Jérôme Le Pavec, and Nadia Nathan
Subjects: medicine.medical_specialty, Pulmonary surfactant, business.industry, medicine.medical_treatment, Internal medicine, medicine, Interstitial lung disease, Lung transplantation, Metabolism, Good outcome, business, medicine.disease, Gastroenterology
Published: 2021
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24. Chronic lung allograft dysfunction is associated with an early increase of circulating cytotoxic CD4+CD57+ILT2+ T cells, selectively inhibited by the immune check-point HLA-G

Author: Olivier Brugière, Domitille Mouren, Julie Trichereau, Alexandre Vallée, Isabelle Kuzniak, Sandrine Hirschi, Benjamin Renaud-Picard, Martine Reynaud-Gaubert, Ana Nieves, Vincent Bunel, Jonathan Messika, Xavier Demant, Julie Macey, Jérôme Le Pavec, Gaëlle Dauriat, Christel Saint-Raymond, Loic Falque, Jean-François Mornex, Adrien Tissot, Aurore Foureau, Aurélie Le Borgne Krams, Véronique Bousseau, Antoine Magnan, Clément Picard, Antoine Roux, Edgardo Carosella, Joel LeMaoult, and Nathalie Rouas-Freiss
Subjects: Pulmonary and Respiratory Medicine, HLA-G Antigens, Transplantation, T-Lymphocytes, Humans, Surgery, Cardiology and Cardiovascular Medicine, Allografts, Lung, Lung Transplantation
Abstract: Survival after lung transplantation (LTx) still remains limited by chronic lung allograft dysfunction (CLAD), thought to represent a form of chronic rejection. We investigated whether the immune checkpoint HLA-G/ILT2 expressed by peripheral T-cell subpopulations could predict CLAD.We used data for 150 LTx recipients from COLT (Cohort-For-Lung-Transplantation) cohort with ≥1 available blood sample at 1-, 6-, or 12-months post-Tx. Analysis of T cells by flow cytometry focused on the ILT2 receptor of HLA-G and other markers (CD57, CD25, CD127). T-cell subset analyses compared stable patients and those with CLAD at 3 years post-LTx.With data for 78 stable and 72 CLAD patients, among 21 T-cell subsets expressing ILT2, only CD4+CD57+ILT2+ T cells were associated with outcome. At 1-month post-Tx, low proportion of CD4+CD57+ILT2+ T cells was associated with reduced 3-year incidence of CLAD (CD4+CD57+ILT2+ T cells ≤ first IQR [25%] vsfirst IQR, log-rank test, p = 0.028). Furthermore, the incidence of CLAD was higher with2.6- vs ≤2.6-fold increased proportion of CD4+CD57+ILT2+ T cells over the first year post-LTx (3-year freedom frequencies: 27% [95%CI: 8-50] vs 64% [95%CI: 48-77] (log-rank test, p = 0.014). On multivariable analysis, increased proportion of CD4+CD57+ILT2+ T cells over the first year predicted CLAD (hazard ratio 1.25; 95%CI: 1.09-1.44; p = 0.001). Focusing on CD4+CD57+ILT2+ T cells, we demonstrated ex vivo that they are cytotoxic CD4+ T cells, selectively inhibited by HLA-G.Our data suggest that an early increase of CD4+CD57+ILT2+ T cells after LTx may be associated with CLAD onset.
Published: 2021

25. Osseous sarcoidosis: A multicenter retrospective case-control study of 48 patients

Author: Catherine Chapelon-Abric, Marc Scherlinger, B. Meunier, Matthieu Groh, David Saadoun, Noémie Chanson, Nathalie Saidenberg-Kermanac’h, Karim Sacre, Camille Glanowski, Nicolas Schleinitz, Patrice Cacoub, Cloé Comarmond, Sandrine Hirschi, Imen Ben Hassine, Hervé Devilliers, Christophe Richez, Christopher Rein, Laurent Arnaud, and Philip Bielefeld
Subjects: Adult, Male, musculoskeletal diseases, medicine.medical_specialty, Sarcoidosis, Caseous necrosis, Risk Assessment, Severity of Illness Index, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, Humans, Medicine, Femur, 030212 general & internal medicine, Glucocorticoids, Lymph node, Pelvis, Retrospective Studies, 030203 arthritis & rheumatology, business.industry, Biopsy, Needle, Hydroxychloroquine, Middle Aged, Prognosis, musculoskeletal system, medicine.disease, Immunohistochemistry, Magnetic Resonance Imaging, 3. Good health, stomatognathic diseases, Methotrexate, Treatment Outcome, medicine.anatomical_structure, Case-Control Studies, Granuloma, Drug Therapy, Combination, Female, France, Lymph Nodes, Radiology, Bone Diseases, business, medicine.drug
Abstract: To describe the clinical presentation, distribution of lesions, treatment, and outcomes of osseous sarcoidosis.A French retrospective multicenter study of patients with biopsy-proven sarcoidosis analyzed patients with 1) a biopsy-proven granuloma without caseous necrosis, and either 2) osseous clinical manifestations, or 3) abnormal osseous imaging. Sarcoidosis patients with osseous involvement (cases) were compared with 264 age- and sex-matched sarcoidosis patients with no osseous manifestations (controls).In the osseous sarcoidosis group (n=88), forty-two (48%) patients had osseous-related symptoms involving the axial (69%) and/or appendicular (58%) skeleton. On imaging, the most commonly affected bones were in the spine (52%), pelvis (42%), hands (22%) and femur (19%). Compared with controls, cases had higher rates of mediastinal (93% vs. 47%) and extra-thoracic lymph node involvement (66% vs. 21%), pulmonary (90% vs. 65%) and cutaneous involvement (44% vs. 23%) (all P0.0001), and hypercalcemia (8.5% vs. 2%, P=0.014). Spleen/liver and gastrointestinal involvement were less frequent in the osseous sarcoidosis group (29% vs. 45%, and 1% vs. 17%, respectively, P0.0001). Response rates to with glucocorticoids alone, glucocorticoids plus methotrexate or glucocorticoids plus hydroxychloroquine were 23/44 (52%), 9/13 (69%) and 4/6 (67%), respectively.In patients with osseous sarcoidosis the spine and pelvis were the most commonly affected bones. Compared with controls, cases with osseous sarcoidosis have higher rates of thoracic and extra-thoracic lymph node involvement, pulmonary and cutaneous involvement, and hypercalcemia. Most patients with osseous sarcoidosis had a good response to glucocorticoids in combination with methotrexate or hydroxychloroquine.
Published: 2019
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26. Clinical characteristics of and outcomes for patients with COVID-19 and comorbid lung diseases primarily hospitalized in a conventional pulmonology unit: A retrospective study

Author: Carole Ederle, Sandrine Hirschi, Eva Chatron, Michele Porzio, Loic Kassegne, Benjamin Renaud-Picard, C. Marcot, Ronald C. Kessler, Tristan Dégot, Pierre Leyendecker, Aissam Labani, Carine Metz-Favre, F. De Blay, Matthieu Canuet, N. Khayath, Marianne Riou, CHU Strasbourg, Université de Strasbourg (UNISTRA), Fédération Hospitalo-Universitaire (OMICARE), Centre de Recherche d’Immunologie et d’Hématologie [Strasbourg], Fédération de Médecine Translationnelle de Strasbourg (FMTS), Nanomédecine Régénérative (NanoRegMed), Université de Strasbourg (UNISTRA)-Institut National de la Santé et de la Recherche Médicale (INSERM), and CCSD, Accord Elsevier
Subjects: Lung Diseases, Male, [SDV]Life Sciences [q-bio], Comorbidity, law.invention, pCO2, partial pressure of arterial carbon dioxide, Hospitals, University, 0302 clinical medicine, law, 030212 general & internal medicine, Hospital Mortality, COVID-19, coronavirus disease 2019, NIV, non invasive ventilation, COPD, Continuous Positive Airway Pressure, Smoking, Middle Aged, Intensive care unit, ICU, intensive care unit, 3. Good health, Anti-Bacterial Agents, [SDV] Life Sciences [q-bio], Hospitalization, Intensive Care Units, Pulmonology, medicine.anatomical_structure, Cohort, Hypertension, Female, Original Article, France, Hydroxychloroquine, Pulmonary and Respiratory Medicine, congenital, hereditary, and neonatal diseases and abnormalities, medicine.medical_specialty, pO2, partial pressure of oxygen, Antibodies, Monoclonal, Humanized, Antiviral Agents, SARS-CoV-2, severe acute respiratory syndrome coronavirus 2, RT-PCR, Real-time reverse transcriptase-polymerase chain reaction, 03 medical and health sciences, Sleep Apnea Syndromes, Internal medicine, medicine, Diabetes Mellitus, Humans, Obesity, Risk factor, IQR, interquartile range, Asthma, Aged, Retrospective Studies, Heart Failure, Lung, Noninvasive Ventilation, business.industry, SARS-CoV-2, Oxygen Inhalation Therapy, Anticoagulants, COVID-19, Retrospective cohort study, medicine.disease, ICS, inhaled corticosteroids, respiratory tract diseases, Coronavirus, 030228 respiratory system, COPD, chronic obstructive pulmonary disease, Chronic Disease, ACOS, asthma-COPD overlap syndrome, CPAP, continuous positive airway pressure, business
Abstract: International audience; Background: Scant data are currently available about a potential link between comorbid chronic lung diseases (CLD) and the risk and severity of the coronavirus disease 2019 (COVID-19) infection.Methods: To describe the clinical characteristics of and outcomes for patients with COVID-19 infection, including patients with comorbid respiratory diseases, who have been primarily hospitalized in the pulmonology department of Strasbourg University Hospital, France. In this retrospective, single-center study, we included all confirmed cases of COVID-19 from March 3 to April 15, 2020. We then compared the symptoms, biological and radiological findings, and outcomes for patients with and without CLD.Results: Of the 124 patients that were enrolled, the median age was 62 years, and 75 patients (60%) were male. Overall, 40% of patients (n=50) had preexisting CLD, including chronic obstructive pulmonary disease (COPD) (n=15, 12%) and asthma (n=19, 15%). Twenty-eight patients were transferred to the intensive care unit (ICU), and six patients died in our unit. CLD were not predictive of ICU hospitalization, but a significantly higher total mortality was observed (17.6% vs. 5.5%, P
Published: 2021
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27. Primary hepatic lymphoma after lung transplantation: a report of 2 cases

