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13 results on '"Cordier, J."'

1. Combined pulmonary fibrosis and emphysema in systemic sclerosis: A syndrome associated with heavy morbidity and mortality.

Author

Champtiaux N, Cottin V, Chassagnon G, Chaigne B, Valeyre D, Nunes H, Hachulla E, Launay D, Crestani B, Cazalets C, Jego P, Bussone G, Bérezné A, Guillevin L, Revel MP, Cordier JF, and Mouthon L

Subjects
Adolescent, Adult, Aged, Case-Control Studies, Female, Humans, Lung diagnostic imaging, Male, Middle Aged, Prognosis, Pulmonary Emphysema diagnostic imaging, Pulmonary Emphysema physiopathology, Pulmonary Fibrosis diagnostic imaging, Pulmonary Fibrosis physiopathology, Radiography, Thoracic, Respiratory Function Tests, Retrospective Studies, Scleroderma, Systemic diagnostic imaging, Scleroderma, Systemic physiopathology, Tomography, X-Ray Computed, Young Adult, Lung physiopathology, Pulmonary Emphysema complications, Pulmonary Fibrosis complications, Scleroderma, Systemic complications
Abstract

Background: The syndrome of combined pulmonary fibrosis and emphysema (CPFE) primarily due to tobacco smoking has been reported in connective tissue disease, but little is known about its characteristics in systemic sclerosis (SSc)., Methods: In this retrospective multi-center case-control study, we identified 36 SSc patients with CPFE, and compared them with 72 SSc controls with interstitial lung disease (ILD) without emphysema., Results: Rate of CPFE in SSc patients with CT scan was 3.6%, and 7.6% among SSc patients with ILD. CPFE-SSc patients were more likely to be male (75 % vs 18%, p < 0.0001), smokers (83 % vs 33%, p < 0.0001), and to have limited cutaneous SSc (53 % vs 24% p < 0.01) than ILD-SSc controls. No specific autoantibody was significantly associated with CPFE. At diagnosis, CPFE-SSc patients had a greater decrease in carbon monoxide diffusing capacity (DLCO 39 ± 13 % vs 51 ± 12% of predicted value, p < 0.0001) when compared to SSc-ILD controls, whereas lung volumes (total lung capacity and forced vital capacity) were similar. During follow-up, CPFE-SSc patients more frequently developed precapillary pulmonary hypertension (PH) (44 % vs 11%, p < 10 -4 ), experienced more frequent unscheduled hospitalizations (50 % vs 25%, p < 0.01), and had decreased survival (p < 0.02 by Kaplan-Meier survival analysis) as compared to ILD-SSc controls., Conclusions: The CPFE syndrome is a distinct pulmonary manifestation in SSc, with higher morbidity and mortality. Early diagnosis of CPFE by chest CT in SSc patients (especially smokers) may result in earlier smoking cessation, screening for PH, and appropriate management., (Copyright © 2018. Published by Elsevier Inc.)

Published
2019
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3. Milestones in lymphangioleiomyomatosis research.

Author

Cottin V, Archer F, Leroux C, Mornex JF, and Cordier JF

Subjects
Biomedical Research, Diagnosis, Differential, Female, Gene Expression Regulation, Humans, Lung metabolism, Lymphangioleiomyomatosis etiology, Lymphangioleiomyomatosis genetics, Lymphangioleiomyomatosis metabolism, Lymphangioleiomyomatosis therapy, Myocytes, Smooth Muscle metabolism, Predictive Value of Tests, Risk Factors, Sex Factors, Signal Transduction, Treatment Outcome, Tuberous Sclerosis complications, Cell Proliferation, Lung pathology, Lymphangioleiomyomatosis pathology, Myocytes, Smooth Muscle pathology
Published
2011
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5. Fibrosing vasculitis in Wegener's granulomatosis: ultrastructural and immunohistochemical analysis of the vascular lesions.

