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1. Lung cancer in combined pulmonary fibrosis and emphysema: a series of 47 Western patients.

Author

Girard N, Marchand-Adam S, Naccache JM, Borie R, Urban T, Jouneau S, Marchand E, Ravel AC, Kiakouama L, Etienne-Mastroianni B, Cadranel J, Cottin V, and Cordier JF

Subjects
Aged, Antineoplastic Agents, Hormonal therapeutic use, Dyspnea etiology, Emphysema physiopathology, Female, Forced Expiratory Volume, France, Humans, Lung Neoplasms diagnosis, Male, Middle Aged, Pneumonectomy, Pulmonary Fibrosis physiopathology, Pulmonary Gas Exchange, Radiotherapy, Adjuvant, Retrospective Studies, Smoking, Survival Rate, Syndrome, Tomography, X-Ray Computed, Vital Capacity, Emphysema diagnostic imaging, Lung Neoplasms pathology, Lung Neoplasms therapy, Pulmonary Fibrosis diagnostic imaging
Abstract

Introduction: The syndrome of combined pulmonary fibrosis and emphysema (CPFE) is characterized by imaging features consisting of the association of centrilobular and/or paraseptal emphysema and pulmonary fibrosis. Virtually all patients are smokers and thus at high risk of developing lung cancer., Methods: This retrospective multicentre study was conducted by the Groupe d'Etudes et de Recherche sur les Maladies "Orphelines" Pulmonaires (GERM"O"P)., Results: A total of 47 patients presenting with lung cancer and CPFE syndrome were identified. All patients were smokers, with a mean of 47 pack-years. A pathological diagnosis of lung cancer was obtained for 38 (81%) patients. Histological type was squamous cell carcinoma in 17 (36%) patients, adenocarcinoma in 14 (30%), non-small-cell lung cancer not otherwise specified in three (6%), small-cell lung cancer in three (6%), and sarcomatoid carcinoma in one (2%). Overall, 20 of the 47 patients could not receive standard-of-care treatment for lung cancer, as per international recommendations or guidelines; this limitation was considered to be directly related to the CPFE syndrome in eight (40%) cases., Conclusion: Lung cancer in patients with CPFE syndrome represents a specific entity with a poor prognosis, that further represents the most characteristic and severe model of tobacco-related disease.

Published
2014
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2. Recommandations pratiques pour le diagnostic et la prise en charge de la fibrose pulmonaire idiopathique – Actualisation 2021. Version intégrale

Author

Cottin, Vincent, Bonniaud, Philippe, Cadranel, Jacques, Crestani, Bruno, Jouneau, Stéphane, Marchand-Adam, Sylvain, Nunes, Hilario, Wémeau-Stervinou, Lidwine, Bergot, Emmanuel, Blanchard, Elodie, Borie, Raphaël, Bourdin, Arnaud, Chenivesse, Cécile, Clément, Annick, Gomez, Emmanuel, Gondouin, Anne, Hirschi, Sandrine, Lebargy, François, Marquette, Charles-Hugo, Montani, David, Prévot, Grégoire, Quetant, Sébastien, Reynaud-Gaubert, Martine, Salaun, Mathieu, Sanchez, Olivier, Trumbic, Bruno, Berkani, Karim, Brillet, Pierre-Yves, Campana, Marion, Chalabreysse, Lara, Chatté, Gérard, Debieuvre, Didier, Ferretti, Gilbert, Fourrier, Jean-Michel, Just, Nicolas, Kambouchner, Marianne, Legrand, Bertrand, Le Guillou, Frédéric, Lhuillier, Jean-Pierre, Mehdaoui, Anas, Naccache, Jean-Marc, Paganon, Catherine, Rémy-Jardin, Martine, Si-Mohamed, Salim, Terrioux, Philippe, 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), 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), Association pour l’amélioration de la prise en charge des maladies pulmonaires rares, Hospices Civils de Lyon, and Université Claude Bernard Lyon 1.