Author: Tristan Dégot, Emanuele Felli, Edoardo Maria Muttillo, Patrick Pessaux, Blandine Guffroy, Marianne Riou, Romain Kessler, Benjamin Renaud-Picard, Sandrine Hirschi, Matthieu Canuet, Pierre-Emmanuel Falcoz, Anne Olland, Nouvel Hôpital Civil de Strasbourg, L'Institut hospitalo-universitaire de Strasbourg (IHU Strasbourg), Institut National de Recherche en Informatique et en Automatique (Inria)-l'Institut de Recherche contre les Cancers de l'Appareil Digestif (IRCAD)-Les Hôpitaux Universitaires de Strasbourg (HUS)-La Fédération des Crédits Mutuels Centre Est (FCMCE)-L'Association pour la Recherche contre le Cancer (ARC)-La société Karl STORZ, l'Institut de Recherche contre les Cancers de l'Appareil Digestif (IRCAD), Università degli Studi di Roma 'La Sapienza' = Sapienza University [Rome] (UNIROMA), Université de Strasbourg (UNISTRA), and Institut de Cancérologie de Strasbourg Europe (ICANS)
Subjects: remission induction, Vincristine, medicine.medical_specialty, diffuse, medicine.medical_treatment, Population, lymphoma, male, immune system diseases, Prednisone, hemic and lymphatic diseases, medicine, lung transplantation, Lung transplantation, education, humans, immunocompromised host, Transplantation, education.field_of_study, Chemotherapy, Everolimus, liver neoplasms, business.industry, adult, medicine.disease, Tacrolimus, Lymphoma, aged, Surgery, Lymphoma, Large B-Cell, Diffuse, Radiology, large B-Cell, business, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology, medicine.drug
Abstract: International audience; Background. Diffuse large B-cell lymphoma (DLBCL) is the most common subtype of non -Hodgkin lymphoma in the posttransplant setting. Treatment is based on chemotherapy; surgery is still debated and should be performed in very select cases. Methods. We observed 2 patients out of 300 who underwent lung transplantation in the Nouvel Hopital Civil between 2013 and 2019 with primary hepatic lymphoma. Chemotherapy with a rituximab-cyclophosphamide, hydroxydaunorubicin, vincristine, prednisone protocol was performed in all patients. Mycophenolate mofetil was interrupted before treatment, and everolimus was introduced after chemotherapy by associating tacrolimus withdrawal. Results. One patient showed complete remission; after 7 years, no recurrence has been noticed. The second is still undergoing chemotherapy with no signs of disease progression. Conclusions. DLBCL risk is higher in solid organ transplant recipients than in the general population. Primary hepatic lymphoma diagnosis is often difficult and based on histologic findings after initial clinical and radiological suspicion of primary or secondary liver neoplasia. Diagnosis is challenging because no clinical, radiological, or biological features exist. Biopsy is always indicated for histologic confirmation. Chemotherapy is the mainstay of therapy, but surgery may be indicated in very select patients.
Published: 2021
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28. Low income and outcome in idiopathic pulmonary fibrosis: An association to uncover

Author: Lucile Sesé, Julien Caliez, Isabella Annesi-Maesano, Vincent Cottin, Giancarlo Pesce, Morgane Didier, Zohra Carton, Dominique Israel-Biet, Bruno Crestani, Stéphanie Guillot Dudoret, Jacques Cadranel, Benoit Wallaert, Abdellatif Tazi, Bernard Maître, Grégoire Prévot, Sylvain Marchand-Adam, Sandrine Hirschi, Sandra Dury, Violaine Giraud, Anne Gondouin, Philippe Bonniaud, Julie Traclet, Karine Juvin, Raphael Borie, Jean François Bernaudin, Dominique Valeyre, Catherine Cavalin, Hilario Nunes, Diane Bouvry, Pierre Yves Brillet, Philippe Camus, Juliette Chabrol, Jean François Cordier, Christophe Cracco, Philippe Delaval, Boris Duchemann, Sevrine Feuillet, Olivia Freynet, Frédéric Gagnadoux, Patrick Germaud, Louise Gindre, André Guetta, Patrick Haussman, Stephane Jouneau, Marianne Kambouchner, Chahera Khouatra, Jacques Lacronique, Anita Molard, Clément Picard, Carole Planes, Paul Andrés Rosental, Olivier Sanchez, Thomas Similowski, Luc Thiberville, Yurdagül Uzuhnan, 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), Institut Desbrest de santé publique (IDESP), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM), 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 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), Institut Pierre Louis d'Epidémiologie et de Santé Publique (iPLESP), Sorbonne Université (SU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Physiopathologie et Epidémiologie des Maladies Respiratoires (PHERE (UMR_S_1152 / U1152)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Paris (UP), 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), 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), CIC - CHU Bichat, Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Pneumologie A [Paris], AP-HP - Hôpital Bichat - Claude Bernard [Paris], Hôpital Albert Calmette, Université de Lille, Droit et Santé-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Service de Pneumologie et Immuno-Allergologie [CHU LIlle], Pole Cardio-vasculaire et pulmonaire [CHU Lille], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Immunologie humaine, physiopathologie & immunothérapie (HIPI (UMR_S_976 / U976)), Service de physiologie, explorations fonctionnelles [Mondor], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Henri Mondor-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), 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), Service de pneumologie [Toulouse], CHU Toulouse [Toulouse], CHU Trousseau [Tours], Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), Centre d’Etude des Pathologies Respiratoires (CEPR), UMR 1100 (CEPR), Université de Tours (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service des maladies respiratoires [CHU de Dijon], Service de Pneumologie Soins Intensifs, Appareillage Respiratoire [CHU de Dijon], 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), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12)-IFR10, CHU Toulouse [Toulouse]-Hôpital Larrey [Toulouse], Université de Tours-Institut National de la Santé et de la Recherche Médicale (INSERM), Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), and Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité)
Subjects: Pulmonary and Respiratory Medicine, Low income, medicine.medical_specialty, Prognostic factor, Vital Capacity, Air pollution, Idiopathic pulmonary fibrosis, Disease-Free Survival, [SHS]Humanities and Social Sciences, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, medicine, 030212 general & internal medicine, Prospective Studies, Prospective cohort study, Socioeconomic status, Biosimilar Pharmaceuticals, Poverty, Proportional Hazards Models, business.industry, 1. No poverty, Environmental Exposure, Occupational exposure, medicine.disease, Prognosis, Annual income, 030228 respiratory system, Income, Particulate Matter, France, business
Abstract: International audience; BackgroundLow income, a known prognostic indicator of various chronic respiratory diseases, has not been properly studied in idiopathic pulmonary fibrosis (IPF). We hypothesize that a low income has an adverse prognostic impact on IPF.MethodsPatients were selected from the French national prospective cohort COFI. Patients’ income was assessed through the median city-level income provided by the French National Institute of Statistics and Economic Studies according to their residential address. Patients were classified in two groups as “low income” vs. “higher income” depending on whether their annual income was estimated to be < or ≥18 170 €/year (the first quartile of the income distribution in the study population). The survival and progression-free survival (PFS) of the groups were compared by a log-rank test and a Cox model in multivariate analysis.Results200 patients were included. The average follow-up was 33.8 ± 22.7 months. Patients in the low income group were significantly more likely to be of non-European origin (p < 0.006), and to have at least one occupational exposure (p < 0.0001), and they tended to have a higher cumulative exposure to fine particles PM2.5 (p = 0.057). After adjusting for age, gender, forced vital capacity at inclusion, geographical origin, and occupational exposure having a low-income level was a factor associated with a worse PFS (HR: 1.81; CI95%: 1.24–2.62, p = 0.001) and overall survival (HR: 1.49; CI95%: 1.0006–2.23, p = 0.049).ConclusionsLow income appears to be a prognostic factor in IPF. IPF patients with low incomes may also be exposed more frequently to occupational exposures.
Published: 2021
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29. Airway complications in lung transplant recipients with telomere‐related interstitial lung disease