Author

Gindre D, Peyrol S, Raccurt M, Sommer P, Loire R, Grimaud JA, and Cordier JF

Subjects
Arteries chemistry, Humans, Male, Microscopy, Electron, Middle Aged, Extracellular Matrix Proteins analysis, Granulomatosis with Polyangiitis pathology, Lung blood supply, Lung chemistry, Pulmonary Fibrosis pathology, Vasculitis pathology
Abstract

This study of two cases of pulmonary Wegener's granulomatosis (WG) focuses on the ultrastructural aspects of the vascular wall injury and on the immunohistochemical characterization of the perivascular connective matrix. The iterative waves of endothelial cell necrosis and regeneration are demonstrated by the multilamellar appearance of the basal lamina. Neutrophils infiltrate the vessel wall and myofibroblasts are recruited to injured vessels. The perivascular connective matrix associates basement-membrane like and fibrillar material with fibrin deposits. The initiation of the fibrosing process was assessed by the visualization of matrix molecules involved in targeting (p-fibronectin), organizing (cellular fibronectin and tenascin) and stabilizing (lysyl-oxidase) the fibrogenic activity. These elementary lesions affect different levels of the vascular tree, and capillaritis is involved in the extension of the pathological process. Lysyl-oxidase labelling reveals the fibrosing front which is located on the border of dense fibrosis. The markers of fibrosing activity disappear in the areas of fibrosis following vasculitis and/or ischaemic necrosis and/or granulomatosis. Vasculitis plays a major role in both the genesis and progression of the fibrosis observed in the late stage of WG.

Published
1995
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6. Characterization of the smooth muscle cell infiltrate and associated connective matrix of lymphangiomyomatosis. Immunohistochemical and ultrastructural study of two cases.

Author

Peyrol S, Gindre D, Cordier JF, Loire R, and Grimaud JA

Subjects
Adult, Cytoskeletal Proteins analysis, Female, Fluorescent Antibody Technique, Humans, Lung chemistry, Lung Neoplasms chemistry, Lymph Nodes ultrastructure, Lymphangiomyoma chemistry, Lung ultrastructure, Lung Neoplasms ultrastructure, Lymphangiomyoma ultrastructure, Muscle, Smooth ultrastructure
Abstract

Lymphangiomyomatosis (LAM) consists of smooth muscle (SM) cell proliferation of unknown origin involving the lymph nodes and the lung interstitium. From morphological studies showing both SM differentiation of the proliferating cells and lymphatic hyperplasia, hypotheses were suggested concerning the origin of the proliferation. Two cases of LAM were investigated by electron microscopy and immunohistochemistry; tissues were obtained by lymph node and open lung biopsies. Cytoplasmic and matrix protein markers were used in order to clarify the pattern of differentiation of the proliferating cells and to characterize their connective tissue environment. The proliferating cells present ultrastructural characteristics of SM cells; they contain vimentin, desmin, and alpha-SM actin and are devoid of Factor VIII, favouring a parieto-arterial origin. The connective tissue matrix inside the infiltrate is composed of interstitial collagens and basement membrane components. At the late stage of the disease, remodelling of the interstitial matrix accompanies the infiltrate and remains perilesional.

Published
1992
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7. Idiopathic bronchiolitis obliterans organizing pneumonia. Definition of characteristic clinical profiles in a series of 16 patients.

Author

Cordier JF, Loire R, and Brune J

Subjects
Adult, Aged, Bronchiolitis Obliterans classification, Bronchoalveolar Lavage Fluid cytology, Female, Humans, Lung diagnostic imaging, Male, Middle Aged, Pneumonia classification, Radiography, Respiratory Function Tests, Bronchiolitis Obliterans diagnosis, Lung pathology, Pneumonia diagnosis
Abstract

Bronchiolitis obliterans organizing pneumonia (BOOP) is a pathologic finding common to various injuries to the lung of either definite or idiopathic etiology. Since the presentation of patients with idiopathic BOOP varies, we studied 16 patients with BOOP on pulmonary histology to define more distinct and homogeneous clinical and imaging profiles of idiopathic BOOP. We distinguished three groups of patients: group 1 (n = 4), with multiple patchy migratory pulmonary involvement of the pneumonia type. Their clinical course was subacute, with cough, fever, weight loss, mild dyspnea, and increased ESR. Chest x-ray film and CT scan showed multiple alveolar opacities. All patients completely recovered with corticosteroid therapy but relapsed when therapy was stopped too rapidly. Group 2 (n = 5) had solitary pulmonary involvement of the pneumonia type occurring in a similar clinical context. Since carcinoma was suspected, they underwent surgical excision of the pneumonic area and recovered without relapse. Group 3 patients (n = 7) presented with diffuse pulmonary involvement of the interstitial lung disease type. They had more progressive onset of more severe dyspnea, crackles heard over all lung surfaces, and interstitial opacities with or without alveolar opacities on chest imaging. Improvement with corticosteroid therapy was obtained in only three patients. In all three groups, lung function test results showed a restrictive pattern. The obstructive pattern characteristic of pure bronchiolitis obliterans was found in none. BAL showed a mixed pattern (increase of both lymphocytes and polymorphonuclear cells) in the patients of the first two groups. Thus, we distinguished three characteristic clinical and imaging profiles in patients with idiopathic BOOP: multiple patchy pneumonia, solitary pneumonia, and diffuse interstitial lung disease. These profiles are so different that they should be distinguished in clinical studies of idiopathic BOOP.