Subjects
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.; Contexte :Depuis les précédentes recommandations françaises publiées en 2017, les connaissances concernant la fibrose pulmonaire idiopathique ont évolué sur de nombreux aspects.Méthodes :Des recommandations pratiques ont été établies à l’initiative du Centre coordonnateur de référence des maladies pulmonaires rares, sous l’égide de la Société de Pneumologie de Langue Française, par un groupe de coordination, un groupe de rédaction, et un groupe de lecture, impliquant l’ensemble du réseau OrphaLung, des pneumologues de divers modes d’exercice, des radiologues, des pathologistes, un médecin généraliste, un cadre de santé, et une association de patients. La méthode a respecté les règles d’élaboration des « Bonnes pratiques cliniques » selon la méthode des « Recommandations pour la pratique clinique » de la Haute Autorité de Santé, incluant un vote en ligne selon une échelle Likert.Résultats :Après une analyse bibliographique, 54 recommandations ont été formulées, améliorées, puis validées par les groupes de travail, portant sur des aspects multiples de la maladie : épidémiologie, modalités diagnostiques, critères de qualité et interprétation du scanner thoracique, indication et réalisation de la biopsie pulmonaire, bilan étiologique, modalités et indications de l’enquête familiale et de l’analyse génétique, évaluation du retentissement fonctionnel et du pronostic, indication et emploi des médicaments antifibrosants, transplantation pulmonaire, prise en charge des symptômes, comorbidités et complications, traitement de l’insuffisance respiratoire chronique, diagnostic et prise en charge des exacerbations aiguës de fibrose.Conclusion :Ces recommandations fondées sur les preuves sont destinées à guider le diagnostic et la prise en charge pratique de la fibrose pulmonaire idiopathique.

Published
2022
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3. Determinants of survival after lung transplantation in telomerase-related gene mutation carriers: A retrospective cohort

Author

Phillips-Houlbracq, Mathilde, Mal, Hervé, Cottin, Vincent, Gauvain, Clément, Beier, Fabian, Sicre de Fontbrune, Flore, Sidali, Sabrina, Mornex, Jean François, Hirschi, Sandrine, Roux, Antoine, Weisenburger, Gaelle, Roussel, Arnaud, Wémeau-Stervinou, Lidwine, Le Pavec, Jérôme, Pison, Christophe, Marchand Adam, Sylvain, Froidure, Antoine, Lazor, Romain, Naccache, Jean-Marc, Jouneau, Stéphane, Nunes, Hilario, Reynaud-Gaubert, Martine, Le Borgne, Aurélie, Boutboul, David, Ba, Ibrahima, Boileau, Catherine, Crestani, Bruno, Kannengiesser, Caroline, Borie, Raphaël, OrphaLung Network, UCL - SSS/IREC/PNEU - Pôle de Pneumologie, ORL et Dermatologie, UCL - (SLuc) Service de pneumologie, 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), AP-HP - Hôpital Bichat - Claude Bernard [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre de Référence des Maladies Pulmonaires Rares [Hôpital Louis Pradel - HCL], Hôpital Louis Pradel [CHU - HCL], Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), 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), CHU Lille, Rheinisch-Westfälische Technische Hochschule Aachen University (RWTH), Hopital Saint-Louis [AP-HP] (AP-HP), Hôpital Beaujon [AP-HP], Les Hôpitaux Universitaires de Strasbourg (HUS), Hôpital Foch [Suresnes], Université Paris-Saclay, Laboratory of Fundamental and Applied Bioenergetics = Laboratoire de bioénergétique fondamentale et appliquée (LBFA), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Grenoble Alpes (UGA), Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), Cliniques Universitaires Saint-Luc [Bruxelles], Centre Hospitalier Universitaire Vaudois [Lausanne] (CHUV), CHU Tenon [AP-HP], 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 ), École des Hautes Études en Santé Publique [EHESP] (EHESP), Hôpital Avicenne [AP-HP], Microbes évolution phylogénie et infections (MEPHI), Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS), Hôpital Nord [CHU - APHM], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Paris (UP), Rheinisch-Westfälische Technische Hochschule Aachen (RWTH), Hypertension arterielle pulmonaire physiopathologie et innovation thérapeutique, Centre chirurgical Marie Lannelongue-Institut National de la Santé et de la Recherche Médicale (INSERM), Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique )-Institut National de la Santé et de la Recherche Médicale (INSERM)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Université d'Angers (UA), Centre National de la Recherche Scientifique (CNRS)-Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU), Hôpital Larrey [Toulouse], CHU Toulouse [Toulouse], Université d'Angers (UA)-Université de Rennes 1 (UR1), and Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique )

Subjects
Male, medicine.medical_specialty, lung disease, Anemia, medicine.medical_treatment, [SDV]Life Sciences [q-bio], transplant social worker, 030204 cardiovascular system & hematology, Neutropenia, Gastroenterology, 03 medical and health sciences, 0302 clinical medicine, Interquartile range, Internal medicine, Pulmonary fibrosis, medicine, lung transplantation, Immunology and Allergy, Lung transplantation, Humans, Pharmacology (medical), Telomerase, pulmonology, Retrospective Studies, Transplantation, Lung, business.industry, fibrosis, Retrospective cohort study, Middle Aged, medicine.disease, Idiopathic Pulmonary Fibrosis, practice, 3. Good health, medicine.anatomical_structure, 030228 respiratory system, clinical research, Cohort, Mutation, business
Abstract