Author: Souheil El-Chemaly, Romain Lazor, Jérôme Le Pavec, Aurélie Le Borgne, Jean François Mornex, A. Roux, Raphael Borie, Antoine Froidure, Sandrine Hirschi, Hilary J. Goldberg, Andrew M. Courtwright, Martine Reynaud-Gaubert, Sébastien Quétant, Bina Choi, Jonathan Messika, Mathilde Phillips Houlbracq, Brigham & Women’s Hospital [Boston] (BWH), Harvard Medical School [Boston] (HMS), 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), Hospital of the University of Pennsylvania (HUP), Perelman School of Medicine, University of Pennsylvania-University of Pennsylvania, 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), Hospices Civils de Lyon (HCL), Centre de Référence des Maladies Pulmonaires Rares, Les Hôpitaux Universitaires de Strasbourg (HUS), Université de Versailles Saint-Quentin-en-Yvelines (UVSQ), Centre Chirurgical Marie Lannelongue (CCML), Service Hospitalier Universitaire Pneumologie-Physiologie [CHU Grenoble Alpes] (Pôle Thorax et Vaisseaux), Centre Hospitalier Universitaire [Grenoble] (CHU), Université Catholique de Louvain = Catholic University of Louvain (UCL), Lausanne University Hospital, Microbes évolution phylogénie et infections (MEPHI), Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), AP-HP - Hôpital Bichat - Claude Bernard [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), École pratique des hautes études (EPHE), ROSSI, Sabine, UCL - (SLuc) Service de pneumologie, and UCL - SSS/IREC/PNEU - Pôle de Pneumologie, ORL et Dermatologie
Subjects: medicine.medical_specialty, [SDV.MHEP.CHI] Life Sciences [q-bio]/Human health and pathology/Surgery, medicine.medical_treatment, Population, Constriction, Pathologic, [SDV.MHEP.CHI]Life Sciences [q-bio]/Human health and pathology/Surgery, telomerase, Internal medicine, medicine, lung transplantation, Humans, Lung transplantation, education, Lung, Retrospective Studies, Transplantation, education.field_of_study, telomere, pulmonary fibrosis, business.industry, Interstitial lung disease, Retrospective cohort study, Odds ratio, respiratory system, medicine.disease, Transplant Recipients, respiratory tract diseases, Stenosis, Cohort, Female, Lung Diseases, Interstitial, business
Abstract: International audience; Introduction Patients with short telomere-related interstitial lung disease (ILD) have worse outcomes after lung transplantation. We hypothesized that post-transplant airway complications, including dehiscence and bronchial stenosis, would be more common in the short telomere ILD lung transplant population. Methods We conducted a multi-institutional (Brigham and Women's Hospital, Groupe de Transplantation de la SPLF) retrospective cohort study of 63 recipients between 2009 and 2019 with ILD and short telomeres, compared to 4359 recipients from the Scientific Registry of Transplant Recipients with ILD and no known telomeropathy. Results In the short telomere cohort, six recipients (9.5%) developed dehiscence and nine recipients (14.3%) developed stenosis, compared to 60 (1.4%) and 149 (3.4%) in the control, respectively. After adjusting for age, sex, and bilaterality, the presence of short telomeres was associated with higher odds of dehiscence (odds ratio (OR) = 8.24, 95% confidence interval (CI) = 3.34 20.29, p < .001) and stenosis (OR = 4.63, 95% CI 2.21 9.69, p < .001). Conclusion The association between the presence of short telomeres and post-transplant dehiscence and stenosis suggest that airway complications may be a contributor to increased morbidity and mortality in patients with telomere-related ILD.
Published: 2021
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30. Risk Factors for Mortality after COVID-19 in Patients with Preexisting Interstitial Lung Disease

Author: Sylvain Marchand-Adam, Romain Lazor, Vincent Cottin, Yurdagul Uzunhan, Dominique Israel-Biet, Pierre Rigaud, Victor Valentin, Raphael Borie, Sandrine Hirschi, Laure Gallay, Université Claude Bernard Lyon 1 (UCBL), Université de Lyon, Université Sorbonne Paris Nord, AP-HP - Hôpital Bichat - Claude Bernard [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), University of Lausanne (UNIL), CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre Hospitalier Régional Universitaire de Tours (CHRU TOURS), Les Hôpitaux Universitaires de Strasbourg (HUS), 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 Régional de PharmacoVigilance Nord-Pas-de-Calais [CHU Lille] (CRPV), Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)-Université Lille 2 - Faculté de Médecine, and Centre Hospitalier Régional Universitaire de Tours (CHRU Tours)
Subjects: Adult, Male, Pulmonary and Respiratory Medicine, medicine.medical_specialty, 2019-20 coronavirus outbreak, Coronavirus disease 2019 (COVID-19), Critical Care and Intensive Care Medicine, [SDV.MHEP.PSR]Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract, 03 medical and health sciences, 0302 clinical medicine, Aged, Aged, 80 and over, COVID-19/complications, COVID-19/mortality, Case-Control Studies, Female, France/epidemiology, Humans, Lung Diseases, Interstitial/complications, Middle Aged, Proportional Hazards Models, Retrospective Studies, Risk Factors, Fibrosis, [SDV.MHEP.MI]Life Sciences [q-bio]/Human health and pathology/Infectious diseases, Internal medicine, Correspondence, medicine, In patient, 030212 general & internal medicine, ComputingMilieux_MISCELLANEOUS, [SDV.MHEP.ME]Life Sciences [q-bio]/Human health and pathology/Emerging diseases, Proportional hazards model, business.industry, Interstitial lung disease, Case-control study, COVID-19, Retrospective cohort study, medicine.disease, 3. Good health, 030228 respiratory system, [SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie, France, Lung Diseases, Interstitial, business
Abstract: International audience
Published: 2021
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31. Bilateral Acute Cardioembolic Limb Ischemia After Coronavirus Disease 2019 Pneumonia in a Lung Transplant Recipient: A Case Report

Author: Benjamin Kretz, Jocelyn Andre, Mickaël Ohana, Floriane Gallais, Floriane Zeyons, Romain Kessler, Laurent Sattler, Benjamin Renaud-Picard, Sandrine Hirschi, Les Hôpitaux Universitaires de Strasbourg (HUS), Department of Radiology, 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), Service de Neurologie [Hôpitaux Civils de Colmar], and Hôpitaux Civils Colmar
Subjects: Male, MRI, Magnetic resonance imaging, medicine.medical_treatment, [SDV]Life Sciences [q-bio], coronavirus, Embolectomy, 030230 surgery, 0302 clinical medicine, Ischemia, Myocardial infarction, IS, immunosuppressive, medicine.diagnostic_test, SOT, solid organ transplant, Thrombosis, 3. Good health, CT, computed tomography, Femoral Artery, medicine.anatomical_structure, Lower Extremity, Lupus Coagulation Inhibitor, acute limb ischemia, 030211 gastroenterology & hepatology, Radiology, Coronavirus Infections, Lung Transplantation, Adult, medicine.medical_specialty, LTx, Lung transplantation, Pneumonia, Viral, intracardiac thrombus, Article, 03 medical and health sciences, Betacoronavirus, medicine, Lung transplantation, Humans, Thrombus, Pandemics, Transplantation, Lung, business.industry, SARS-CoV-2, COVID-19, medicine.disease, Transplant Recipients, Pneumonia, SARS-CoV-2, Severe Acute Respiratory Syndrome Coronavirus 2, Angiography, Surgery, business
Abstract: Very few cases of lung transplant patients affected by coronavirus disease 2019 (COVID-19) have been reported to date. A 31-year-old patient who underwent bilateral lung transplantation for cystic fibrosis in 2012 was admitted for severe acute lower limb pain. He had a confirmed exposure to COVID-19 and a 3-week history of upper respiratory tract infection. Whole-body computed tomography (CT) angiography revealed an occlusion of the 2 common femoral arteries. CT angiography detected an intracardiac thrombus in the left ventricle. Chest CT angiography showed ground-glass opacities consistent with COVID-19. A bilateral femoral surgical embolectomy using Fogarty catheter was successfully performed. Specific reverse transcription polymerase chain reaction for severe acute respiratory syndrome coronavirus 2 performed on an extracted thrombus was negative, but IgM antibodies specific for COVID-19 were detected. Cardiac magnetic resonance imaging demonstrated a subendocardial and almost transmural late gadolinium enhancement in the mid and distal inferolateral and inferior wall segments, consistent with a nonrecent myocardial infarction and an apical centimetric thrombus adjacent to the lesion. Thrombophilia laboratory tests found the presence of a positive lupus anticoagulant. Treatment with low-molecular-weight heparin and aspirin was prescribed. On day 13, the patient was discharged from the hospital. This case underlines the need to be vigilant with respect to the thrombotic complications of COVID-19 and raises the issue of thrombosis prevention in COVID-19 patients.
Published: 2020
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32. COVID-19 in Lung Transplant Recipients

