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2. Role of bronchoscopy for respiratory involvement in granulomatosis with polyangiitis and microscopic polyangiitis

Author

Thomas Villeneuve, Grégoire Prévot, Grégory Pugnet, Gavin Plat, Valentin Héluain, Stanislas Faguer, and Nicolas Guibert

Subjects
Medicine
Abstract

Objectives This study describes data from bronchoscopy performed at the diagnosis of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Methods We conducted a retrospective study between 2004 and 2019 in patients aged >18 years with a diagnosis of microscopic polyangiitis (MPA) or granulomatosis with polyangiitis (GPA) who underwent bronchoscopy at onset of the disease. We collected bronchoalveolar lavage (BAL) and histological findings obtained during bronchoscopy. Results 274 patients with AAV were identified. Among 92 bronchoscopies, 62 were performed at diagnosis, and 58 procedures were finally analysed. Cough was more frequent in patients with MPA than GPA (p=0.02). The presence of endobronchial lesions (24.1%) was found to be significantly associated with GPA (p

Published
2023
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3. Sine scleroderma, limited cutaneous, and diffused cutaneous systemic sclerosis survival and predictors of mortality

Author

Sébastien De Almeida Chaves, Tiphaine Porel, Mickael Mounié, Laurent Alric, Léonardo Astudillo, Antoine Huart, Olivier Lairez, Martin Michaud, Grégoire Prévot, David Ribes, Laurent Sailler, Francis Gaches, Daniel Adoue, and Gregory Pugnet

Subjects
Autoimmune diseases, Systemic sclerosis, Mortality, Prognostic factors, Diseases of the musculoskeletal system, RC925-935
Abstract

Highlights • Male sex, cardiac involvement, DLCO 5 mg/l are strong predictors of mortality in systemic sclerosis. • This study shows the survival of subtypes and in particular sine scleroderma. • Sine scleroderma subtype has better survival than diffuse or limited cutaneous subtypes. • Non-systemic sclerosis-related deaths are more frequent than systemic sclerosis-related deaths. • Cardiovascular events non-systemic sclerosis-related are the main deaths.

Published
2021
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6. Balloon pulmonary angioplasty versus riociguat for the treatment of inoperable chronic thromboembolic pulmonary hypertension (RACE): a multicentre, phase 3, open-label, randomised controlled trial and ancillary follow-up study

Author

Xavier Jaïs, Philippe Brenot, Hélène Bouvaist, Mitja Jevnikar, Matthieu Canuet, Céline Chabanne, Ari Chaouat, Vincent Cottin, Pascal De Groote, Nicolas Favrolt, Delphine Horeau-Langlard, Pascal Magro, Laurent Savale, Grégoire Prévot, Sébastien Renard, Olivier Sitbon, Florence Parent, Romain Trésorier, Cécile Tromeur, Céline Piedvache, Lamiae Grimaldi, Elie Fadel, David Montani, Marc Humbert, and Gérald Simonneau

Subjects
Pulmonary and Respiratory Medicine
Published
2022

7. French recommendations for the diagnosis and management of lymphangioleiomyomatosis

Author

Vincent Cottin, Elodie Blanchard, Mallorie Kerjouan, Romain Lazor, Martine Reynaud-Gaubert, Camille Taille, Yurdagül Uzunhan, Lidwine Wemeau, Claire Andrejak, Dany Baud, Philippe Bonniaud, Pierre-Yves Brillet, Alain Calender, Lara Chalabreysse, Isabelle Court-Fortune, Nicolas Pierre Desbaillets, Gilbert Ferretti, Anne Guillemot, Laurane Hardelin, Marianne Kambouchner, Violette Leclerc, Mathieu Lederlin, Marie-Claire Malinge, Alain Mancel, Sylvain Marchand-Adam, Jean-Michel Maury, Jean-Marc Naccache, Mouhamad Nasser, Hilario Nunes, Gaële Pagnoux, Grégoire Prévot, Christine Rousset-Jablonski, Olivier Rouviere, Salim Si-Mohamed, Renaud Touraine, Julie Traclet, Ségolène Turquier, Stéphane Vagnarelli, Kaïs Ahmad, Infections Virales et Pathologie Comparée - UMR 754 (IVPC), École Pratique des Hautes Études (EPHE), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE), Centre de recherche Cardio-Thoracique de Bordeaux [Bordeaux] (CRCTB), Université Bordeaux Segalen - Bordeaux 2-CHU Bordeaux [Bordeaux]-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Pontchaillou [Rennes], Centre Hospitalier Universitaire Vaudois [Lausanne] (CHUV), Assistance Publique - Hôpitaux de Marseille (APHM), Microbes évolution phylogénie et infections (MEPHI), Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS), AP-HP - Hôpital Bichat - Claude Bernard [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Physiopathologie et Epidémiologie des Maladies Respiratoires (PHERE (UMR_S_1152 / U1152)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité), Plateforme F-CRIN, Université Toulouse III - Paul Sabatier (UT3), Université de Toulouse (UT)-Université de Toulouse (UT)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Institut National de la Santé et de la Recherche Médicale (INSERM), Hypoxie et Poumon : pneumopathologies fibrosantes, modulations ventilatoires et circulatoires (H&P), UFR SMBH-Université Sorbonne Paris Nord, Hôpital Avicenne [AP-HP], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), CHU Amiens-Picardie, Agents infectieux, résistance et chimiothérapie - UR UPJV 4294 (AGIR ), Université de Picardie Jules Verne (UPJV)-CHU Amiens-Picardie, EA3920 University of Burgundy Franche-Comté Besançon, Equipe CADIR (LNC - U1231), Lipides - Nutrition - Cancer [Dijon - U1231] (LNC), Université de Bourgogne (UB)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut Agro Dijon, Institut national d'enseignement supérieur pour l'agriculture, l'alimentation et l'environnement (Institut Agro)-Institut national d'enseignement supérieur pour l'agriculture, l'alimentation et l'environnement (Institut Agro)-Université de Bourgogne (UB)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut Agro Dijon, Institut national d'enseignement supérieur pour l'agriculture, l'alimentation et l'environnement (Institut Agro)-Institut national d'enseignement supérieur pour l'agriculture, l'alimentation et l'environnement (Institut Agro), Laboratoire de Biologie Tissulaire et d'ingénierie Thérapeutique UMR 5305 (LBTI), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Centre National de la Recherche Scientifique (CNRS), Hôpital Louis Pradel [CHU - HCL], Hospices Civils de Lyon (HCL), Centre pour l'innovation en cancérologie de Lyon (CICLY), Université de Lyon-Université de Lyon, Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E), Institute for Advanced Biosciences / Institut pour l'Avancée des Biosciences (Grenoble) (IAB), Centre Hospitalier Universitaire [Grenoble] (CHU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Etablissement français du sang - Auvergne-Rhône-Alpes (EFS)-Centre National de la Recherche Scientifique (CNRS)-Université Grenoble Alpes (UGA), and Centre Hospitalier Universitaire [Grenoble] (CHU)

Subjects
Pulmonary and Respiratory Medicine, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
Abstract

International audience; BackgroundThe present article is an English-language version of the French National Diagnostic and Care Protocol, a pragmatic tool to optimize and harmonize the diagnosis, care pathway, management and follow-up of lymphangioleiomyomatosis in France.MethodsPractical recommendations were developed in accordance with the method for developing a National Diagnosis and Care Protocol for rare diseases of the Haute Autorité de Santé and following international guidelines and literature on lymphangioleiomyomatosis. It was developed by a multidisciplinary group, with the help of patient representatives and of RespiFIL, the rare disease network on respiratory diseases.ResultsLymphangioleiomyomatosis is a rare lung disease characterised by a proliferation of smooth muscle cells that leads to the formation of multiple lung cysts. It occurs sporadically or as part of a genetic disease called tuberous sclerosis complex (TSC). The document addresses multiple aspects of the disease, to guide the clinicians regarding when to suspect a diagnosis of lymphangioleiomyomatosis, what to do in case of recurrent pneumothorax or angiomyolipomas, what investigations are needed to make the diagnosis of lymphangioleiomyomatosis, what the diagnostic criteria are for lymphangioleiomyomatosis, what the principles of management are, and how follow-up can be organised. Recommendations are made regarding the use of pharmaceutical specialties and treatment other than medications.ConclusionThese recommendations are intended to guide the diagnosis and practical management of pulmonary lymphangioleiomyomatosis.

Published
2023

8. Management of pulmonary toxicity associated with immune checkpoint inhibitors

Author

Myriam Delaunay, Grégoire Prévot, Samia Collot, Laurent Guilleminault, Alain Didier, and Julien Mazières

Subjects
Diseases of the respiratory system, RC705-779
Abstract

Immunotherapy has become a standard of care in oncology, following the recent approvals of cytotoxic T-lymphocyte-associated protein-4 and programmed cell death-1 inhibitors in lung cancer, melanoma, renal cell carcinoma, Hodgkin's lymphoma, bladder, head and neck cancers. Besides their efficacy, these agents also generate specific immune-related adverse events. Due to the increasing prescription of immune-checkpoint inhibitors, the incidence of immune toxicity will continue to rise. The awareness of immune-related adverse events is key to ensuring both diagnosis and management of the possible serious adverse events. Although severe immune-related adverse events remain rare, they can lead to discontinued treatment or to death if they are not forecasted and managed properly. Even if lung toxicity is not the most frequent adverse event, it remains critical as it can be life-threatening. Herein, the main aspects of pulmonary toxicity are reviewed and guidelines are also proposed in order to manage the possible side-effects.