Published
1989
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8. [Pulmonary collagens and their pathology (author's transl)].

Author

Cordier JF, Grimaud JA, Lasne Y, and Touraine R

Subjects
Bleomycin therapeutic use, Collagen biosynthesis, Collagen Diseases physiopathology, Ehlers-Danlos Syndrome pathology, Humans, Lung ultrastructure, Microscopy, Electron, Pulmonary Fibrosis drug therapy, Collagen physiology, Lung metabolism
Abstract

Collagen is a macro-molecule of polypeptide chains arranged in a triple helix. The biosynthesis and degradation of collagen require complex enzymes systems; considerable progress has been made in their understanding in recent years. Collagen is the most important connective tissue of the body and five types are present in the lower respiratory tract. Genetic disorders of collagen (e.g. the Ehlers-Danlos or Marfan's syndrome) may affect the lung. Furthermore certain pulmonary diseases such as fibrosis are associated with changes in the ratio of the different types of collagen. Collagen can no longer be dissociated from the study of tissue changes in pulmonary pathology; the aim of our study is to provide elementary data concerning this aspect of modern pneumology.

Published
1982

9. Gallium-67 scanning in the staging of cryptogenetic fibrosing alveolitis and hypersensitivity pneumonitis.

Author

Vanderstappen M, Mornex JF, Lahneche B, Chauvot P, Bouvier JF, Wiesendanger T, Pages J, Webert P, Cordier JF, and Brune J

Subjects
Citric Acid, Female, Humans, Male, Middle Aged, Radionuclide Imaging, Respiratory Function Tests, Alveolitis, Extrinsic Allergic diagnostic imaging, Citrates, Lung diagnostic imaging, Pulmonary Fibrosis diagnostic imaging
Abstract

Gallium-67 citrate is known to localize within inflammatory sites. Gallium-67 scanning is used for the evaluation of lung inflammation (i.e. alveolitis) during interstitial lung diseases. We investigated 27 patients with cryptogenetic fibrosing alveolitis (n = 17) and hypersensitivity pneumonitis (n = 10) using gallium-67 lung scanning and lung function tests (forced vital capacity, diffusing capacity, resting and exercise blood gases). Investigations were performed before and after one year of methylprednisolone treatment. None of eight healthy volunteers had any abnormal gallium-67 uptake. In all patients with cryptogenetic fibrosing alveolitis an initial abnormal gallium-67 uptake was observed (mean fixation index: 163 +/- 18). In addition, analysis of lung function tests a year after initial evaluation showed that unchanged or improving patients presented initially with a lower gallium-67 index than patients with evidence of deterioration (153.9 +/- 23.7 vs 251.0 +/- 23.3.; p less than 0.01). Similarly, among patients with hypersensitivity pneumonitis the index was lower in unchanged or improving patients than in those with deterioration (74.9 +/- 22 vs 226.7 +/- 4.9; p less than 0.05). Thus gallium-67 scanning is useful in the management of cryptogenetic fibrosing alveolitis and hypersensitivity pneumonitis.