Author: Nicolas Carlier, Adrien Tissot, Martine Reynaud-Gaubert, Benjamin Renaud-Picard, Xavier Demant, Sacha Mussot, Ana Nieves, Christel Saint Raymond, Sandrine Hirschi, Philippine Eloy, Aurélie Le Borgne, Jonathan Messika, Marie-Pierre Debray, Véronique Boussaud, Agathe Sénéchal, Jean-François Mornex, Loïc Falque, Jérôme Le Pavec, L. Beaumont, Hervé Mal, Jacques Jougon, Antoine Roux, AP-HP - Hôpital Bichat - Claude Bernard [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), INSERM UMR1152, Service de Pneumologie et Transplantation Pulmomaire, Hôpital Bichat - Claude Bernard, Assistance Publique - Hôpitaux de Paris (AP-HP), Département d’Epidémiologie et Recherche Clinique [AP-HP Hôpital Bichat - Claude Bernard] (CIC‑EC 1425), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7), Université de Versailles Saint-Quentin-en-Yvelines - UFR Sciences de la santé Simone Veil (UVSQ Santé), Université de Versailles Saint-Quentin-en-Yvelines (UVSQ), Les Hôpitaux Universitaires de Strasbourg (HUS), Aix Marseille Université (AMU), Université Paris-Saclay, School of Medicine, Le Kremlin-Bicêtre, France, «Pulmonary Hypertension: Pathophysiology and Novel Therapies», Hôpital Marie Lannelongue, Le Plessis-Robinson, hôpital Louis-Pradel, CHU de Lyon, 69500 Bron, France., Centre Hospitalier Universitaire [Grenoble] (CHU), AP-HP - Hôpital Cochin Broca Hôtel Dieu [Paris], Hôpital Haut-Lévêque [CHU Bordeaux], CHU Bordeaux [Bordeaux], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Centre hospitalier universitaire de Nantes (CHU Nantes), Hôpital Foch [Suresnes], Microbes évolution phylogénie et infections (MEPHI), Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS), 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 Hospitalier Universitaire Grenoble Alpes (CHU Grenoble Alpes), CHU Toulouse [Toulouse], École pratique des hautes études (EPHE), and HAL UVSQ, Équipe
Subjects: Adult, Male, medicine.medical_specialty, [SDV]Life Sciences [q-bio], medicine.medical_treatment, 030230 surgery, law.invention, 03 medical and health sciences, 0302 clinical medicine, law, Internal medicine, Humans, Medicine, Lung transplantation, Letters to the Editor, Survival rate, Retrospective Studies, Univariate analysis, Transplantation, SARS-CoV-2, business.industry, COVID-19, Retrospective cohort study, Middle Aged, medicine.disease, Intensive care unit, Transplant Recipients, 3. Good health, [SDV] Life Sciences [q-bio], Intensive Care Units, Pneumonia, Cohort, ComputingMethodologies_DOCUMENTANDTEXTPROCESSING, Female, 030211 gastroenterology & hepatology, business, Immunosuppressive Agents, Lung Transplantation, Cohort study
Abstract: International audience; BACKGROUND: A concern about the susceptibility of immunocompromised patients to the worldwide pandemic of coronavirus disease 2019 (COVID-19) has been raised. We aimed at describing COVID-19 infections in the French cohort of lung transplant (LT) patients. METHODS: Multicenter nationwide cohort study of all LT recipients with COVID-19 diagnosed from March 1 to May 19, 2020. Recipient main characteristics and their management were retrieved. Hospitalization characteristics, occurrence of complications and survival were analyzed. RESULTS: Thirty-five LT patients with a COVID-19 infection were included. Median age was 50.4 (40.6-62.9) years, 16 (45.7%) were female, and 80% were double-LT recipients. Infection was community-acquired in 25 (71.4%). Thirty-one (88.6%) required hospitalization, including 13 (41.9%) in the intensive care unit. The main symptoms of COVID-19 were fever, cough, and diarrhea, present in 71.4%, 54.3%, and 31.4% of cases, respectively. Extension of pneumonia on chest CT was moderate to severe in 51.4% of cases. Among the 13 critically ill patients, 7 (53.9%) received invasive mechanical ventilation. Thrombotic events occurred in 4 patients. Overall survival rate was 85.7% after a median follow-up of 50 days (41.0-56.5). Four of 5 nonsurvivors had had bronchial complications or intensification of immunosuppression in the previous weeks. On univariate analysis, overweight was significantly associated with risk of death (odds ratio, 16.0; 95% confidence interval, 1.5-170.6; P = 0.02). CONCLUSIONS: For the 35 LT recipients with COVID-19, the presentation was severe, requiring hospitalization in most cases, with a survival rate of 85.7%. Copyright
Published: 2020
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33. The social and clinical impact of the COVID‐19 epidemic on the Strasbourg lung transplant cohort: A single‐center retrospective cohort study

Author: Floriane Gallais, A. Schuller, Sophie Freudenberger, Tristan Dégot, Eva Chatron, Sandrine Hirschi, Michele Porzio, Marianne Riou, Romain Kessler, Julien Stauder, and Benjamin Renaud-Picard
Subjects: Adult, Male, medicine.medical_specialty, Coronavirus disease 2019 (COVID-19), Adolescent, Social Determinants of Health, Health Behavior, Physical Distancing, Single Center, Risk Assessment, Hospitals, University, Young Adult, Postoperative Complications, Risk Factors, Internal medicine, Diabetes mellitus, Medicine, Humans, Epidemics, Life Style, Aged, Retrospective Studies, Aged, 80 and over, Transplantation, Lung, business.industry, Social distance, COVID-19, Retrospective cohort study, Original Articles, Middle Aged, medicine.disease, Obesity, Transplant Recipients, medicine.anatomical_structure, Cohort, Patient Compliance, Original Article, Female, France, Contact Tracing, business, Lung Transplantation
Abstract: The clinical and social impacts of the COVID‐19 epidemic on lung transplant (LTx) recipients remain poorly known. We aimed to evaluate its social, clinical, and behavioral consequences on the LTx patients followed in Strasbourg university hospital. A questionnaire was used to collect details concerning patients’ lifestyles, their protection methods used to avoid COVID‐19 contamination, and clinical infection‐related information for March 2020. A specific score was created to quantify patients’ contacts and the associated risk of infectious contagion. Data were collected from 322 patients (91.2%). A majority reported a higher application than usual of social distancing and barrier measures. 43.8% described infectious‐related symptoms and 15.8% needed an anti‐infective treatment. There was no difference in symptom onset according to age, native lung disease, diabetes, or obesity. Nineteen patients were tested for COVID‐19, and four were diagnosed positive, all with a favorable outcome. The infection risk contact score was higher for symptomatic patients (p: 0.007), those needing extra‐medical appointments (p
Published: 2020

34. 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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35. Gender differences in idiopathic pulmonary fibrosis: are men and women equal or not?