Published
2019
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9. Serum and Pulmonary Expression Profiles of the Activin Signaling System in Pulmonary Arterial Hypertension

Author

Christophe Guignabert, Laurent Savale, Athénaïs Boucly, Raphaël Thuillet, Ly Tu, Mina Ottaviani, Christopher J. Rhodes, Pascal De Groote, Grégoire Prévot, Emmanuel Bergot, Arnaud Bourdin, Luke S. Howard, Elie Fadel, Antoine Beurnier, Anne Roche, Mitja Jevnikar, Xavier Jaïs, David Montani, Martin R. Wilkins, Olivier Sitbon, and Marc Humbert

Subjects
Physiology (medical), Cardiology and Cardiovascular Medicine
Abstract

BACKGROUND: Activins are novel therapeutic targets in pulmonary arterial hypertension (PAH). We therefore studied whether key members of the activin pathway could be used as PAH biomarkers. METHODS: Serum levels of activin A, activin B, α-subunit of inhibin A and B proteins, and the antagonists follistatin and follistatin-like 3 (FSTL3) were measured in controls and in patients with newly diagnosed idiopathic, heritable, or anorexigen-associated PAH (n=80) at baseline and 3 to 4 months after treatment initiation. The primary outcome was death or lung transplantation. Expression patterns of the inhibin subunits, follistatin, FSTL3, Bambi, Cripto, and the activin receptors type I (ALK), type II (ACTRII), and betaglycan were analyzed in PAH and control lung tissues. RESULTS: Death or lung transplantation occurred in 26 of 80 patients (32.5%) over a median follow-up of 69 (interquartile range, 50–81) months. Both baseline (hazard ratio, 1.001 [95% CI, 1.000–1.001]; P =0.037 and 1.263 [95% CI, 1.049–1.520]; P =0.014, respectively) and follow-up (hazard ratio, 1.003 [95% CI, 1.001–1.005]; P =0.001 and 1.365 [95% CI, 1.185–1.573]; P P =0.009) and 0.17 (95% CI, 0.06–0.45; P P =0.019) and 0.27 (95% CI, 0.09–0.78, P =0.015), respectively. Prognostic values of activin A and FSTL3 were confirmed in an independent external validation cohort. Histological analyses showed a nuclear accumulation of the phosphorylated form of Smad2/3, higher immunoreactivities for ACTRIIB, ALK2, ALK4, ALK5, ALK7, Cripto, and FSTL3 in vascular endothelial and smooth muscle layers, and lower immunostaining for inhibin-α and follistatin. CONCLUSIONS: These findings offer new insights into the activin signaling system in PAH and show that activin A and FSTL3 are prognostic biomarkers for PAH.

Published
2023

10. 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

12. Sine scleroderma, limited cutaneous, and diffused cutaneous systemic sclerosis survival and predictors of mortality

Author

Antoine Huart, Leonardo Astudillo, Sébastien De Almeida Chaves, Grégoire Prévot, Daniel Adoue, David Ribes, Laurent Alric, T. Porel, Laurent Sailler, Mickael Mounié, Grégory Pugnet, Francis Gaches, Martin Michaud, and Olivier Lairez

Subjects
Male, medicine.medical_specialty, Scleroderma, Systemic, business.industry, Hypertension, Pulmonary, Autoimmune diseases, Diseases of the musculoskeletal system, Prognosis, medicine.disease, Prognostic factors, Dermatology, Scleroderma, RC925-935, Scleroderma, Limited, Scleroderma, Diffuse, medicine, Humans, Systemic sclerosis, Mortality, Lung Diseases, Interstitial, business, Research Article
Abstract

Background Systemic sclerosis (SSc) is associated with a variability of mortality rates in the literature. Objective To determine the mortality and its predictors in a long-term follow-up of a bi-centric cohort of SSc patients. Methods A retrospective observational study by systematically analyzing the medical records of patients diagnosed with SSc in Toulouse University Hospital and Ducuing Hospital. Standardized Mortality Ratio (SMR), mortality at 1, 3, 5, 10, and 15 years of disease and causes of death were described. Predictors of mortality using Cox regression were assessed. Results Three hundred seventy-five patients were included: 63 with diffuse cutaneous SSc, 279 with limited cutaneous SSc, and 33 with sine scleroderma. The SMR ratio was 1.88 (95% CI 1.46–1.97). The overall survival rates were 97.6% at 1 year, 93.4% at 3 years, 87.1% at 5 years, 77.9% at 10 years, and 61.3% at 15 years. Sixty-nine deaths were recorded. 46.4% were SSc related deaths secondary to interstitial lung disease (ILD) (34.4%), pulmonary hypertension (31.2%), and digestive tract involvement (18.8%). 53.6% were non-related to SSc: cardiovascular disorders (37.8%) and various infections (35.1%) largely distanced those from cancer (13.5%). Four significant independent predictive factors were identified: carbon monoxide diffusing capacity (DLCO) < 70% (HR=3.01; p=0.0053), C-reactive protein (CRP) >5 mg/l (HR=2.13; p=0.0174), cardiac involvement (HR=2.86; p=0.0012), and the fact of being male (HR=3.25; p=0.0004). Conclusion Long-term data confirmed high mortality of SSc. Male sex, DLCO 5mg/l were identified as independent predictors of mortality. Supplementary Information The online version contains supplementary material available at 10.1186/s13075-021-02672-y., • Male sex, cardiac involvement, DLCO 5 mg/l are strong predictors of mortality in systemic sclerosis. • This study shows the survival of subtypes and in particular sine scleroderma. • Sine scleroderma subtype has better survival than diffuse or limited cutaneous subtypes. • Non-systemic sclerosis-related deaths are more frequent than systemic sclerosis-related deaths. • Cardiovascular events non-systemic sclerosis-related are the main deaths. Supplementary Information The online version contains supplementary material available at 10.1186/s13075-021-02672-y.

Published
2021

13. Association between Initial Treatment Strategy and Long-Term Survival in Pulmonary Arterial Hypertension

Author

Vincent Cottin, David Montani, Jérémie Pichon, Martine Reynaud-Gaubert, Xavier Jaïs, Pascal Magro, Gérald Simonneau, Florence Parent, Fabrice Bauer, Marianne Riou, Laurent Bertoletti, Pamela Moceri, Ari Chaouat, Andrei Seferian, Antoine Beurnier, Sébastien Renard, Pierre Mauran, Delphine Horeau-Langlard, Pascal de Groote, Laurent Savale, Mitja Jevnikar, Sophie Bulifon, Pascal Roblot, Hélène Bouvaist, Yuanchao Feng, Patrice Poubeau, Sylvain Palat, Zhiying Liang, Emmanuel Bergot, François Picard, Etienne-Marie Jutant, C. Chabanne, Olivier Sitbon, Athénaïs Boucly, Grégoire Prévot, Jean-François Mornex, Cécile Tromeur, Marc Humbert, Bruno Degano, Claire Dauphin, Arnaud Bourdin, Olivier Sanchez, Nicolas Favrolt, Jason Weatherald, Hypertension arterielle pulmonaire physiopathologie et innovation thérapeutique, Centre chirurgical Marie Lannelongue-Institut National de la Santé et de la Recherche Médicale (INSERM), Université Paris-Saclay, AP-HP Hôpital Bicêtre (Le Kremlin-Bicêtre), Endothélium, valvulopathies et insuffisance cardiaque (EnVI), Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Rouen, Normandie Université (NU), CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN), Université de Caen Normandie (UNICAEN), Biologie intégrative du tissu osseux, Université Jean Monnet [Saint-Étienne] (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre Hospitalier Universitaire de Saint-Etienne (CHU de Saint-Etienne), Physiologie & médecine expérimentale du Cœur et des Muscles [U 1046] (PhyMedExp), Université de Montpellier (UM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Centre Hospitalier Universitaire [Grenoble] (CHU), Laboratoire Traitement du Signal et de l'Image (LTSI), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES), CHU Pontchaillou [Rennes], Défaillance Cardiovasculaire Aiguë et Chronique (DCAC), Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lorraine (UL), Service de Pneumologie [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), 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), CHU Gabriel Montpied [Clermont-Ferrand], CHU Clermont-Ferrand, Facteurs de Risque et Déterminants Moléculaires des Maladies liées au Vieillissement - U 1167 (RID-AGE), 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 Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), University of Calgary, Centre hospitalier universitaire de Nantes (CHU Nantes), Hôpital Nord Laennec [CHU Nantes], CHU Trousseau [Tours], Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), Centre Hospitalier Universitaire de Reims (CHU Reims), American Memorial Hospital (Hôpital des enfants) [Reims], Centre Hospitalier Universitaire de Nice (CHU Nice), Université Côte d'Azur (UCA), Hôpital Dupuytren [CHU Limoges], Hôpital Haut-Lévêque [CHU Bordeaux], CHU Bordeaux [Bordeaux], Université de Bordeaux (UB), Centre Hospitalier Universitaire de La Réunion (CHU La Réunion), Hôpital Larrey [Toulouse], CHU Toulouse [Toulouse], Hôpital de la Timone [CHU - APHM] (TIMONE), Assistance Publique - Hôpitaux de Marseille (APHM), Aix Marseille Université (AMU), Nouvel Hôpital Civil de Strasbourg, Centre hospitalier universitaire de Poitiers (CHU Poitiers), 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), CIC Brest, Université de Brest (UBO)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Hôpital de la Cavale Blanche, Centre Chirurgical Marie Lannelongue (CCML)-Institut National de la Santé et de la Recherche Médicale (INSERM), Biologie Intégrative du Tissu Osseux (LBTO), Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM)-Centre National de la Recherche Scientifique (CNRS), Université de Rennes (UR)-Institut National de la Santé et de la Recherche Médicale (INSERM), École Pratique des Hautes Études (EPHE), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre chirurgical Marie Lannelongue, 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), and MORNET, Dominique