Published
1988

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

Author

Cottin, V., Crestani, B., Cadranel, J., Cordier, J.-F., Marchand-Adam, Sylvain, Prévôt, G., Wallaert, B., Bergot, E., Camus, P., Dalphin, J.-C., Dromer, C., Gomez, E., Israel-Biet, D., Jouneau, S., Kessler, R., Marquette, C.-H., Reynaud-Gaubert, M., Aguilaniu, B., Bonnet, D., Carré, P., Danel, C., Faivre, J.-B., Ferretti, G., Just, N., Lebargy, F., Philippe, B., Terrioux, P., Thivolet-Béjui, F., Trumbic, B., Valeyre, D., Centre de Référence des Maladies Pulmonaires Rares [Hôpital Louis Pradel - HCL], Hôpital Louis Pradel [CHU - HCL], Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Service de Pneumologie A, AP-HP - Hôpital Bichat - Claude Bernard [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Centre de compétence des maladies pulmonaires rares, 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), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Service de Pneumologie et Immuno-Allergologie [CHU LIlle], Pole Cardio-vasculaire et pulmonaire [CHU Lille], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN), Service de Pneumologie Soins Intensifs, Appareillage Respiratoire [CHU de Dijon], Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), Laboratoire Chrono-environnement (UMR 6249) (LCE), Centre National de la Recherche Scientifique (CNRS)-Université de Franche-Comté (UFC), Université Bourgogne Franche-Comté [COMUE] (UBFC)-Université Bourgogne Franche-Comté [COMUE] (UBFC), CHU Bordeaux [Bordeaux], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Service de Pneumologie, Paris, Institut de recherche en santé, environnement et travail (Irset), Université d'Angers (UA)-Université de Rennes (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 ), Nouvel Hôpital Civil Strasbourg, Centre Hospitalier Universitaire de Nice (CHU Nice), Unité de Recherche sur les Maladies Infectieuses et Tropicales Emergentes (URMITE), Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR48, Institut des sciences biologiques (INSB-CNRS)-Institut des sciences biologiques (INSB-CNRS)-Centre National de la Recherche Scientifique (CNRS), Université Grenoble Alpes [2016-2019] (UGA [2016-2019]), Centre Hospitalier de la Côte Basque (CHCB), Centre Hospitalier de Carcassonne, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Centre Hospitalier de Roubaix, Plasticité de l'épithélium respiratoire dans les conditions normales et pathologiques - UMR-S 903 (PERPMP), Université de Reims Champagne-Ardenne (URCA)-Centre Hospitalier Universitaire de Reims (CHU Reims)-Institut National de la Santé et de la Recherche Médicale (INSERM)-SFR CAP Santé (Champagne-Ardenne Picardie Santé), Université de Reims Champagne-Ardenne (URCA)-Université de Reims Champagne-Ardenne (URCA), Centre Hospitalier de Meaux, Groupe Hospitalier Est, Centre de Pathologie Est, Hospices Civils de Lyon (HCL), Service des maladies respiratoires, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Groupe Hospitalier Avicenne-Jean Verdier-René Muret, Hospices Civils de Lyon ( HCL ) -Hospices Civils de Lyon ( HCL ), AP-HP - Hôpital Bichat - Claude Bernard [Paris]-Centre de compétence des maladies pulmonaires rares, Service de pneumologie et réanimation [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Tenon [APHP], Université de Tours-Institut National de la Santé et de la Recherche Médicale ( INSERM ), CHU Toulouse [Toulouse], Service de Pneumologie et Immuno-Allergologie, Centre Hospitalier Régional Universitaire [Lille] ( CHRU Lille ), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand ( CHU Dijon ), Centre Hospitalier Régional Universitaire [Besançon] ( CHRU Besançon ), Centre Hospitalier Régional Universitaire de Nancy ( CHRU Nancy ), Institut de recherche, santé, environnement et travail ( Irset ), Université d'Angers ( UA ) -Université de Rennes 1 ( UR1 ), Université de Rennes ( UNIV-RENNES ) -Université de Rennes ( UNIV-RENNES ) -École des Hautes Études en Santé Publique [EHESP] ( EHESP ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ) -Université des Antilles ( UA ), CHU Nice, Unité de Recherche sur les Maladies Infectieuses et Tropicales Emergentes ( URMITE ), Institut de Recherche pour le Développement ( IRD ) -Aix Marseille Université ( AMU ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -IFR48, INSB-INSB-Centre National de la Recherche Scientifique ( CNRS ), Université Grenoble Alpes ( UGA ), Le CHCB, Centre Hospitalier de la Côte Basque, Plasticité de l'épithélium respiratoire dans les conditions normales et pathologiques - UMR-S 903 ( PERPMP ), Université de Reims Champagne-Ardenne ( URCA ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -SFR CAP Santé (Champagne-Ardenne Picardie Santé), Université de Reims Champagne-Ardenne ( URCA ) -Université de Picardie Jules Verne ( UPJV ) -Université de Reims Champagne-Ardenne ( URCA ) -Université de Picardie Jules Verne ( UPJV ) -CHU de Reims - Hôpital Maison Blanche, Laboratoire Chrono-environnement ( LCE ), Université Bourgogne Franche-Comté ( UBFC ) -Centre National de la Recherche Scientifique ( CNRS ) -Université de Franche-Comté ( UFC ), Hospices Civils de Lyon ( HCL ), Assistance publique - Hôpitaux de Paris (AP-HP)-Groupe Hospitalier Avicenne-Jean Verdier-René Muret, 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), 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), INSB-INSB-Centre National de la Recherche Scientifique (CNRS), Université de Reims Champagne-Ardenne (URCA)-Université de Picardie Jules Verne (UPJV)-Université de Reims Champagne-Ardenne (URCA)-Université de Picardie Jules Verne (UPJV), Laboratoire Chrono-environnement - CNRS - UBFC (UMR 6249) (LCE), Université de Tours-Institut National de la Santé et de la Recherche Médicale (INSERM), 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 ), SFR CAP Santé (Champagne-Ardenne Picardie Santé), and Université de Reims Champagne-Ardenne (URCA)-Université de Picardie Jules Verne (UPJV)-Université de Reims Champagne-Ardenne (URCA)-Université de Picardie Jules Verne (UPJV)-Centre Hospitalier Universitaire de Reims (CHU Reims)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Reims Champagne-Ardenne (URCA)