Author: Violaine Giraud, Philippe Bonniaud, Abdellatif Tazi, Julie Traclet, Karine Juvin, Sandrine Hirschi, Bruno Crestani, Carole Planès, Vincent Cottin, Dominique Valeyre, S. Guillot Dudoret, Lucile Sesé, Hilario Nunes, Sandra Dury, Raphael Borie, G. Prévot, Benoit Wallaert, Bernard Maitre, Sylvain Marchand-Adam, Yurdagul Uzunhan, Jacques Cadranel, Thomas Gille, Olivia Freynet, Dominique Israel-Biet, Zohra Carton, Anne Gondouin, and Julien Caliez
Subjects: Pulmonary and Respiratory Medicine, education.field_of_study, medicine.medical_specialty, Proportional hazards model, business.industry, medicine.medical_treatment, Population, medicine.disease, respiratory tract diseases, Log-rank test, FEV1/FVC ratio, Idiopathic pulmonary fibrosis, Internal medicine, Cohort, medicine, Lung transplantation, Prospective cohort study, education, business
Abstract: Background Few studies analyzed gender-related outcomes and characteristics differences of patients with idiopathic pulmonary fibrosis (IPF). The aim of the study was to explore gender differences in a French IPF cohort. Methods 236 patients with incident IPF were included in the multicentric longitudinal prospective cohort COhorte FIbrose (COFI), and followed for 5 years. Men and women were compared for characteristics atinclusion (t-test, Chi2 or ANOVA) and survival (Log rank, Cox model). Results The population consisted of 51 (22%) women and 185(78%) men. At inclusion women significantly differed from men for an older age (> 65 years, 78 vs. 61%, P = 0.028), a lower proportion of smokers (P Conclusions At diagnosis women appear to have a distinct imaging pattern and a better FVC, which may be due to less exposure history compared to men. However, outcomes remain comparable in both sexes. Less access to lung transplantation in women may be due to age and comorbidities.
Published: 2020
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36. Low income and progression free survival in idiopathic pulmonary fibrosis: an association to uncover

Author: Julie Traclet, Dominique Valeyre, Sandra Dury, Benoit Wallaert, Abdellatif Tazi, Hilario Nunes, Julien Caliez, Morgane Didier, Catherine Cavalin, Jean-François Bernaudin, Vincent Cottin, Bernard Maitre, Karine Juvin, Violaine Giraud, Dominique Israel-Biet, Bruno Crestani, Zohra Carton, Anne Gondouin, Lucile Sesé, Raphael Borie, Philippe Bonniaud, S. Guillot Dudoret, G. Prévot, Sylvain Marchand-Adam, Sandrine Hirschi, Giancarlo Pesce, Isabella Annesi-Maesano, and Jacques Cadranel
Subjects: Pulmonary and Respiratory Medicine, Low income, Idiopathic pulmonary fibrosis, Prognostic factor, medicine.medical_specialty, business.industry, Internal medicine, medicine, Cumulative Exposure, Progression-free survival, medicine.disease, business, Prospective cohort study
Abstract: Background Low income is a known prognostic indicator of various chronic respiratory diseases but has not been studied in idiopathic pulmonary fibrosis (IPF). We hypothesize that a low income has an adverse prognostic impact on IPF. Methods Patients were selected from the French national prospective cohort COFI. Patient's income was assessed through the median city-level income provided by the French National Institute of Statistics and Economic Studies according to his/her residential address. Patients were classified in two groups as “low income” vs. “higher income” depending on whether their annual city-level income was estimated to be Results 200 patients were included. The average follow-up was 33.8 ± 22.7 months. Patients in the low income group were more likely to be of non-European origin (42 vs. 17%, P Conclusions Low income appears to be a prognostic factor of progression free survival in IPF. And patients with low incomes may also be exposed more frequently to occupational exposures and to higher cumulative exposure to fine particles.
Published: 2020
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37. Lung transplantation for sarcoidosis: outcome and prognostic factors

Author: Jens Gottlieb, Jean-François Mornex, Jean-François Bernaudin, Dominique Valeyre, Hans Henrik Schultz, Martine Reynaud-Gaubert, Sergio Harari, Laurent Savale, Michael Perch, Jesper Magnusson, Are Martin Holm, P. Gazengel, Christiane Knoop, Vincent Bunel, Clément Picard, Sandrine Hirschi, Pierre-Yves Brillet, Jérôme Le Pavec, Elodie Blanchard, Marc Humbert, Elie Fadel, Laurent Godinas, Aurélie Le Borgne, Hilario Nunes, Adrien Tissot, and Rosalía Laporta
Subjects: Pulmonary and Respiratory Medicine, Male, medicine.medical_specialty, Sarcoidosis, medicine.medical_treatment, Gastroenterology, Sarcoidosis, Pulmonary, Fibrosis, Internal medicine, Pulmonary fibrosis, Medicine, Lung transplantation, Humans, Aged, Retrospective Studies, Lung, business.industry, Retrospective cohort study, medicine.disease, Prognosis, Pulmonary hypertension, Transplantation, medicine.anatomical_structure, business, Lung Transplantation
Abstract: Study questionIn patients with sarcoidosis, past and ongoing immunosuppressive regimens, recurrent disease in the transplant and extrapulmonary involvement may affect outcomes of lung transplantation. We asked whether sarcoidosis lung phenotypes can be differentiated and, if so, how they relate to outcomes in patients with pulmonary sarcoidosis treated by lung transplantation.Patients and methodsWe retrospectively reviewed data from 112 patients who met international diagnostic criteria for sarcoidosis and underwent lung or heart–lung transplantation between 2006 and 2019 at 16 European centres.ResultsPatient survival was the main outcome measure. At transplantation, median (interaquartile range (IQR)) age was 52 (46–59) years; 71 (64%) were male. Lung phenotypes were individualised as follows: 1) extended fibrosis only; 2) airflow obstruction; 3) severe pulmonary hypertension (sPH) and airflow obstruction; 4) sPH, airflow obstruction and fibrosis; 5) sPH and fibrosis; 6) airflow obstruction and fibrosis; 7) sPH; and 8) none of these criteria, in 17%, 16%, 17%, 14%, 11%, 9%, 5% and 11% of patients, respectively. Post-transplant survival rates after 1, 3, and 5 years were 86%, 76% and 69%, respectively. During follow-up (median (IQR) 46 (16–89) months), 31% of patients developed chronic lung allograft dysfunction. Age and extended lung fibrosis were associated with increased mortality. Pulmonary fibrosis predominating peripherally was associated with short-term complications.Answer to the study questionPost-transplant survival in patients with pulmonary sarcoidosis was similar to that in patients with other indications for lung transplantation. The main factors associated with worse survival were older age and extensive pre-operative lung fibrosis.
Published: 2020
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38. Demographics and Baseline Characteristics of Patients with Idiopathic Pulmonary Fibrosis (IPF) in a Real-World Setting: Results of 847 Patients Enrolled in the Radico-ILD Cohort in France

Author: Hilario Nunes, A. Clément, Sylvain Marchand-Adam, J.-M. Naccache, Dominique Israel-Biet, S. Jouneau, I. Dufaure-Garé, Martine Reynaud-Gaubert, RaDiCo-ILD, P. Bonniaud, Sandrine Hirschi, Sébastien Quétant, Lidwine Wemeau, M. Chevereau, Bruno Crestani, David Montani, Vincent Cottin, and J.-C. Dalphin
Subjects: medicine.medical_specialty, Idiopathic pulmonary fibrosis, Demographics, business.industry, Baseline characteristics, Internal medicine, Cohort, medicine, medicine.disease, business
Published: 2020
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39. Epithelial-mesenchymal transition and membrane microparticles: potential implications for bronchiolitis obliterans syndrome after lung transplantation