Subjects
Pulmonary and Respiratory Medicine, medicine.medical_specialty, business.industry, [SDV]Life Sciences [q-bio], Critical Care and Intensive Care Medicine, medicine.disease, survival, Pulmonary hypertension, 3. Good health, [SDV] Life Sciences [q-bio], pulmonary arterial hypertension, Internal medicine, pulmonary hypertension, Long term survival, therapeutics, Cardiology, Medicine, Initial treatment, business
Abstract

International audience; Rationale: The relationship between the initial treatment strategy and survival in pulmonary arterial hypertension (PAH) remains uncertain. Objectives: To evaluate the long-term survival of patients with PAH categorized according to the initial treatment strategy. Methods: A retrospective analysis of incident patients with idiopathic, heritable, or anorexigen-induced PAH enrolled in the French Pulmonary Hypertension Registry (January 2006 to December 2018) was conducted. Survival was assessed according to the initial strategy: monotherapy, dual therapy, or triple-combination therapy (two oral medications and a parenteral prostacyclin). Measurements and Main Results: Among 1,611 enrolled patients, 984 were initiated on monotherapy, 551 were initiated on dual therapy, and 76 were initiated on triple therapy. The triple-combination group was younger and had fewer comorbidities but had a higher mortality risk. The survival rate was higher with the use of triple therapy (91% at 5 yr) as compared with dual therapy or monotherapy (both 61% at 5 yr) (P < 0.001). Propensity score matching of age, sex, and pulmonary vascular resistance also showed significant differences between triple therapy and dual therapy (10-yr survival, 85% vs. 65%). In high-risk patients (n = 243), the survival rate was higher with triple therapy than with monotherapy or dual therapy, whereas there was no difference between monotherapy and double therapy. In intermediate-risk patients (n = 1,134), survival improved with an increasing number of therapies. In multivariable Cox regression, triple therapy was independently associated with a lower risk of death (hazard ratio, 0.29; 95% confidence interval, 0.11-0.80; P = 0.017). Among the 148 patients initiated on a parenteral prostacyclin, those on triple therapy had a higher survival rate than those on monotherapy or dual therapy. Conclusions: Initial triple-combination therapy that includes parenteral prostacyclin seems to be associated with a higher survival rate in PAH, particularly in the youngest high-risk patients.

Published
2021

14. Severe pulmonary hypertension associated with chronic obstructive pulmonary disease: A prospective French multicenter cohort

Author

Cédric Laouénan, Gérald Simonneau, Bruno Degano, David Montani, Vincent Cottin, Clément Boissin, Martine Reynaud-Gaubert, Yolande Costa de Beauregard, Olivier Sitbon, Marc Humbert, Drifa Belhadi, Amina Bencherif, Emmanuel Bergot, Hervé Mal, Ari Chaouat, Olivier Sanchez, Mathieu Canuet, Grégoire Prévot, Cécile Tromeur, G. Dauriat, Bouchra Lamia, and Gabriel Thabut

Subjects
Male, Pulmonary and Respiratory Medicine, medicine.medical_specialty, Exacerbation, Hypertension, Pulmonary, 030204 cardiovascular system & hematology, Pulmonary function testing, Pulmonary Disease, Chronic Obstructive, 03 medical and health sciences, 0302 clinical medicine, Interquartile range, medicine.artery, Internal medicine, medicine, Humans, Prospective Studies, Pulmonary Wedge Pressure, Pulmonary wedge pressure, Aged, Transplantation, COPD, business.industry, Incidence, Middle Aged, medicine.disease, Pulmonary hypertension, Respiratory Function Tests, medicine.anatomical_structure, 030228 respiratory system, Pulmonary artery, Cardiology, Vascular resistance, Female, Vascular Resistance, Surgery, France, Cardiology and Cardiovascular Medicine, business, Follow-Up Studies
Abstract

Background A small proportion of patients with chronic obstructive pulmonary disease (COPD) patients present severe pulmonary hypertension (PH), defined by mean pulmonary artery pressure (mPAP) ≥35 mm Hg measured by right heart catheterization. Little is known about the characteristics of severe PH-COPD. The aim of the study based on a national registry was to describe this phenotype. Methods We prospectively included and followed patients with incident PH-COPD. Clinical, functional, hemodynamic data at inclusion and follow-up were retrieved. Survival assessed by Kaplan-Meier analysis was the primary end-point. Results From 2012 to 2016, 99 patients from 13 French centers were included in the study (82 males; median age 66.0 years [interquartile range 62.0-72.0]). At inclusion, most patients had marked dyspnea (55.6% and 22.2% New York Heart Association class III and IV, respectively). During 12 months before inclusion, 42.9% had an exacerbation requiring a hospitalization. Pulmonary function tests showed a moderate obstructive pattern with median (interquartile range) FEV1 50.0 [35.0-63.0] % predicted and low diffusing capacity for carbon monoxide, median 20.0 [16.5-30.6] % predicted. The median values for PaO2 and PaCO2 on room air were 50.0 [44.8-62.0] and 36.0 [31.1-43.0] mm Hg. Median values of mPAP, pulmonary artery occlusion pressure, cardiac index and pulmonary vascular resistance were 42.0 [37.0-48.0] mm Hg, 11.0 [9.0-14.0] mm Hg, 3.0 [2.4-3.6] L/min/m2, and 6.3 [4.2-7.9] WU, respectively. Mean restricted survival was 15.0 [13.9-16.0] months. Conclusions Severe PH-COPD is characterized by moderate airway obstruction but marked dyspnea and marked hypoxemia, low DLCO and high mPAP. This phenotype is associated with poor prognosis.

Published
2021

15. 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

17. 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

20. Diagnosis and management of idiopathic pulmonary fibrosis: French practical guidelines

Author

Bruno Crestani, Dominique Valeyre, Benoit Wallaert, Jacques Cadranel, Jean-Charles Dalphin, Philippe Delaval, Dominique Israel-Biet, Romain Kessler, Martine Reynaud-Gaubert, Bernard Aguilaniu, Benoit Bouquillon, Philippe Carré, Claire Danel, Jean-Baptiste Faivre, Gilbert Ferretti, Nicolas Just, Serge Kouzan, François Lebargy, Sylvain Marchand-Adam, Bruno Philippe, Grégoire Prévot, Bruno Stach, Françoise Thivolet-Béjui, and Jean-François Cordier

Subjects
Diseases of the respiratory system, RC705-779
Abstract

Idiopathic pulmonary fibrosis (IPF) is the most frequent chronic idiopathic interstitial pneumonia in adults. The management of rare diseases in France has been organised by a national plan for rare diseases, which endorsed a network of expert centres for rare diseases throughout France. This article is an overview of the executive summary of the French guidelines for the management of IPF, an initiative that emanated from the French National Reference Centre and the Network of Regional Competence Centres for Rare Lung Diseases. This review aims at providing pulmonologists with a document that: 1) combines the current available evidence; 2) reviews practical modalities of diagnosis and management of IPF; and 3) is adapted to everyday medical practice. The French practical guidelines result from the combined efforts of a coordination committee, a writing committee and a multidisciplinary review panel, following recommendations from the Haute Autorité de Santé. All recommendations included in this article received at least 90% agreement by the reviewing panel. Herein, we summarise the main conclusions and practical recommendations of the French guidelines.

Published
2014
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21. Role of C5 inhibition in Idiopathic Inflammatory Myopathies and Scleroderma Renal Crisis–Induced Thrombotic Microangiopathies

Author

Grégoire Prévot, Antoine Huart, Stanislas Faguer, David Ribes, Véronique Frémeaux-Bacchi, Grégory Pugnet, Olivier Lairez, Julie Belliere, Anna Gouin, Magali Colombat, and Dominique Chauveau

Subjects
Pathology, medicine.medical_specialty, Thrombotic microangiopathy, C5b9, Scleroderma Renal Crisis, 030232 urology & nephrology, connective disease, 030204 cardiovascular system & hematology, lcsh:RC870-923, urologic and male genital diseases, Inflammatory myopathy, 03 medical and health sciences, 0302 clinical medicine, Clinical Research, Edema, Biopsy, medicine, Complement component 5, Kidney, medicine.diagnostic_test, business.industry, Eculizumab, medicine.disease, lcsh:Diseases of the genitourinary system. Urology, C5 blocking, thrombotic microangiopathy, medicine.anatomical_structure, Nephrology, medicine.symptom, business, medicine.drug
Abstract

Introduction Connective tissue diseases, including systemic sclerosis and idiopathic inflammatory myopathies (IIMs), are a very rare cause of thrombotic microangiopathies (TMAs). Whether dysregulation of the complement pathways underlies these secondary forms of TMA and may be targeted by complement blocking agents remains elusive. Methods Kidney pathology and outcomes of 18 critically ill patients with TMA related to inflammatory myopathy flare-up (IIM, n=7) or scleroderma renal crisis (SRC, n=11; biopsy n=9) are assessed. Results IIM-TMA is characterized by acute thrombotic lesions only, whereas SRC-TMA patients also harbored chronic vascular lesions and more interstitial fibrosis. C5b9 deposits, a marker of complement component 5 (C5) cleavage, were observed in the 2 subgroups at the junction of media and intima of arterioles, colocalizing with subendothelial edema. Thus, kidney biopsy distinguished between acute and chronic renal phenotypes that may help to individualize treatment. Treatment of IIM-TMA patients with combined full-code organ support, corticosteroids, B-cell depletion, and complement C5 blocking led to 1-year survival of 72%, compared with 19% in historical cohorts. Treatment of SRC-TMA was more heterogenous and relied on conversion enzyme inhibitor only or with eculizumab (n=6) and immunosuppressor (n=5). One-year survival of SRC-TMA patients was 52%, a result similar to historical cohorts. Eculizumab was followed by a rapid dramatic improvement of TMA in all the treated patients. Conclusion C5 blocking may reverse hematologic abnormalities in IIM- and SRC-TMA, and adding an early and aggressive immunosuppressive regimen may improve the survival of IIM-TMA. Underlying chronic vascular and interstitial lesions mitigate renal response in SRC-TMA., Graphical abstract