Subjects
Pulmonary and Respiratory Medicine, [SDV.EE.SANT]Life Sciences [q-bio]/Ecology, environment/Health, Biopsy, Bronchoalveolar Lavage, Idiopathic Pulmonary Fibrosis, 3. Good health, [ SDV.EE.SANT ] Life Sciences [q-bio]/Ecology, environment/Health, Diagnosis, Differential, 03 medical and health sciences, 0302 clinical medicine, 030228 respiratory system, Evidence-Based Practice, Pulmonary Medicine, Humans, Radiography, Thoracic, France, 030212 general & internal medicine, Tomography, X-Ray Computed, Lung, Algorithms, ComputingMilieux_MISCELLANEOUS
Abstract

International audience; no abstract

Published
2017

11. Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity

Author

Cottin, V., Nunes, H., Brillet, P.-Y., Delaval, P., Devouassaoux, G., Tillie-Leblond, I., Israel-Biet, D., Court-Fortune, I., Valeyre, D., Cordier, J.-F., Carré, P., Chabot, F., Chatté, G., Coëtmeur, D., Crestani, B., Dalphin, J.C., Dietemann, A., Gentil, B., Humbert, M., Lacronique, J., Mairesse, M., Marchand, Eric, Reynaud-Gaubert, M., Rétrovirus et Pathologie Comparée (RPC), Institut National de la Recherche Agronomique (INRA)-École pratique des hautes études (EPHE), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Ecole Nationale Vétérinaire de Lyon (ENVL), Centre Hospitalier Universitaire de Lyon, Service de Pneumologie, Centre Hospitalier Lyon Sud [CHU - HCL] (CHLS), Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Hôpital Avicenne [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), CHU Pontchaillou [Rennes], Centre Hospitalier Universitaire de Lille (CHU de Lille), Centre Hospitalier Universitaire de Saint-Etienne (CHU de Saint-Etienne), Hospices Civils de Lyon (HCL), UCL - MD/MINT - Département de médecine interne, and UCL - (MGD) Service de pneumologie

Subjects
Adult, Male, Pulmonary and Respiratory Medicine, Spirometry, medicine.medical_specialty, Hypertension, Pulmonary, [SDV]Life Sciences [q-bio], Interstitial lung disease, EMPHYSEMA, 030204 cardiovascular system & hematology, Pulmonary arterial hypertension, Pulmonary fibrosis, Pulmonary function testing, 03 medical and health sciences, FEV1/FVC ratio, 0302 clinical medicine, Internal medicine, medicine, Humans, PULMONARY ARTERIAL HYPERTENSION, Aged, Retrospective Studies, Aged, 80 and over, Emphysema, INTERSTITIAL LUNG DISEASE, Lung, medicine.diagnostic_test, business.industry, Smoking, Middle Aged, respiratory system, medicine.disease, Survival Analysis, Pulmonary hypertension, Combined pulmonary fibrosis and emphysema, Respiratory Function Tests, respiratory tract diseases, 3. Good health, Surgery, PULMONARY FIBROSIS, medicine.anatomical_structure, Pulmonary Emphysema, 030228 respiratory system, Cardiology, Female, Tomography, X-Ray Computed, business
Abstract