Author: Benjamin Renaud-Picard, Mohamad Kassem, Romain Kessler, Sandrine Hirschi, Michele Porzio, Guillaume Kreutter, Kevin Vallière, Marie-Pierrette Chenard, Fatiha El-Ghazouani, Justine Toussaint, Florence Toti, Laurence Kessler, Ali El-Habhab, Anne Olland, Nanomédecine Régénérative (NanoRegMed), Université de Strasbourg (UNISTRA)-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Strasbourg, Fédération de Médecine Translationnelle de Strasbourg (FMTS), Université de Strasbourg (UNISTRA), and univOAK, Archive ouverte
Subjects: Adult, Male, congenital, hereditary, and neonatal diseases and abnormalities, Pathology, medicine.medical_specialty, Epithelial-Mesenchymal Transition, [SDV.MHEP.CHI] Life Sciences [q-bio]/Human health and pathology/Surgery, medicine.medical_treatment, Immunology, Down-Regulation, Bronchiolitis obliterans, Vimentin, Respiratory Mucosa, [SDV.MHEP.CHI]Life Sciences [q-bio]/Human health and pathology/Surgery, Microparticles, [SDV.MHEP.PSR]Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract, Monocytes, Postoperative Complications, Immunophenotyping, Cell-Derived Microparticles, Humans, Immunology and Allergy, Medicine, Lung transplantation, Epithelial–mesenchymal transition, Respiratory system, Lung, Transplantation, biology, business.industry, Mesenchymal stem cell, nutritional and metabolic diseases, Biomarker, Middle Aged, Cadherins, medicine.disease, [SDV.IMM.IA]Life Sciences [q-bio]/Immunology/Adaptive immunology, [SDV.IMM.IA] Life Sciences [q-bio]/Immunology/Adaptive immunology, biology.protein, Immunohistochemistry, [SDV.MHEP.PSR] Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract, Female, business
Abstract: International audience; Long term survival post lung transplantation (LTx) is limited by the occurrence of bronchiolitis obliterans syndrome (BOS). One mechanism involved is the epithelial-mesenchymal transition (EMT). Membrane microparticles (MPs) are known to be involved in some respiratory diseases and in other organs allograft rejection episodes. We hypothesized that leukocyte-derived MPs likely contribute to EMT. To emphasize this physiological concept, our objectives were to: (1) confirm the presence of EMT on explanted lungs from patients who underwent a second LTx for BOS; 2) characterize circulating MPs in transplanted patients, with or without BOS; (3) evaluate in vitro the effect of monocyte-derived MPs in EMT of human bronchial epithelial cells. Our IHC analysis on explanted graft lungs revealed significant pathological signs of EMT with an inhomogeneous destruction of the bronchial epithelium, with decreased expression of the epithelial protein E-cadherin and increased expression of the mesenchymal protein Vimentin. The immunophenotyping of MPs demonstrated that the concentration of MPs carrying E-cadherin was lower in patients affected by BOS (p = .007). In vitro, monocyte-derived MPs produced with LPS were associated with decreased E-cadherin expression (p < .05) along with significant morphological and functional cell modifications. MPs may play a role in EMT onset in bronchial epithelium following LTx.
Published: 2020
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40. Neurol-Neuroimmunol

Author: Cornelia Kuhnert, Vincent Poindron, Morgane Solis, Thierry Martin, Sandrine Hirschi, Aurélien Guffroy, Laurent Kremer, Anne Molitor, Raphael Carapito, Yves Hansmann, Vincent Gies, Anne-Sophie Korganow, Yannick Dieudonné, and Cédric Lenormand
Subjects: Autoimmune disease, 0303 health sciences, Systemic lupus erythematosus, business.industry, medicine.medical_treatment, Progressive multifocal leukoencephalopathy, Interleukin, Immunosuppression, Sciences du Vivant [q-bio]/Médecine humaine et pathologie, medicine.disease, 3. Good health, 03 medical and health sciences, 0302 clinical medicine, Neurology, Immune reconstitution inflammatory syndrome, Immunology, medicine, Primary immunodeficiency, Neurology (clinical), Sarcoidosis, business, 030217 neurology & neurosurgery, 030304 developmental biology
Abstract: ObjectiveTo report the association of JC virus infection of the brain (progressive multifocal encephalopathy [PML]) during the course of sarcoidosis and the challenging balance between immune reconstitution under targeted cytokine interleukin 7 (IL7) therapy for PML and immunosuppression for sarcoidosis.MethodsOriginal case report including deep sequencing (whole-exome sequencing) to exclude a primary immunodeficiency (PID) and review of the literature of cases of PML and sarcoidosis.ResultsWe report and discuss here a challenging case of immune reconstitution with IL7 therapy for PML in sarcoidosis in a patient without evidence for underling PID or previous immunosuppressive therapy.ConclusionsNew targeted therapies in immunology and infectiology open the doors of more specific and more specialized therapies for patients with immunodeficiencies, autoimmune diseases, or cancers. However, before instauration of these treatments, the risk of immune reconstitution inflammatory syndrome and potential exacerbation of an underlying disease must be considered. It is particularly true in case of autoimmune disease such as sarcoidosis or lupus.
Published: 2020

41. Discordance entre SaO 2 – PaO 2 : ne pas oublier les hémoglobinopathies

Author: M. Oswald, Tristan Dégot, E. Virot, Ronald C. Kessler, A.-C. Galoisy, Serge Pissard, L. Kiger, Sandrine Hirschi, and Matthieu Canuet
Subjects: 0301 basic medicine, Pulmonary and Respiratory Medicine, Gynecology, 03 medical and health sciences, medicine.medical_specialty, 030104 developmental biology, 0302 clinical medicine, Hemoglobinopathy, 030228 respiratory system, Chemistry, medicine, medicine.disease
Abstract: Resume Introduction Plusieurs parametres clinico-biologiques permettent d’estimer la quantite d’oxygene disponible pour l’organisme. La saturation en oxygene mesuree a l’oxymetre de pouls (SpO2), la saturation arterielle en oxygene (SaO2) et la pression partielle arterielle en oxygene (PaO2) mesurees par gazometrie sont les plus utilisees. Observation Nous rapportons le cas d’une patiente hospitalisee pour bilan d’insuffisance respiratoire decouverte dans un contexte de dyspnee d’effort chronique. La SpO2 etait diminuee tout comme la SaO2 mesuree par gazometrie malgre une PaO2 dans les normes. La dissociation entre les valeurs de PaO2 et de SaO2 nous a permis d’aboutir au diagnostic d’hemoglobinopathie (hemoglobine Bassett). Conclusion Le diagnostic d’hemoglobinopathie est a evoquer devant une desaturation en oxygene avec bilan respiratoire et cardiologique sans anomalie. Il ne semble pas y avoir d’argument pour proposer a ces patients une oxygenotherapie en condition stable.
Published: 2018
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42. Association between influenza vaccination and the occurrence of acute exacerbation inidiopathic pulmonary fibrosis

Author: Bernard Maitre, Zohra Carton, Dominique Israel-Biet, Anne Gondouin, Bruno Crestani, Karine Juvin, Sandra Dury, Vincent Cottin, Violaine Giraud, Abdellatif Tazi, Thomas Gille, Stephanie Guillot-Dudoret, Sylvain Marchan-Adam, Hilario Nunes, Dominique Valeyre, Jacques Cadranel, Julien Caliez, Sandrine Hirschi, Raphael Borie, Philippe Bonniaud, Lucile Sesé, Grégoire Prévot, Julie Traclet, and Benoit Wallaert
Subjects: Pulmonary and Respiratory Medicine, education.field_of_study, medicine.medical_specialty, Exacerbation, business.industry, Proportional hazards model, Population, medicine.disease, Vaccination, Idiopathic pulmonary fibrosis, Statistical significance, Internal medicine, Cohort, Medicine, Prospective cohort study, business, education
Abstract: Background International guidelines recommend that annual influenza vaccination be performed in patients with idiopathic pulmonary fibrosis (IPF). Viral infections are recognized to be triggers of acute exacerbation (AE). The purpose of this study was to investigate the role of annual influenza vaccination on AE occurrence. Methods 236 patients with incident IPF were included in the multicentric longitudinal prospective cohort COhorte FIbrose (COFI), and followed for 5 years. Influenza vaccination status was requested annually. We have defined aone-year vaccination coverage from September the first to August the 30th of the following year. 638 annual observations were available with an informed vaccination status. To evaluate the association between annual influenza vaccination and AE occurrence, two mixed-effect Cox regression was used. For the second model we divided patients in 3 groups: – patients vaccinated every year; – patients vaccinated at least half of the years of follow-up; – patients vaccinated less than half the years of follow-up. Only the first AE was considered. The definition of AE was that of Akira et al. Results Mean follow-up was 33.2 ± 23.6 months. 36 AEs occurred in 33 patients. Of the 33 patients, only 27 had an informed vaccination status in the year of their AE. Nine (33%) patients who experienced an AE weren’t vaccinated. For 141 (22%) annual observations, the patient didn’t receive the vaccine. In this model, influenza vaccination was associated with a decreased occurrence of AE but this did not reach the level of significance in this small population (HR:0.58 CI95%0.23-1.46, P = 0.25). In the first group patients were vaccinated every year and consisted of hundred and thirty-three individuals. The second group was composed of patients vaccinated at least half of the years of follow-up, with 35 patients. And 36 patients were vaccinated less than half the years of follow-up in the last group. In the second model, patients vaccinated less than half the years of follow-up were significantly more at risk to present AE compared to patients who were vaccinated every year (HR:2.77 CI95%1.11-6.93, P = 0.03). Conclusions In our IPF cohort, only about half of the patients had influenza vaccination coverage throughout the follow-up period. Annual influenza vaccination seems to reduce the risk of AE but due to a lack of power this association was not significant. However patients vaccinated less than half the years of follow-up were significantly more at risk to present AE compared to patients who were vaccinated every year. A study with a larger number of patients is highly desirable to confirm this association.
Published: 2021
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43. Infinitix-BOS Trial: Multi-Center, Randomised, Double-Blind Placebo-Controlled Trial of Nintedanib in Lung Transplant Recipients with Bronchiolitis Obliterans Syndrome (BOS) Grade 0-p and Grade 1-2