Published
2021

22. Portopulmonary hypertension in the current era of pulmonary hypertension management

Author

François Durand, Laurent Savale, Clément Boissin, Olivier Sitbon, Claire Francoz, Hélène Bouvaist, Jean-Charles Duclos-Vallée, Ari Chaouat, Manuel Guimas, Pamela Moceri, Emmanuel Bergot, Pascal Magro, David Montani, Nicolas Lamblin, Vincent Cottin, Marianne Riou, Filomena Conti, Marc Humbert, Nicolas Favrolt, Romain Trésorier, Xavier Jaïs, Sébastien Renard, Philippe Hervé, Grégoire Prévot, Cécile Tromeur, Bruno Degano, Céline Chabanne, Delphine Bourlier, Nathan Ebstein, Sylvain Palat, Didier Samuel, Gérald Simonneau, Elise Artaud-Macari, Delphine Horeau-Langlard, Pascal de Groote, Anne-Claire Simon, Mitja Jevnikar, Audrey Coilly, and Marie Fertin

Subjects
0301 basic medicine, medicine.medical_specialty, Portopulmonary hypertension, Cirrhosis, Hepatology, business.industry, medicine.medical_treatment, Liver transplantation, Chronic liver disease, medicine.disease, Pulmonary hypertension, Transplantation, 03 medical and health sciences, Liver disease, 030104 developmental biology, 0302 clinical medicine, Internal medicine, medicine, 030211 gastroenterology & hepatology, business, Contraindication
Abstract

Background & Aims Long-term outcomes in portopulmonary hypertension (PoPH) are poorly studied in the current era of pulmonary hypertension management. We analysed the effect of pulmonary arterial hypertension (PAH)-targeted therapies, survival and predictors of death in a large contemporary cohort of patients with PoPH. Methods Data from patients with PoPH consecutively enrolled in the French Pulmonary Hypertension Registry between 2007 and 2017 were collected. The effect of initial treatment strategies on functional class, exercise capacity and cardiopulmonary haemodynamics were analysed. Survival and its association with PAH- and hepatic-related characteristics were also examined. Results Six hundred and thirty-seven patients (mean age 55 ± 10 years; 58% male) were included. Fifty-seven percent had mild cirrhosis, i.e. Child-Pugh stage A. The median model for end-stage liver disease (MELD) score was 11 (IQR 9–15). Most patients (n = 474; 74%) were initiated on monotherapy, either with a phosphodiesterase-5 inhibitor (n = 336) or with an endothelin-receptor antagonist (n = 128); 95 (15%) were initiated on double oral combination therapy and 5 (1%) on triple therapy. After a median treatment time of 4.5 months, there were significant improvements in functional class (p Conclusion Survival of patients with PoPH is strongly associated with the severity of liver disease. Patients who underwent liver transplantation had the best long-term outcomes. Lay summary Portopulmonary hypertension is defined by the presence of pulmonary arterial hypertension in the context of chronic liver disease and is characterized by progressive shortness of breath and exercise limitation. The presence of severe pulmonary arterial hypertension in liver transplant candidates represents a contraindication for such a surgery; however, treatments targeting pulmonary arterial hypertension are efficacious, allowing for safe transplantation and conferring good survival outcomes in those who undergo liver transplantation.

Published
2020

23. Lung Involvement in Destombes-Rosai-Dorfman Disease

Author

Jean-François Emile, Samia Boussouar, Q. Moyon, Fleur Cohen Aubart, Grégoire Prévot, Philippe Grenier, Nathalie Aladjidi, Philippe Maksud, Julien Haroche, Frédéric Charlotte, Zahir Amoura, and Jean Donadieu

Subjects
Pulmonary and Respiratory Medicine, Cobimetinib, Pathology, medicine.medical_specialty, business.industry, MEK inhibitor, Standardized uptake value, Disease, Critical Care and Intensive Care Medicine, medicine.disease, 3. Good health, 03 medical and health sciences, Histiocytosis, chemistry.chemical_compound, 0302 clinical medicine, 030228 respiratory system, chemistry, Pulmonary fibrosis, Medicine, 030212 general & internal medicine, Cardiology and Cardiovascular Medicine, business, Interstitial Disease, Rosai–Dorfman disease
Abstract

Poster: "ESTI 2019 / P-0109 / Lung involvement in Destombes-Rosai-Dorfman disease: clinical and radiological features, and response to the MEK inhibitor cobimetinib " by: "Q. moyon, S. Boussouar , F. cohen aubart, P. maksud, N. Aladjidi, G. Prevot, J. donadieu, Z. amoura, P. A. Grenier, J. haroche; Paris/FR"

Published
2020

24. Cytokines as prognostic biomarkers in pulmonary arterial hypertension

Author

Athénaïs Boucly, Ly Tu, Christophe Guignabert, Christopher Rhodes, Pascal De Groote, Grégoire Prévot, Emmanuel Bergot, Arnaud Bourdin, Antoine Beurnier, Anne Roche, Mitja Jevnikar, Xavier Jaïs, David Montani, Martin R. Wilkins, Marc Humbert, Olivier Sitbon, and Laurent Savale

Subjects
Pulmonary and Respiratory Medicine
Abstract

BackgroundRisk stratification and assessment of disease progression in patients with pulmonary arterial hypertension (PAH) are challenged by the lack of accurate disease-specific and prognostic biomarkers. To date, brain natriuretic peptide (BNP) and/or its N-terminal fragment (NT-proBNP) are the only markers for right ventricular dysfunction used in clinical practice, in association with echocardiographic and invasive haemodynamic variables to predict outcome in patients with PAH.MethodsThis study was designed to identify an easily measurable biomarker panel in the serum of 80 well-phenotyped PAH patients with idiopathic, heritable or drug-induced PAH at baseline and at first follow-up. The prognostic value of identified cytokines of interest was secondly analysed in an external validation cohort of 125 PAH patients.ResultsAmong the 20 biomarkers studied with the multiplex Ella platform, we identified a three-biomarker panel composed of β-NGF, CXCL9 and TRAIL that were independently associated with prognosis both at the time of PAH diagnosis and at the first follow-up after initiation of PAH therapy. β-NGF and CXCL9 were predictors of death or transplantation, whereas high levels of TRAIL were associated with a better prognosis. Furthermore, the prognostic value of the three cytokines was more powerful for predicting survival than usual non-invasive variables (New York Heart Association Functional Class, 6-min walk distance and BNP/NT-proBNP). The results were validated in a fully independent external validation cohort.ConclusionThe monitoring of β-NGF, CXCL9 and TRAIL levels in serum should be considered in the management and treatment of patients with PAH to objectively guide therapeutic options.

Published
2022

25. Outcomes of patients with decreased arterial oxyhaemoglobin saturation on pulmonary arterial hypertension drugs

Author

Laurent Savale, Anne Guillaumot, Laura Textoris, Antoine Beurnier, François Chabot, Arnaud Maurac, Xavier Jaïs, Emmanuel Gomez, Ari Chaouat, Simon Valentin, François Picard, Grégoire Prévot, David Montani, Jean-François Mornex, Renaud Fay, Olivier Sitbon, Marc Humbert, Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Université de Lorraine (UL), Université Paris-Saclay, AP-HP Hôpital Bicêtre (Le Kremlin-Bicêtre), INSERM UMR_S 999 «Pulmonary Hypertension: Pathophysiology and Novel Therapies», Hôpital Marie Lannelongue, 92350 Le Plessis-Robinson, France, Hôpital Bicêtre, Université de Bordeaux (UB), Hôpital Louis Pradel [CHU - 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), and CHU Toulouse [Toulouse]

Subjects
Pulmonary and Respiratory Medicine, medicine.medical_specialty, Heart disease, medicine.medical_treatment, [SDV.MHEP.PSR]Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, Oxygen therapy, Diffusing capacity, Medicine, Humans, Familial Primary Pulmonary Hypertension, 030212 general & internal medicine, Respiratory system, Retrospective Studies, Pulmonary Arterial Hypertension, Lung, business.industry, Hazard ratio, Retrospective cohort study, medicine.disease, Pulmonary hypertension, 3. Good health, medicine.anatomical_structure, 030228 respiratory system, Pharmaceutical Preparations, Oxyhemoglobins, Cardiology, business
Abstract

BackgroundDrugs approved for the treatment of pulmonary arterial hypertension (PAH) improve long-term outcomes. These drugs have pulmonary vasodilator properties which may potentially cause a decrease in arterial oxyhaemoglobin saturation (SaO2) in some patients. The present retrospective study of the French Pulmonary Hypertension Registry aimed to describe the clinical characteristics and outcomes of patients showing a ≥3% decrease inSaO2while treated with PAH drugs.MethodsWe reviewed 719 PAH patients. The exclusion criteria were PAH associated with congenital heart disease and PAH with overt features of venous/capillaries involvement.Results173 (24%) patients had a ≥3% decrease inSaO2. At diagnosis, they were older with a lower diffusing capacity of the lung for carbon monoxide and a shorter 6-min walk distance compared with those who did not display a ≥3% decrease inSaO2. The percentage of patients meeting the European Society of Cardiology/European Respiratory Society (ESC/ERS) low-risk criteria at re-evaluation was significantly lower in those with a ≥3% decrease inSaO2and more patients started long-term oxygen therapy in this group (16%versus5%; pSaO2was associated with a poorer survival (hazard ratio 1.81, 95% CI 1.43–2.34; pSaO2was a prognostic factor independent of age at diagnosis and ESC/ERS risk stratification at follow-up.ConclusionsWhen treated with PAH drugs, a large subset of patients experience a ≥3% decrease inSaO2, which is associated with worse long-term outcomes and reduced survival.