The syndrome resulting from combined pulmonary fibrosis and emphysema has not been comprehensively described. The current authors conducted a retrospective study of 61 patients with both emphysema of the upper zones and diffuse parenchymal lung disease with fibrosis of the lower zones of the lungs on chest computed tomography. Patients (all smokers) included 60 males and one female, with a mean age of 65 yrs. Dyspnoea on exertion was present in all patients. Basal crackles were found in 87% and finger clubbing in 43%. Pulmonary function tests were as follows (mean ± SD): total lung capacity 88% ± 17, forced vital capacity (FVC) 88% ± 18, forced expiratory volume in one second (FEV1) 80% ± 21 (% predicted), FEV1/FVC 69% ± 13, carbon monoxide diffusion capacity of the lung 37% ± 16 (% predicted), carbon monoxide transfer coefficient 46% ± 19. Pulmonary hypertension was present in 47% of patients at diagnosis, and 55% during follow-up. Patients were followed for a mean of 2.1 ± 2.8 yrs from diagnosis. Survival was 87.5% at 2 yrs and 54.6% at 5 yrs, with a median of 6.1 yrs. The presence of pulmonary hypertension at diagnosis was a critical determinant of prognosis. The authors hereby individualise the computer tomography-defined syndrome of combined pulmonary fibrosis and emphysema characterised by subnormal spirometry, severe impairment of gas exchange, high prevalence of pulmonary hypertension, and poor survival. Copyright©ERS Journals Ltd 2005.

Published
2005

12. Orphan lung diseases from definition to organisation of care

Author

Jean-François Cordier, Vincent Cottin, Infections Virales et Pathologie Comparée - UMR 754 (IVPC), École pratique des hautes études (EPHE)-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), Cottin, V. and Cordier, J. F. and Richeldi, L., Chautard, Marie-Gabrielle, Cottin, V. and Cordier, J. F. and Richeldi, L., Rétrovirus et Pathologie Comparée (RPC), Institut National de la Recherche Agronomique (INRA)-École pratique des hautes études (EPHE), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Université Claude Bernard Lyon 1 (UCBL), and Université de Lyon-Université de Lyon-Ecole Nationale Vétérinaire de Lyon (ENVL)

Subjects
medicine.medical_specialty, Pediatrics, Lung, business.industry, [SDV]Life Sciences [q-bio], Disease, 3. Good health, [SDV] Life Sciences [q-bio], 03 medical and health sciences, 0302 clinical medicine, medicine.anatomical_structure, Clinical research, 030228 respiratory system, Drug development, Epidemiology, medicine, 030212 general & internal medicine, Intensive care medicine, business, ComputingMilieux_MISCELLANEOUS
Abstract

The rare diseases have been called “orphan” because this term means that patients affected by such conditions feel that most physicians are reluctant to care for their disease because of its rarity, and that insufficient research is developed to improve diagnosis, clinical management, and especially the more distressing manifestations. The patients with rare diseases thus feel abandoned and orphan when comparing with patients with common diseases who benefit from extensive research worldwide and continuous drug development.

Published
2015

13. Smoking-related interstitial lung disease

Author

Fabrizio Luppi, Stefania Cerri, Luca Richeldi, Paolo Spagnolo, Cordier, J-F, Cerri, S, Spagnolo, P, Luppi, F, and Richeldi, L

Subjects
interstitial lung disease, medicine.medical_specialty, Lung, business.industry, Interstitial lung disease, Cigarette smoke, respiratory system, medicine.disease, Desquamative interstitial pneumonia, Combined pulmonary fibrosis and emphysema, Pulmonary hypertension, smoking, lung, respiratory tract diseases, Lung Disorder, Histiocytosis, emphysema, medicine.anatomical_structure, Internal medicine, Epidemiology, medicine, interstitial fibrosi, business
Abstract

Cigarette smoking has a clear epidemiological association with lung diseases, characterised by chronic inflammation of both the bronchiolar and the interstitial lung compartments. There are several different smoking-related interstitial lung diseases, mainly desquamative interstitial pneumonia, respiratory bronchiolitis-associated interstitial lung disease and pulmonary Langerhans' cell histiocytosis. The epidemiology of such diseases is largely unknown, although the prevalence of cigarette smoking, particularly in low-income developing countries, indicates that smoking-induced interstitial lung disorders represent a high burden of disease worldwide. The role of chest high-resolution computed tomography has become increasingly important in differential diagnosis and follow-up. A new entity, the syndrome of combined pulmonary fibrosis and emphysema, emerged as another important smoking-related lung disorder with a poor prognosis, associated with the high prevalence of pulmonary hypertension. At the moment the role of anti-inflammatory and immunosuppressive treatment remains unclear, although in clinical practice most of these patients will receive at least one course of corticosteroid therapy. It is vital to stress the importance of identifying these patients and helping them quit smoking.

Published
2011

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