Carriers of germline telomerase-related gene (TRG) mutations can show poor prognosis, with an increase in common hematological complications after lung transplantation (LT) for pulmonary fibrosis. The aim of this study was to describe the outcomes after LT in recipients carrying a germline TRG mutation and to identify the predictors of survival. In a multicenter cohort of LT patients, we retrospectively reviewed those carrying pathogenic TRG variations (n = 38; TERT, n = 23, TERC, n = 9, RTEL1, n = 6) between 2009 and 2018. The median age at LT was 54 years (interquartile range [IQR] 46-59); 68% were male and 71% had idiopathic pulmonary fibrosis. During the diagnosis of pulmonary fibrosis, 28 (74%) had a hematological disease, including eight with myelodysplasia. After a median follow-up of 26 months (IQR 15-46), 38 patients received LT. The overall post-LT median survival was 3.75 years (IQR 1.8-NA). The risk of death after LT was increased for patients with myelodysplasia (HR 4.1 [95% CI 1.5-11.5]) or short telomere (HR 2.2 [1.0-5.0]) before LT. After LT, all patients had anemia, 66% had thrombocytopenia, and 39% had neutropenia. Chronic lung allograft dysfunction frequency was 29% at 4 years. The present findings support the use of LT in TRG mutation carriers without myelodysplasia. Hematological evaluation should be systematically performed before LT.

Published
2021
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4. Interstitial lung diseases associated with mutations of poly(A)‐specific ribonuclease: A multicentre retrospective study.

Author

Philippot, Quentin, Kannengiesser, Caroline, Debray, Marie Pierre, Gauvain, Clément, Ba, Ibrahima, Vieri, Margherita, Gondouin, Anne, Naccache, Jean‐Marc, Reynaud‐Gaubert, Martine, Uzunhan, Yurdagul, Bondue, Benjamin, Israël‐Biet, Dominique, Dieudé, Philippe, Fourrage, Cécile, Lainey, Elodie, Manali, Effrosyne, Papiris, Spyros, Wemeau, Lidwine, Hirschi, Sandrine, and Mal, Hervé

Subjects
TELOMERASE reverse transcriptase, RIBONUCLEASES, INTERSTITIAL lung diseases, IDIOPATHIC pulmonary fibrosis, VITAL capacity (Respiration), PULMONARY fibrosis, GENETIC mutation
Abstract

Background and objective: 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. Methods: 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. Results: 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. Conclusion: IPF is common among individuals with PARN mutation, but other ILD subtypes may be observed. The pulmonary phenotypes of 31 patients with interstitial lung disease (ILD) and heterozygous poly(A)‐specific ribonuclease (PARN) mutations in this cohort were heterogeneous, but idiopathic pulmonary fibrosis was the most frequent diagnosis. Haematological and hepatic features were less frequent than in patients affected with telomerase reverse transcriptase (TERT)‐ or telomerase RNA component (TERC)‐associated ILDs. [ABSTRACT FROM AUTHOR]

Published
2022
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5. Lung cancer in combined pulmonary fibrosis and emphysema: a series of 47 Western patients

Author

Girard, Nicolas, Marchand-Adam, Sylvain, Naccache, Jean-Marc, Borie, Raphael, Urban, Thierry, Jouneau, Stéphane, Marchand, Eric, Ravel, Anne-Claire, Kiakouama, Lize, Etienne-Mastroianni, Bénédicte, Cadranel, Jacques, Cottin, Vincent, Cordier, Jean-François, Maladies \\'Orphelines Pulmonaires (GERMOP), Groupe, Pathologies Respiratoires : Protéolyse et Aérosolthérapie, Université de Tours (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM), Institut de recherche en santé, environnement et travail (Irset), Université d'Angers (UA)-Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM), Interactions cellulaires tumorales et leur environnement et réponse aux agents anti-cancéreux, Université Pierre et Marie Curie - Paris 6 (UPMC), Service de Pneumologie - Oncologie Thoracique - Maladies Pulmonaires Rares [CHU Tenon], CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Intergroupe Francophone de Cancérologie Thoracique [Paris] (IFCT), Intergroupe Francophone de Cancérologie thoracique, Department of Pneumology [Lyon], Hospices Civils de Lyon (HCL), Centre de Référence des Maladies Pulmonaires Rares [Hôpital Louis Pradel - HCL], Hôpital Louis Pradel [CHU - HCL], Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Université de Tours-Institut National de la Santé et de la Recherche Médicale (INSERM), 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 ), and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)