Published
2021

26. 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

29. 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

30. Severe COVID-19 pneumonia: clinical, functional, and imaging outcomes at 4 months

Author

T. Viatgé, Pascal Thomas, Marion Dupuis, S. Pontier, Alain Didier, Grégoire Prévot, Samia Collot, Stein Silva Sifontes, Julien Mazieres, Laure Crognier, Benoit Lepage, Laurent Mhanna, Jon Idoate Lacasia, Elise Noel-Savina, Sihem Bouharaoua, and Guillaume Faviez

Subjects
medicine.medical_specialty, Pneumonia, Coronavirus disease 2019 (COVID-19), business.industry, Internal medicine, medicine, business, medicine.disease
Published
2021

31. Adherence to guidelines in idiopathic pulmonary fibrosis: a follow-up national survey

Author

Vincent Cottin, Emmanuel Bergot, Arnaud Bourdin, Jacques Cadranel, Philippe Camus, Bruno Crestani, Jean-Charles Dalphin, Philippe Delaval, Claire Dromer, Dominique Israel-Biet, Romain Kessler, Sylvain Marchand-Adam, Charles Hugo Marquette, Grégoire Prévot, Martine Reynaud-Gaubert, Dominique Valeyre, Benoit Wallaert, Benoit Bouquillon, and Jean-François Cordier

Subjects
Medicine
Abstract

A new survey coordinated by the French expert centres for rare pulmonary diseases investigated French pulmonologists' diagnostic and therapeutic practice for idiopathic pulmonary fibrosis (IPF) and explored changes since a previous survey in 2011–2012. From May 16 to August 30, 2014, 524 pulmonologists were contacted. Those following at least one patient with IPF were invited to complete a questionnaire administered by telephone or e-mail. 166 (31.7%) pulmonologists, 161 (97%) of whom had participated to the first survey, completed the questionnaire. Of those, 46% and 52%, respectively, discussed the cases with radiologists and pathologists. Out of 144 pulmonologists practicing outside of expert centres, 80% indicated referring patients to those centres. The 2013 French practical guidelines for IPF were known by 92% of pulmonologists involved in IPF, 96% of whom considered them appropriate for practice. The multidisciplinary discussion form for IPF diagnosis was known by 74% and considered appropriate by 94%. Diagnosis and management resulted from multidisciplinary discussion in 50% of the cases. About 58% of patients were diagnosed with “mild to moderate IPF” as defined by forced vital capacity ≥50% of the predicted value and diffusing capacity for carbon monoxide ≥35% of predicted. At the time of the survey, 31% of physicians were using pirfenidone to treat patients with “mild-to-moderately severe IPF” and 30% generally prescribed no treatment. Substantial improvement has occurred since the 2011–2012 survey with regard to knowledge of guidelines and proper management of IPF. Early diagnosis still needs to be improved through the network of expert centres.

Published
2015
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32. Impact of pulmonary hypertension on lung cancer management

Author

Lucile Durin, Elise Noël-Savina, Valentin Héluain, Pierre Mattei, Julien Mazières, and Grégoire Prévot

Subjects
Male, Pulmonary and Respiratory Medicine, Lung Neoplasms, Hypertension, Pulmonary, Biopsy, Humans, Female, Lung, Aged, Retrospective Studies
Abstract

The diagnosis and management of lung cancer is challenging among patients followed-up for pulmonary hypertension (PH). Many interventional procedures are not suitable for severely ill patients, thus limiting the diagnosis and treatment of cancer. We report on patients diagnosed with both conditions in our Institution.We conducted a retrospective observational cohort study at Toulouse University Hospital. We analysed both management and outcome for patients followed-up for precapillary PH following diagnosis of primary lung cancer.Out of 764 patients followed-up for PH, 25 went on to develop bronchopulmonary neoplasia. The median age was 69 years with a predominance of males (56%) and smokers (92%). Fifty-two percent had group 1 PH and 36% severe group 3 PH. The comorbidity burden was high and 76% were oxygen-dependent. Twenty-eight percent of patients were considered ineligible for tissue biopsy, the diagnosis being made by a multidisciplinary team (MDT) based on radio-clinical presentation. Fifty-four percent of patients did not benefit from any treatment. Sixteen percent of pulmonary diagnostic procedures were associated with complications (severe hypoxaemia, intra-alveolar hemorrhage, haemothorax). Patients were undertreated compared to disease stage guidelines (2 surgical procedures for 9 localised stages). Median survival after cancer diagnosis was 6 months.The management of lung cancer is complex in PH patients. The high rate of complications during the diagnosis and therapy steps coupled with very poor patient outcome for both conditions should prompt physicians to thoroughly discuss the benefit/risk benefit in each case.

Published
2022

33. Association between Leflunomide and Pulmonary Hypertension

Author

Thomas Lacoste Palasset, Sophie Bulifon, Xavier Jaïs, Laurent Savale, Laurent Bertoletti, Gérald Simonneau, Marie-Camille Chaumais, Martine Reynaud-Gaubert, Céline Chabanne, Mathilde Volpato, Frédéric Perros, Grégoire Manaud, Laura C. Price, Kais Ahmad, Nicolas Favrolt, O. Sitbon, Anne Guillaumot, Athénaïs Boucly, Jason Weatherald, Nicolas Lamblin, Grégoire Prévot, Pierre Fesler, Charles Khouri, David Montani, Andrei Seferian, Delphine Horeau-Langlard, Stefana Pancic, Mitja Jevnikar, David Launay, Marc Humbert, 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, Imperial College London, Centre Hospitalier Universitaire [Grenoble] (CHU), University of Calgary, Laboratoire Traitement du Signal et de l'Image (LTSI), Université de Rennes (UR)-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Pontchaillou [Rennes], Hospices Civils de Lyon (HCL), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Centre hospitalier universitaire de Nantes (CHU Nantes), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Physiologie & médecine expérimentale du Cœur et des Muscles [U 1046] (PhyMedExp), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM)-Centre National de la Recherche Scientifique (CNRS), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Biologie intégrative du tissu osseux, Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM), CIC Saint Etienne, Centre Hospitalier Universitaire de Saint-Etienne (CHU de Saint-Etienne)-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Hôpital Nord (Saint Etienne), CHU Marseille, Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), MORNET, Dominique, Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES), CHU Toulouse [Toulouse], Université de Montpellier (UM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Université Jean Monnet [Saint-Étienne] (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre Hospitalier Universitaire de Saint-Etienne (CHU de Saint-Etienne)-Université Jean Monnet [Saint-Étienne] (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Hôpital Nord (Saint Etienne), Biologie Intégrative du Tissu Osseux (LBTO), Centre d'Investigation Clinique - Epidémiologie Clinique Saint-Etienne (CIC-EC), and Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E)-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)

Subjects
Pulmonary and Respiratory Medicine, Cardiac Catheterization, medicine.medical_specialty, antirheumatic agents, Hypertension, Pulmonary, [SDV]Life Sciences [q-bio], Cardiac index, Hemodynamics, 030204 cardiovascular system & hematology, Gastroenterology, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, Pharmacovigilance, pulmonary hypertension, medicine, Humans, Risk factor, Lung, Leflunomide, business.industry, Endothelial Cells, medicine.disease, Pulmonary hypertension, 3. Good health, [SDV] Life Sciences [q-bio], medicine.anatomical_structure, 030228 respiratory system, pharmacovigilance, Toxicity, translational medical research, Vascular resistance, business, medicine.drug
Abstract

International audience; Rationale: Pulmonary hypertension (PH) has been described in patients treated with leflunomide. Objectives: To assess the association between leflunomide and PH. Methods: We identified incident cases of PH in patients treated with leflunomide from the French PH Registry and through the pharmacoVIGIlAnce in Pulmonary ArTerial Hypertension (VIGIAPATH) program between September 1999 to December 2019. PH etiology, clinical, functional, radiologic, and hemodynamic characteristics were reviewed at baseline and follow-up. A pharmacovigilance disproportionality analysis using the World Health Organization's global database was conducted. We then investigated the effect of leflunomide on human pulmonary endothelial cells. Data are expressed as median (min-max). Results: Twenty-eight patients treated with leflunomide before PH diagnosis was identified. A total of 21 (75%) had another risk factor for PH and 2 had two risk factors. The median time between leflunomide initiation and PH diagnosis was 32 months (1-120). Right heart catheterization confirmed precapillary PH with a cardiac index of 2.37 L⋅min-1 ⋅m-2 (1.19-3.1) and elevated pulmonary vascular resistance at 9.63 Wood Units (3.6-22.1) without nitric oxide reversibility. Five patients (17.9%) had no other risk factor for PH besides exposure to leflunomide. No significant hemodynamic improvement was observed after leflunomide withdrawal. The pharmacovigilance disproportionality analysis using the World Health Organization's database revealed a significant overrepresentation of leflunomide among reported pulmonary arterial hypertension-adverse drug reactions. In vitro studies showed the dose-dependent toxicity of leflunomide on human pulmonary endothelial cells. Conclusions: PH associated with leflunomide is rare and usually associated with other risk factors. The pharmacovigilance analysis suggests an association reinforced by experimental data.