Author: C. Macey, G. Dauriat, C. Saint Raymond, Adrian Crutu, a. Briault, O. Brugière, Benjamin Coiffard, L. Beaumont, Anne-Françoise Roux, G. Weisenburger, J. Le Pavec, Martine Reynaud-Gaubert, A. Hamid, b. Renaud Picard, Christophe Pison, Clément Picard, c. Bon, Sandrine Hirschi, Tristan Dégot, Xavier Demant, Vincent Bunel, Jonathan Messika, and B. Coltey
Subjects: Pulmonary and Respiratory Medicine, Transplantation, medicine.medical_specialty, Lung, business.industry, medicine.medical_treatment, Placebo-controlled study, Bronchiolitis obliterans, medicine.disease, Placebo, humanities, law.invention, chemistry.chemical_compound, medicine.anatomical_structure, chemistry, Randomized controlled trial, Bronchiolitis, law, Internal medicine, medicine, Lung transplantation, Surgery, Nintedanib, Cardiology and Cardiovascular Medicine, business
Abstract: Purpose Lung transplantation (TxP) is now a validated treatment of end-stage pulmonary diseases, but long-term survival are still hampered by the development of chronic allograft dysfunction (CLAD) affecting> 50% of patients. Bronchiolitis obliterans syndrome/obliterative bronchiolitis (BOS/OB) is the most common manifestation of CLAD. Survival after onset of BOS is poor ( Methods A phase III clinical randomized trial to assess the efficacy of Nintedanib in LTx recipients with BOS.Inclusion criteria: n=80 LTx recipients with BOS 0p-1-2, with a decline of FEV1> 200ml within the previous year.Primary Objective: to assess Nintedanib efficacy in the reduction of the rate of decline of FEV1 (forced expiratory volume in 1 sec) at a dose of 150 mg twice daily (bid) compared to placebo over 6 months. Secondary Objectives: to assess Nintedanib efficacy and tolerance in the treatment of BOS 0p-1-2, based on the change over 6 months of : 6WT, QOL, BOS grade/graft failure, O2 saturation, KL-6, SPD, VEGF, PDGF parameters, and tolerance. Results The trial was started in December 2019 among 8 french LTx centers (Hopital Foch, Bichat, HEGP/Cochin, Marie-Lannelongue, Marseille, Bordeaux, Strasbourg, and Grenoble), for a total duration of inclusion anticipated at 36 months. On October 15th 2020, 14 patients were already included. Conclusion Infinitix-BOS is the first therapeutic randomized trial testing the efficacy of nintedanib in LTx recipients with BOS. In case of demonstrated effectiveness of Nintedanib, the benefit for LTx patients with BOS seems high in terms of stabilization of lung function and enhancement of survival.
Published: 2021
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44. Essai multicentrique, randomisé double aveugle contre placebo, évaluant l’efficacité du Nintedanib dans le traitement du syndrome de bronchiolite oblitérante grade 0-p et grade 1-2 chez les patients transplantés pulmonaires

Author: L. Beaumont, C. Macey, Xavier Demant, O. Brugière, Y. Maurer, G. Dauriat, C. Couffignal, C. Saint Raymond, Vincent Bunel, R. Antoine, Martine Reynaud-Gaubert, Adrian Crutu, Benjamin Renaud-Picard, B. Coltey, Jonathan Messika, Christophe Pison, G. Weisenburger, A. Briot, Tristan Dégot, Sandrine Hirschi, c. Bon, J. Le Pavec, S. Makhlouf, and Clément Picard
Subjects: Pulmonary and Respiratory Medicine
Abstract: Introduction La survie apres transplantation pulmonaire (TxP) reste limitee par la survenue d’une dysfunction chronique pulmonaire (CLAD), qui touche > 50 % des greffes. La forme obstructive de CLAD, bronchiolite obliterante (BO)/syndrome de BO (SBO), est le phenotype le plus frequent de CLAD (80% des cas). Les lesions de BO semblent dues a des agressions repetees de l’epithelium bronchique avec developpement d’une cicatrice fibreuse. Il n’existe actuellement pas de traitement demontre pour stabiliser le SBO. Le role crucial de la dysfonction de reparation fibrotique est demontre dans la BO via le remodellage bronchique, l’augmentation de matrice extra-cellulaire, la transition epithelio-mesenchymateuse, l’augmentation des facteurs de croissance PDGF, VEGF, FGF, IGF- Le TKI Nintedanib, demontre efficace dans la fibrose pulmonaire idiopathique, et qui cible ces facteurs de croissance, est une molecule candidate. Un essai therapeutique testant le nintedanib dans la BOS post-TxP a debute en France fin 2019. Methodes Essai multicentrique, randomise double aveugle contre placebo, evaluant l’efficacite du Nintedanib dans le traitement du syndrome de bronchiolite obliterante grade 0-p et grade 1-2 chez les patients greffes pulmonaires. Criteres d’inclusion: BOS 0-p, 1, 2 et forme mixte de CLAD (criteres ISHLT). Objectif principal: evaluer l’efficacite du nintedanib sur la reduction du taux de declin du VEMS dans le SBO post-TxP (150 mg × 2/j versus placebo pendant 6 mois (± 100 mg × 2/j si effets secondaires). Objectifs secondaires: evaluer l’efficacite du nintedanib sur l’evolution de: 6WT, QOL (SGRQ); mesures repetees VEMS; grade BOS; Saturation O2; ainsi que frequence des effets secondaires, et evolution de parametres sanguins KL-6, SPD, VEGF, PDGF. Resultats L’essai a ete ouvert entre dec 2019 et mars 2020 parmi 8 centres de greffe en France (Hopital Foch, Bichat, HEGP/Cochin, Marie-Lannelongue, Marseille, Bordeaux, Strasbourg et Grenoble), pour une duree d’inclusion prevue de 36 mois. Au 31/08/2020, 7 patients greffes ont ete inclus dans 3 centres. Afin d’augmenter le nombre de patients eligibles, une modification du protocole du 2/07/2020, permet d’inclure desormais les patients avec BOS grade 0-p et les regreffes. Conclusion InfinitixBOS est le 1er essai therapeutique randomise controle evaluant l’efficacite du Nintedanib dans les formes obstructives/mixtes de CLAD. En cas de demonstration d’une efficacite sur la fonction pulmonaire, le benefice au patient est eleve au vu de la frequence du SBO.
Published: 2021
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45. Rôle de la vaccination antigrippale sur le risque d’exacerbation aiguë dans la fibrose pulmonaire idiopathique

Author: Karine Juvin, Julie Traclet, L. Sesé, Z. Carton, Stéphanie Guillot, Dominique Valeyre, Bruno Crestani, Bernard Maitre, Violaine Giraud, Philippe Bonniaud, Sandrine Hirschi, J. Caliez, Sandra Dury, A. Alari, Hilario Nunes, Sylvain Marchand-Adam, G. Prévot, Giancarlo Pesce, Thomas Gille, Raphael Borie, Jacques Cadranel, Dominique Israel-Biet, Vincent Cottin, Abdellatif Tazi, Anne Gondouin, and Benoit Wallaert
Subjects: Pulmonary and Respiratory Medicine
Abstract: Introduction La vaccination antigrippale est recommandee chaque annee chez les patients atteints de fibrose pulmonaire idiopathique (FPI). Les infections virales sont reconnues comme des triggers d’exacerbation aigue (EA). L’objectif de cette etude etait d’etudier l’influence de la vaccination annuelle contre la grippe sur le risque d’EA. Methodes La cohorte prospective longitudinale multicentrique COhorte FIbrose (COFI) comporte 236 FPI et suivi a dure 5 ans. Le statut de vaccinal contre la grippe a ete demande chaque annee. Nous avons defini la couverture vaccinale du 1er septembre au 30 aout de l’annee suivante. 638 visites annuelles etaient disponibles avec un statut vaccinal renseigne. L’etude entre la vaccination grippale et l’incidence des EA a ete realisee par un premier modele de Cox a effets aleatoire puis par un second modele de Cox en divisant les patients en trois groupes: (1) les patients vaccines chaque annee, (2) ceux vaccines pendant au moins la moitie des annees de suivi, (3) ceux vaccines moins de la moitie des annees de suivi. Resultats Le suivi moyen etait de 33,2 ± 23,6 mois. 36 EA sont survenues chez 33 patients. Sur les 33 patients, seuls 27 avaient un statut vaccinal precise l’annee de l’EA. Neuf (33%) des patients ayant presente une EA n’ont pas ete vaccines. Pour 141 (22%) visites annuelles, les patients n’ont pas ete vaccines. 133 patients ont ete vaccines chaque annee de suivi. 35 ont ete vaccines pendant au moins la moitie des annees de suivi, et 36 patients ont ete vaccines pendant moins de la moitie des annees de suivi. Dans le premier modele, la vaccination contre la grippe est associee a une diminution de la frequence des EA, mais l’association est non significative (HR: 0,58 CI95%0,23–1,46, p = 0,25). Dans le second modele, les patients vaccines pendant moins de la moitie des annees de suivi etaient significativement plus exposes au risque d’EA compares aux patients vaccines chaque annee (HR: 2,77 CI95%1.11–6.93, p = 0,03). Conclusion Dans COFI seulement environ la moitie des patients avaient une couverture vaccinale contre la grippe tout au long du suivi. La vaccination annuelle contre la grippe semble reduire le risque d’EA, mais par manque de puissance, cette association n’etait pas significative. Les patients vaccines pendant moins de la moitie des annees de suivi etaient plus exposes au risque d’EA que les patients vaccines chaque annee.
Published: 2021
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46. Early and Late Atrial Arrhythmias After Lung Transplantation ― Incidence, Predictive Factors and Impact on Mortality ―