Subjects
Male, Pulmonary and Respiratory Medicine, Pathology, medicine.medical_specialty, Lung Neoplasms, Antineoplastic Agents, Hormonal, Pulmonary Fibrosis, medicine.medical_treatment, [SDV]Life Sciences [q-bio], Vital Capacity, Gastroenterology, 03 medical and health sciences, Pneumonectomy, 0302 clinical medicine, Forced Expiratory Volume, Internal medicine, Pulmonary fibrosis, medicine, Humans, Lung cancer, Sarcomatoid carcinoma, Survival rate, Aged, Retrospective Studies, Emphysema, Pulmonary Gas Exchange, business.industry, Smoking, Not Otherwise Specified, Syndrome, Middle Aged, respiratory system, medicine.disease, Combined pulmonary fibrosis and emphysema, 3. Good health, respiratory tract diseases, Survival Rate, Dyspnea, 030228 respiratory system, Oncology, 030220 oncology & carcinogenesis, Adenocarcinoma, Female, Radiotherapy, Adjuvant, France, Tomography, X-Ray Computed, business
Abstract

International audience; INTRODUCTION: The syndrome of combined pulmonary fibrosis and emphysema (CPFE) is characterized by imaging features consisting of the association of centrilobular and/or paraseptal emphysema and pulmonary fibrosis. Virtually all patients are smokers and thus at high risk of developing lung cancer. METHODS: This retrospective multicentre study was conducted by the Groupe d'Etudes et de Recherche sur les Maladies "Orphelines" Pulmonaires (GERM"O"P). RESULTS: A total of 47 patients presenting with lung cancer and CPFE syndrome were identified. All patients were smokers, with a mean of 47 pack-years. A pathological diagnosis of lung cancer was obtained for 38 (81%) patients. Histological type was squamous cell carcinoma in 17 (36%) patients, adenocarcinoma in 14 (30%), non-small-cell lung cancer not otherwise specified in three (6%), small-cell lung cancer in three (6%), and sarcomatoid carcinoma in one (2%). Overall, 20 of the 47 patients could not receive standard-of-care treatment for lung cancer, as per international recommendations or guidelines; this limitation was considered to be directly related to the CPFE syndrome in eight (40%) cases. CONCLUSION: Lung cancer in patients with CPFE syndrome represents a specific entity with a poor prognosis, that further represents the most characteristic and severe model of tobacco-related disease.

Published
2014
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6. Usual interstitial pneumonia in ANCA-associated vasculitis: A poor prognostic factor.

Author

Maillet, Thibault, Goletto, Tiphaine, Beltramo, Guillaume, Dupuy, Henry, Jouneau, Stéphane, Borie, Raphael, Crestani, Bruno, Cottin, Vincent, Blockmans, Daniel, Lazaro, Estibaliz, Naccache, Jean-Marc, Pugnet, Grégory, Nunes, Hilario, de Menthon, Mathilde, Devilliers, Hervé, Bonniaud, Philippe, Puéchal, Xavier, Mouthon, Luc, Bonnotte, Bernard, and Guillevin, Loïc

Subjects
*GRANULOMATOSIS with polyangiitis, *PULMONARY fibrosis, *VASCULITIS, *MULTIVARIATE analysis, *INTERSTITIAL lung diseases
Abstract

Progressive fibrosing interstitial lung disease (ILD) is rarely associated with antineutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV). This study focused on the outcomes of ILD patients with associated AAV (AAV–ILD). AAV–ILD (cases: microscopic polyangiitis (MPA) or granulomatosis with polyangiitis (GPA) with ILD) were compared to AAV patients without ILD (controls). ILD was defined as a usual interstitial pneumonia (UIP) or non-specific interstitial pneumonia (NSIP) pattern. Two controls were matched to each case for age (>or ≤65 years), ANCA status (PR3-or MPO-positive) and creatininemia (≥or <150 μmol/L). Sixty-two cases (89% MPO-ANCA+) were included. Median age at AAV diagnosis was 66 years. ILD (63% UIP), was diagnosed before (52%) or simultaneously (39%) with AAV. Cases versus 124 controls less frequently had systemic vasculitis symptoms. One-, 3- and 5-year overall survival rates, respectively, were: 96.7%, 80% and 66% for cases versus 93.5%, 89.6% and 83.8% for controls (p = 0.008). Multivariate analyses retained age >65 years (hazard ratio (HR) 4.54; p < 0.001), alveolar haemorrhage (HR 2.25; p = 0.019) and UIP (HR 2.73; p = 0.002), but not immunosuppressant use, as factors independently associated with shorter survival. For AAV–ILD patients, only UIP was associated with poorer prognosis. Immunosuppressants did not improve the AAV–ILD prognosis. But in analogy to idiopathic pulmonary fibrosis, anti-fibrosing agents might be useful and should be assessed in AAV–ILD patients with a UIP pattern. • Progressive fibrosing interstitial lung disease is a rare comorbidity of MPO-vasculitis. • This condition is associated with shorter survival when it has a CT-scan UIP pattern. • Immunosuppressants did not improve the prognosis of these patients. • Our results support the evaluation of anti-fibrotic drugs in this condition. [ABSTRACT FROM AUTHOR]

Published
2020
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