Published
2021

34. Are serum immunoglobulin concentrations a predictive biomarker of response to anti-IL5/IL5Rα therapies?

Author

Simon Lauret, Elise Noel-Savina, Grégoire Prévot, Nicolas Guibert, Laurent Reber, Danièle Brouquières, Alain Didier, and Laurent Guilleminault

Subjects
Pulmonary and Respiratory Medicine, Eosinophils, Male, Immunoglobulin G, Interleukin-5 Receptor alpha Subunit, Humans, Female, Interleukin-5, Middle Aged, Asthma, Biomarkers
Abstract

Approval of biologics has recently revolutionized T2 severe asthma management. However, predictive biomarkers remain highly needed to improve patient's selection.This study aims to determine whether serum immunoglobulins (Igs) levels might be predictive biomarkers of response to anti-interleukin-5 (IL5)/IL5Rα therapies.Severe asthma patients eligible for mepolizumab or benralizumab were included herein. Serum immunoglobulin quantification was performed at baseline before mepolizumab or benralizumab initiation. After a 6-month treatment of mepolizumab or benralizumab, patients presented a second serum immunoglobulin quantification. The treatment response was evaluated by the GETE (Global Evaluation of Treatment Effectiveness) score at 6 months.A total of 50 patients were included. Median age was 56 [IQR 48.8-65.3] and 50% were females. Compared to baseline, a significant increase in IgG was observed at 6 months (9.2 [7.8-10.2] g/l vs 10.1 [8.8-11.1] g/l, p = 0.04). The area under the ROC curve was 0.58 [95%IC 0.40-0.77] for blood eosinophil count (p = 0.37), 0.75 [95%IC: 0.58-0.92] for serum IgG concentration (p = 0.009) for predicting the treatment response. According to the Youden index, serum IgG concentration ≥ 9.2 g/l predicts the response to anti-IL5 therapies with a sensitivity of 76.9% and a specificity of 75.7%.Baseline serum IgG concentrations may be a useful tool to predict the response to anti-IL5/IL5Rα therapies but should be confirmed in larger clinical trials. Interestingly, anti-IL5/IL5Rα therapies are associated with a significant increase in serum IgG concentrations at 6 months.

Published
2021

35. Cardiac magnetic resonance imaging with late gadolinium enhancement in acute myocarditis: Towards differentiation between immune-mediated and viral-related aetiologies

Author

Yoan Lavie-Badie, Stanislas Faguer, David Ribes, Grégory Pugnet, Didier Carrié, Michel Galinier, Fatiha Sebai, Olivier Lairez, Antoine Petermann, Hervé Rousseau, Grégoire Prévot, S. Brun, Eve Cariou, Hôpital de Rangueil, CHU Toulouse [Toulouse], Université Toulouse III - Paul Sabatier (UT3), and Université Fédérale Toulouse Midi-Pyrénées

Subjects
Adult, Gadolinium DTPA, Male, medicine.medical_specialty, Myocarditis, [SDV]Life Sciences [q-bio], Contrast Media, 030204 cardiovascular system & hematology, Diagnosis, Differential, Young Adult, 03 medical and health sciences, Basal (phylogenetics), 0302 clinical medicine, Immune system, Predictive Value of Tests, Risk Factors, Cardiac magnetic resonance imaging, Internal medicine, medicine, Humans, Late gadolinium enhancement, In patient, cardiovascular diseases, 030212 general & internal medicine, Aged, Retrospective Studies, medicine.diagnostic_test, business.industry, General Medicine, Middle Aged, medicine.disease, Magnetic Resonance Imaging, Acute myocarditis, Acute Disease, Etiology, Cardiology, Female, Cardiology and Cardiovascular Medicine, business
Abstract

Diagnosing immune-mediated myocarditis is challenging because of non-specific clinical signs and symptoms. Cardiac magnetic resonance imaging (CMR) provides subepicardial late gadolinium enhancement (LGE) in the setting of acute myocarditis, but the diagnostic value of LGE pattern for differentiating between immune-mediated and viral-related aetiologies remains unknown.To determine the value of LGE pattern for differentiating between immune-mediated and viral-related aetiologies in patients with acute myocarditis.One hundred and five patients with acute myocarditis who underwent CMR, including LGE variables, were included retrospectively. Viral-related aetiology was retained with a negative autoimmune and autoinflammatory assessment at diagnosis and 6-month follow-up.Aetiology was immune-mediated in 31 patients and viral-related in 74 patients. Patients with immune-mediated myocarditis were older (55±16 vs. 31±12years; P0.001) and more likely to be female (52% vs. 14%; P0.001) than those with viral-related myocarditis. There was no difference in left ventricular ejection fraction between the immune-mediated and viral-related myocarditis groups (53±15% vs. 57±8%; P=0.61). Regarding LGE, patients with viral-related myocarditis were more likely to have basal anteroseptal, mid anteroseptal, mid anterior and basal anterolateral location. Patients with immune-mediated myocarditis were more likely to have apical septal, apical inferior, apical lateral, mid anterolateral and basal inferior location. Segments with difference in prevalence of LGE between aetiologies were summed to build a score where positive significant association with immune-mediated myocarditis was quoted 1 and positive significant association with viral-related myocarditis was quoted -1. A score≥0 differentiated immune-mediated from viral-related myocarditis with 94% sensitivity and 77% specificity (area under the receiver operating characteristic curve 0.88; P0.001).CMR provides arguments for differentiating immune-mediated from viral-related acute myocarditis by showing preferential LGE localization in apical septal, apical inferior, apical lateral and basal inferior segments.

Published
2019

36. Clinical impact and prognosis of cryoglobulinemia and cryofibrinogenemia in systemic sclerosis

Author

Sébastien, De Almeida Chaves, Bénédicte, Puissant, Tiphaine, Porel, Eva, Bories, Daniel, Adoue, Laurent, Alric, Léonardo, Astudillo, Antoine, Huart, Olivier, Lairez, Martin, Michaud, David, Ribes, Grégoire, Prévot, Laurent, Sailler, Francis, Gaches, and Gregory, Pugnet

Subjects
Scleroderma, Systemic, Cryoglobulinemia, Immunology, Humans, Immunology and Allergy, Prognosis, Cryoglobulins
Abstract

An association of systemic sclerosis (SSc) with cryoglobulin and/or cryofibrinogenemia has been described. However, clinical, biological, morphological and prognostic implications are unknown. The objective of this study was to describe the phenotype and evaluate the prognosis of cryoglobulinemia and/or cryofibrinogenemia in the progression of SSc.Patients were included from the Systemic Scleroderma Toulouse Cohort (SSTC), between June 1, 2005 and May 31, 2018, and underwent a measurement of a cryoglobulin and/or cryofibrinogen in immunology laboratory at the Toulouse University Hospital Center. Patients with and without cryoglobulinemia50 mg/l and patients with and without cryofibrinogenemia were compared to identified the impact of cryoprcipitate on the phenotype. Mortality based on cryoprecipitate was explored.166 patients were included in the study. 43.3% and 46.6% had a cryoglobulinemia50 mg/l and cryofibrinogenemia, respectively. Cryoglobulin50 mg was not associated with microvascular damage. Cryoglobulin does not influence the phenotype. 5-and 10-years survival were 97.6% and 88.8% respectively in patients with cryoglobulinemia50 mg/l versus 91.9% and 78.4% in patients without cryoglobulin50 mg/l. 10-years survival was better for patients with cryoglobulinemia50 mg/l (log-rank 0.0363). Cryofibrinogenemia was not associated with neoplasia, any clinical (in particular ischemic damage), biological or morphological features. Cryofibrinogenemia had no influence on the mortality of these patients.Cryoglobulinemia and cryofibrinogenemia are frequent in SSc. The presence of cryoprecipitate (cryoglobulin or cryofibrinogen) not influence the phenotype and has not associated with a poor survival.

Published
2022

37. 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

38. Desquamative interstitial pneumonia after tear gas exposure

Author

Grégoire Prévot, Magali Colombat, Thomas Villeneuve, Samia Collot, and Alain Didier

Subjects
Pulmonary and Respiratory Medicine, Pathology, medicine.medical_specialty, Tear gas, business.industry, Humans, Medicine, Lung Diseases, Interstitial, Tear Gases, business, Desquamative interstitial pneumonia, medicine.disease, Lung
Published
2022

39. Alternative processing of the U2 small nuclear RNA produces a 19-22nt fragment with relevance for the detection of non-small cell lung cancer in human serum.

Author

Julien Mazières, Caroline Catherinne, Olivier Delfour, Sandrine Gouin, Isabelle Rouquette, Marie-Bernadette Delisle, Grégoire Prévot, Roger Escamilla, Alain Didier, David H Persing, Mike Bates, and Bernard Michot

Subjects
Medicine, Science
Abstract

RNU2 exists in two functional forms (RNU2-1 and RNU2-2) distinguishable by the presence of a unique 4-bases motif. Detailed investigation of datasets obtained from deep sequencing of five human lung primary tumors revealed that both forms express at a high rate a 19-22nt fragment (miR-U2-1 and -2) from its 3' region and contains the 4-bases motif. Deep sequencing of independent pools of serum samples from healthy donors and lung cancer patients revealed that miR-U2-1 and -2 are pervasively processed in lung tissue by means of endonucleolytic cleavages and stably exported to the blood. Then, microarrays hybridization experiments of matched normal/tumor samples revealed a significant over-expression of miR-U2-1 in 14 of 18 lung primary tumors. Subsequently, qRT-PCR of miR-U2-1 using serum from 62 lung cancer patients and 96 various controls demonstrated that its expression levels identify lung cancer patients with 79% sensitivity and 80% specificity. miR-U2-1 expression correlated with the presence or absence of lung cancer in patients with chronic obstructive pulmonary disease (COPD), other diseases of the lung - not cancer, and in healthy controls. These data suggest that RNU2-1 is a new bi-functional ncRNA that produces a 19-22nt fragment which may be useful in detecting lung cancer non-invasively in high risk patients.