Author: Olivier Morel, Pierre Emmanuel Falcoz, Michel Kindo, Gilbert Massard, Anne Olland, Nicola Santelmo, Han S. Lim, Romain Kessler, Halim Marzak, Sandrine Hirschi, Michel Chauvin, Patrick Ohlmann, Jeremie Barraud, Laurence Jesel, and Nathan Messas
Subjects: Adult, medicine.medical_specialty, Adolescent, medicine.medical_treatment, 030204 cardiovascular system & hematology, 03 medical and health sciences, Postoperative Complications, 0302 clinical medicine, Risk Factors, Internal medicine, Atrial Fibrillation, medicine, Humans, Lung transplantation, 030212 general & internal medicine, Mortality, Child, Aged, business.industry, Incidence, Incidence (epidemiology), Arrhythmias, Cardiac, Atrial fibrillation, General Medicine, Odds ratio, Atrial arrhythmias, Middle Aged, medicine.disease, Confidence interval, Predictive factor, Atrial Flutter, Cardiology, Cardiology and Cardiovascular Medicine, business, Atrial flutter, Lung Transplantation
Abstract: Background Atrial arrhythmias (AAs) are frequent after lung transplantation (LT) and late postoperatively. Several predictive factors of early postoperative AAs after LT have been identified but those of late AAs remain unknown. Whether AA after LT affects mortality is still being debated. This study assessed in a large cohort of LT patients the incidence of AAs early and late after surgery, their predictive factors and their effect on mortality.Methods and Results:We studied 271 consecutive LT patients over 9 years. Mean follow-up was 2.9±2.4 years. 33% patients developed postoperative AAs. Age (odds ratio (OR) 2.35; confidence interval (CI) [1.31-4.24]; P=0.004) and chronic obstructive pulmonary disease (OR 2.13; CI [1.12-4.03]; P=0.02) were independent predictive factors of early AAs. Late AAs occurred 2.2±2.7 years after transplant in 8.8% of the patients. Pretransplant systolic pulmonary arterial pressure (PTsPAP) was the only independent predictive factor of late AA (OR 1.028; CI [1.001-1.056]; P=0.04). Double LT was associated with long-term freedom from atrial fibrillation (AF) but not from atrial flutter (AFL). Early and late AAs after surgery had no effect on mortality. Double LT was associated with better survival. Conclusions Early AA following LT is common in contrast with the low occurrence of late, often organized, AA. Early and late AAs do not affect mortality. PTsPAP is an independent predictor of late AA. Double LT protects against late AF but not AFL.
Published: 2017
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47. Revisiting the systemic vasculitis in eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

Author: Jean-François Cordier, Paolo Bottero, Benoit Wallaert, Sylvain Marchand-Adam, Camille Taillé, Renato Alberto Sinico, Chahéra Khouatra, Romain Lazor, Pasupathy Sivasothy, Klaus Dalhoff, Sandrine Hirschi, Philippe Delaval, Christian Pagnoux, Anne Gondouin, Matthieu Groh, Christophe Briault, Elisabeth H. Bel, Marc Humbert, Loïc Guillevin, Philippe Bonniaud, Grégoire Prévot, Gérard Chatté, Vincent Cottin, Michael E. Wechsler, Jacques Cadranel, Bertrand Dunogué, and David Jayne
Subjects: 030203 arthritis & rheumatology, Pathology, medicine.medical_specialty, Systemic disease, Mononeuritis Multiplex, business.industry, Immunology, medicine.disease, Capillaritis, Dermatology, 3. Good health, 03 medical and health sciences, 0302 clinical medicine, 030228 respiratory system, Eosinophilic, medicine, Immunology and Allergy, Eosinophilia, medicine.symptom, Granulomatosis with polyangiitis, business, Vasculitis, Systemic vasculitis
Abstract: Objective To guide nosology and classification of patients with eosinophilic granulomatosis with polyangiitis (EGPA) based on phenotype and presence or absence of ANCA. Methods Organ manifestations and ANCA status were retrospectively analyzed based on the presence or not of predefined definite vasculitis features or surrogates of vasculitis in patients asthma, eosinophilia, and at least one systemic organ manifestation attributable to systemic disease. Results The study population included 157 patients (mean age 49.4 ± 14.1), with a follow-up of 7.4 ± 6.4 years. Patients with ANCA (31%) more frequently had weight loss, myalgias, arthralgias, biopsy-proven vasculitis, glomerulonephritis on biopsy, hematuria, leukocytoclastic capillaritis and/or eosinophilic infiltration of arterial wall on biopsy, and other renal disease. A total of 41% of patients had definite vasculitis manifestations (37%) or strong surrogates of vasculitis (4%), of whom only 53% had ANCA. Mononeuritis multiplex was associated with systemic vasculitis (p = 0.005) and with the presence of ANCA (p
Published: 2017
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48. 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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49. 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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50. Revised UIP/IPF diagnoses after the 2018 international guidelines: a 15-year Padova-Strasbourg experience

Author: Chiara Giraudo, Elisabetta Cocconcelli, Federico Rea, Elisabetta Balestro, Eleonora Faccioli, Francesca Lunardi, Mickaël Ohana, Romain Kessler, Aissam Labani, Marie-Pierrette Chenard, Antonin Fattori, Fiorella Calabrese, Roberta Polverosi, and Sandrine Hirschi
Subjects: Fibroblastic foci, medicine.medical_specialty, business.industry, Diagnostic accuracy, respiratory system, medicine.disease, humanities, respiratory tract diseases, Idiopathic pulmonary fibrosis, Usual interstitial pneumonia, Internal medicine, Metaplasia, medicine, Medical diagnosis, medicine.symptom, Good practice, business, Hypersensitivity pneumonitis
Abstract: Improved knowledge has highlighted the importance of additional histological and clinico/radiological features for the diagnosis of Usual Interstitial Pneumonia(UIP)-non Idiopathic Pulmonary Fibrosis(IPF) patients. We present preliminary findings of bicentric revised UIP/IPF cases after a multidisciplinary discussion (MDD) according to the 2018 ATS/ERS/JRS/ALAT Guidelines. All 2002-2017 cases with diagnosis of IPF were retrieved. Histological review was performed considering features suggestive of other diagnoses, i.e.connective tissue diseases (CTD) (germinal centers, >4 lymphoid follicles/cm2, lymphocyte/plasma-cell ratio ≥1, lymphocytic pleuritis, vessel remodelling) and chronic hypersensitivity pneumonitis (CHP) (bridging fibrosis, peribronchial metaplasia, centrolobular fibroblastic foci, granulomas/giant-cells). Clinico/radiological data were reviewed and all cases were re-categorized after MDD. 62 cases were reviewed:in 47% the final diagnosis was IPF, in 11% IPF(likely), in 13% indeterminate IPF and in 29% non-IPF. Among the “indeterminate IPF”, the most prevalent diagnosis was CHP, while in the “non-IPF”, CHP and CTD were almost equally detected (see Figure). In conclusion, the rate of IPF diagnoses appears to have been overestimated. The current knowledge and good practice of MDD have significantly improved the diagnostic accuracy.
Published: 2019
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