Published
2013
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40. 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

41. Impact of pulmonary perfusion defects by scintigraphy on pulmonary vascular resistances, functional capacity and right ventricular systolic function in patients with chronic thromboembolic pulmonary hypertension

Author

Damien, Eyharts, Pierre, Pascal, Yoan, Lavie-Badie, Eve, Cariou, Stéphanie, Cazalbou, Clément, Karsenty, Grégoire, Prévot, Didier, Carrié, Isabelle, Berry, Elise, Noël-Savina, and Olivier, Lairez

Subjects
Original Article
Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is a major cause of chronic pulmonary hypertension leading to right heart failure and death. Ventilation/perfusion single photon emission computed tomography (V/Q SPECT) is the screening test of choice showing mismatch in at least one segment or two sub-segments. Our aim was to investigate the relationship between the extent of pulmonary perfusion defects and hemodynamic, echocardiographic, biological and functional parameters. Between 2012 and 2019, 46 patients with CTEPH were retrospectively enrolled in the study. The diagnosis of pulmonary hypertension was made by the referral team of the expert center according to the European guidelines. All patients underwent pulmonary V/Q SPECT, right heart catheterization, transthoracic echocardiography (TTE), functional tests and natriuretic peptides assays. There was a slight correlation between the extent of pulmonary perfusion defects and pulmonary vascular resistances (R=0.510, P < 0.001). However, there was no correlation between the extent of pulmonary perfusion defects and NYHA stage, NT-proBNP level, functional parameters (6 minutes-walk distance-6 MWD), right ventricular function assessed by TTE. Pulmonary perfusion defects extension by V/Q lung SPECT are correlated with pulmonary vascular resistances in CTEPH. However, it is not correlated with right ventricular function and functional parameters.

Published
2020

42. Are serum immunoglobulins a predictive biomarker of response for anti-IL5 therapies?

Author

Simon Lauret, Alain Didier, Danielle Brouquieres, Laurent Guilleminault, Elise Noel-Savina, and Grégoire Prévot

Subjects
medicine.medical_specialty, Treatment response, biology, business.industry, Youden's J statistic, Igg subclasses, Gastroenterology, Internal medicine, medicine, biology.protein, Antibody, business, Area under the roc curve, Blood eosinophil, Interleukin 5, Predictive biomarker
Abstract

Approval of biologics has recently revolutionized the management of severe asthma. However, predictive biomarkers remain highly needed to improve the patients’ selection. This study aims to determine if serum immunoglobulins (Ig) may be predictive biomarkers of response for anti- interleukin 5 (IL5) therapies. Inclusion criteria were: patients with severe asthma being treated with anti-IL5 therapies, Ig and IgG subclasses quantification before treatment and at 6-month. The treatment response was evaluated by the GETE score (Global Evaluation of Treatment Effectiveness) at 6 months. A total of 50 patients were included. Median age was 56 [IQR 48.8-65.3] and 50% were females. Compared to baseline, a significant increase in IgG was observed at 6 months (9.2 [7.8-10.2] g/l vs 10.1 [8.8-11.1] g/l, p=0.04). A non-significant decrease in serum IgG4 was also observed at 6 months (0.68 [0.36-1.13] g/l vs 0.50 [0.33-0.72] g/l, p=0.09). The area under the ROC curve for the prediction of the treatment response was 0.58 [95%IC 0.40-0.77] for blood eosinophil count (p=0.37), 0.75 [95%IC: 0.58-0.92] for serum IgG concentration (p=0.009) and 0.66 [95%IC: 0.49-0.82] for serum IgG4 concentration (p=0.09) (Figure 1). According to Youden index, serum IgG concentration ≥ 9.2 g/l predicts the response to anti-IL5 therapies with a sensitivity of 76.9% and a specificity of 75.7%. Conclusion: Baseline serum IgG concentration may be a useful tool to predict the anti-IL5 therapies response.

Published
2020

43. Perioperative management of patients with pulmonary hypertension in non-cardiac surgery, a retrospective study in a reference center

Author

Grégoire Prévot, Elise Noel Savina, Olivier Mathe, and nina cugnin

Subjects
medicine.medical_specialty, Perioperative management, business.industry, Mortality rate, Retrospective cohort study, Perioperative, medicine.disease, Pulmonary hypertension, Surgery, Walk test, Non cardiac surgery, Anesthetic, medicine, business, medicine.drug
Abstract

We carried out a retrospective study to assemble perioperative data from patients with group 1 and 4 pulmonary hypertension (PH) undergoing non-cardiac surgery and followed in the reference center of Toulouse university hospital. Patients who were rejected for anesthetic procedures were also included. In total, 43 patients were included from january 2012 to january 2020, 32 in the operated group and 11 in the contraindicated group. In the operated group, 10 patients (22%) underwent emergency surgery and 22 patients (69%) a scheduled procedure. Most of the procedures were major surgeries (62.6%) and were performed under general anesthesia (65.6%). 11 patients suffered from major complications (34.4%), with a mortality rate of 12.5% (n=4), 4.5% (n=1) in scheduled surgery and 30.3% (n=3) in emergency surgeries. The cause was right heart failure in 3 cases out of 4.PH of the patients was well controlled with 68.8% of patients with a NYHA score at I-II and average 6-minute walk test at 415m. Most patients were receiving specific therapy. Risk factors for perioperative mortality were emergency surgery (p=0.03), use of peroperative norepinephrine (p=0.0001), high or intermediate risk assessment score (p=0.03) and a right atrial surface greater than 26 cm² at cardiac echography (p=0.009). Patients in contraindicated group were more severe, with significantly higher NYHA score, mPAP and lower 6MWT. Patients seemed less severe than in the previous studies, probably due to a constant improvement in PH management. The high mortality rate found in this study can be explained by the large number of emergency surgeries and the importance of patients comorbidities.

Published
2020

44. Initial combination therapy of macitentan and tadalafil in pulmonary arterial hypertension

Author

Olivier Sitbon, Gérald Simonneau, Pierre Clerson, Laurent Bertoletti, Grégoire Prévot, Virginie Gressin, Loïc Perchenet, François Picard, Hélène Bouvaist, Vincent Cottin, Emmanuel Bergot, and Matthieu Canuet

Subjects
Pulmonary and Respiratory Medicine, Endothelin Receptor Antagonists, Pulmonary Arterial Hypertension, Sulfonamides, Download, business.industry, Conflict of interest, Advertising, Research Letters, Tadalafil, 03 medical and health sciences, 0302 clinical medicine, Pyrimidines, 030228 respiratory system, Health care, Medicine, Humans, Drug Therapy, Combination, 030212 general & internal medicine, business, Initial therapy, Production team, Agora
Abstract

Initial combination therapy plays a central role in managing pulmonary arterial hypertension (PAH) [1–4]. Patients with low- or intermediate-risk of 1-year mortality at diagnosis should be treated with initial combination therapy with an endothelin receptor antagonist (ERA) and phosphodiesterase type-5 inhibitor (PDE5i) [2–4]. Benefits of initial therapy with the ERA ambrisentan and PDE5i tadalafil were demonstrated in AMBITION [1]; prospective evidence for other treatment combinations within these drug classes is needed., Initial combination therapy with macitentan and tadalafil is well tolerated and improves cardiopulmonary haemodynamics and functional capacity in newly diagnosed PAH patients https://bit.ly/3aWZagH

Published
2020

45. Child–Adult Transition in Sarcoidosis: A Series of 52 Patients

Author

Simon Chauveau, Florence Jeny, Marie-Emeline Montagne, Rola Abou Taam, Véronique Houdouin, Ulrich Meinzer, Christophe Delacourt, Ralph Epaud, Fleur Cohen Aubart, Catherine Chapelon-Abric, Dominique Israël-Biet, Karine Juvin, Antoine Dossier, Bahram Bodaghi, Grégoire Prévot, Jean-Marc Naccache, Sarah Mattioni, Antoine Deschildre, Jacques Brouard, Abdellatif Tazi, Roderich Meckenstock, Morgane Didier, Julien Haroche, Annick Clement, Jean-François Bernaudin, Hilario Nunes, Dominique Valeyre, Nadia Nathan, and for the French Sarcoidosis Group (GSF)

Subjects
Pediatrics, medicine.medical_specialty, medicine.drug_class, Severe disease, lcsh:Medicine, Disease, 030204 cardiovascular system & hematology, Article, 03 medical and health sciences, 0302 clinical medicine, children, transition of care, 0502 economics and business, medicine, sarcoidosis, Adverse effect, interstitial lung disease, Series (stratigraphy), Cumulative dose, business.industry, 05 social sciences, lcsh:R, Interstitial lung disease, General Medicine, medicine.disease, Corticosteroid, 050211 marketing, Sarcoidosis, business
Abstract

(1) Background: Pediatric sarcoidosis is a rare and mostly severe disease. Very few pediatric series with a prolonged follow-up are reported. We aimed to evaluate the evolution of pediatric sarcoidosis in adulthood. (2) Material and methods: Patients over 18-years-old with a pediatric-onset sarcoidosis (&le, 15-year-old) who completed at least a three-year follow-up in French expert centers were included. Clinical information at presentation and outcome in adulthood were studied. (3) Results: A total of 52 patients were included (34 prospectively in childhood and 18 retrospectively in adulthood), with a mean age of 12 (&plusmn, 2.7) at diagnosis. The median duration time of follow-up was 11.5 years (range 3&ndash, 44.5). Relapses mostly occurred during treatment decrease (84.5%), others within the three years after treatment interruption (9.1%), and rarely when the disease was stable for more than three years (6.4%). Sarcoidosis was severe in 11 (21.2%) in adulthood. Patients received a high corticosteroid cumulative dose (median 17,900 mg) for a median duration of five years (range 0&ndash, 32), resulting in mostly mild (18, 35.3%) and rarely severe (2, 3.8%) adverse events. (4) Conclusions: Pediatric-onset sarcoidosis needed a long-term treatment in almost half of the patients. Around one fifth of pediatric-onset sarcoidosis patients had severe sarcoidosis consequences in adulthood.

Published
2020

46. Phenotype and Outcomes of Pulmonary Hypertension Associated with Neurofibromatosis Type 1

Author

Pamela Moceri, Maria-Rosa Ghigna, Olivier Sitbon, Céline Chabanne, Marc Humbert, Pascal de Groote, Pierre Wolkenstein, Julie Traclet, Xavier Mignard, Emmanuel Bergot, François Goupil, Delphine Bourlier, Xavier Jaïs, Etienne-Marie Jutant, David Montani, Frédéric Perros, Laurent Bertoletti, Jean-Pierre Gueffet, Gérald Simonneau, Thibaud Soumagne, Nicolas Favrolt, Grégoire Prévot, Cécile Tromeur, Pierre-Yves Brillet, Barbara Girerd, Mitja Jevnikar, Fabrice Bauer, Ari Chaouat, Pascal Magro, Raphael Borie, Elie Fadel, Laurent Savale, and Claire Dauphin

Subjects
Pulmonary and Respiratory Medicine, Adult, Male, congenital, hereditary, and neonatal diseases and abnormalities, medicine.medical_specialty, Lung Neoplasms, Neurofibromatosis 1, Adolescent, Hypertension, Pulmonary, Critical Care and Intensive Care Medicine, Gastroenterology, Young Adult, Internal medicine, medicine, Humans, Neurofibromatosis, neoplasms, Neurofibromin 1, business.industry, Interstitial lung disease, Middle Aged, medicine.disease, Prognosis, Phenotype, Pulmonary hypertension, eye diseases, nervous system diseases, Female, France, business, Complication, Lung Transplantation
Abstract

Rationale: Pulmonary hypertension (PH) associated with neurofibromatosis type 1 (NF1) is a rare and largely unknown complication of NF1.Objectives: To describe characteristics and outcomes of PH-NF...

Published
2020

47. Zoster after Cyclophosphamide for Systemic Lupus Erythematosus or Vasculitis: Incidence, Risk Factors, and Effect of Antiviral Prophylaxis

Author

David Ribes, Laurent Sailler, Guillaume Moulis, Laurent Alric, Antoine Huart, Grégoire Prévot, Hélène Derumeaux, Pierre Delobel, Catherine Mengelle, Camille Garnier, Daniel Adoue, Dominique Chauveau, Grégory Pugnet, Service de Maladies Infectieuses et Tropicales, Hôpital Purpan [Toulouse], CHU Toulouse [Toulouse]-CHU Toulouse [Toulouse], Centre de Référence du Sud Ouest des Maladies Rénales Rares, CHU Toulouse [Toulouse]-Hôpital des Enfants, CHU Toulouse [Toulouse], Epidémiologie et analyses en santé publique : risques, maladies chroniques et handicaps (LEASP), Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre d'investigation clinique de Toulouse (CIC 1436), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-CHU Toulouse [Toulouse]-Institut National de la Santé et de la Recherche Médicale (INSERM), Département de pneumologie [CHU Toulouse], Pharmacochimie et Biologie pour le Développement (PHARMA-DEV), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Institut de Chimie de Toulouse (ICT-FR 2599), Institut National Polytechnique (Toulouse) (Toulouse INP), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Centre National de la Recherche Scientifique (CNRS)-Institut de Recherche pour le Développement (IRD)-Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Institut de Chimie du CNRS (INC)-Institut National Polytechnique (Toulouse) (Toulouse INP), Université Fédérale Toulouse Midi-Pyrénées-Centre National de la Recherche Scientifique (CNRS)-Institut de Recherche pour le Développement (IRD)-Institut de Chimie du CNRS (INC)-Institut de Recherche pour le Développement (IRD), Laboratoire de Virologie, Service Maladies infectieuses et tropicales [CHU Toulouse], Pôle Inflammation, infection, immunologie et loco-moteur [CHU Toulouse] (Pôle I3LM Toulouse), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Université de Toulouse (UT)-Université de Toulouse (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM), Université de Toulouse (UT)-Université de Toulouse (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Pôle Santé publique et médecine publique [CHU Toulouse], Service Pneumologie-Allergologie [CHU Toulouse], Pôle Clinique des Voies respiratoires [CHU Toulouse], Institut de Recherche pour le Développement (IRD)-Institut de Chimie de Toulouse (ICT), Institut de Recherche pour le Développement (IRD)-Université Toulouse III - Paul Sabatier (UT3), Université de Toulouse (UT)-Université de Toulouse (UT)-Institut de Chimie du CNRS (INC)-Centre National de la Recherche Scientifique (CNRS)-Institut National Polytechnique (Toulouse) (Toulouse INP), Université de Toulouse (UT)-Institut de Recherche pour le Développement (IRD)-Université Toulouse III - Paul Sabatier (UT3), Université de Toulouse (UT), Laboratoire Virologie [CHU Toulouse], Institut Fédératif de Biologie (IFB), and Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Pôle Biologie [CHU Toulouse]

Subjects
Adult, Male, Vasculitis, ANCA-ASSOCIATED VASCULITIS, medicine.medical_specialty, Cyclophosphamide, [SDV]Life Sciences [q-bio], Immunology, SYSTEMIC VASCULITIS, Antiviral Agents, Herpes Zoster, Young Adult, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, Risk Factors, Internal medicine, Epidemiology, EPIDEMIOLOGY, Humans, Lupus Erythematosus, Systemic, Immunology and Allergy, Medicine, 030212 general & internal medicine, Aged, Retrospective Studies, 030203 arthritis & rheumatology, business.industry, Postherpetic neuralgia, Incidence, Incidence (epidemiology), Retrospective cohort study, Middle Aged, medicine.disease, 3. Good health, SYSTEMIC LUPUS ERYTHEMATOSUS, Cohort, VALACYCLOVIR, Female, business, Immunosuppressive Agents, Systemic vasculitis, medicine.drug
Abstract

Objective.To assess the incidence and the risk factors for zoster in patients exposed to intravenous cyclophosphamide (CYC) for systemic vasculitis or systemic lupus erythematosus (SLE), as well as the protective effect of prophylaxis by valacyclovir (VCV).Methods.This retrospective study included all adults treated by intravenous CYC for SLE or systemic vasculitis between 2011 and 2015 at Toulouse University Hospital, France. Zoster occurrence was recorded using medical chart review, laboratory data, and patient interviews. Univariate Cox models were computed to assess the risk factors for zoster and the protective effect of prophylaxis by VCV.Results.The cohort consisted of 110 patients (81 systemic vasculitis and 29 SLE). During a mean followup of 3.4 years after CYC initiation, 10 cases of zoster occurred, leading to an overall incidence of 27.9/1000 patient-years (95% CI 15.2–50.6); it was 59.4/1000 patients (95% CI 27.5–123.6) during the year after CYC initiation. Four patients experienced persistent postherpetic neuralgia. Probable risk factors were lymphopenia < 500/µl at CYC initiation (HR 5.11, 95% CI 0.94–27.93) and female sex (HR 4.36, 95% CI 0.51–37.31). The incidence was higher in patients with SLE (HR as compared with systemic vasculitis patients = 2.68, 95% CI 0.54–13.26). None of the 19 patients exposed to VCV during the followup developed zoster.Conclusion.The incidence of zoster is high in systemic vasculitis and in patients with SLE exposed to intravenous CYC. CYC may favor postherpetic neuralgia. Prophylaxis by VCV should be considered, particularly in cases of lymphopenia < 500/µl at CYC initiation and during the year after.

Published
2018

48. Pulmonary Arterial Hypertension Associated With Systemic Lupus Erythematosus

Author

Vincent Sobanski, Grégoire Prévot, Pierre Clerson, Christophe Pison, Jean-Claude Brouet, Anne-Laure Fauchais, Céline Chabanne, David Montani, Jean-François Mornex, Vincent Cottin, Marie-Hélène Balquet, Alain Didier, Laurence Rottat, Jean-François Chabot, Eric Hachulla, David Launay, Gaël Cinquetti, Gérald Simonneau, Jean-Claude Meurice, J.-F. Velly, Jean-Pierre Clauvel, Jean-Marc Ziza, Olivier Sitbon, Marc Humbert, Elena Fois, Luc Mouthon, Jacques Cadranel, Philippe Mabo, Xavier Jaïs, Pierre-Dominique Dos Santos, Julie Traclet, Loïc Guillevin, Zahir Amoura, Véronique Le Guern, and Gilbert Habib

Subjects
030203 arthritis & rheumatology, Pulmonary and Respiratory Medicine, medicine.medical_specialty, business.industry, Hazard ratio, Interstitial lung disease, 030204 cardiovascular system & hematology, Critical Care and Intensive Care Medicine, medicine.disease, Pulmonary hypertension, 3. Good health, Pulmonary function testing, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, Cardiology, Medicine, Risk factor, skin and connective tissue diseases, Cardiology and Cardiovascular Medicine, business, Prospective cohort study, Pulmonary wedge pressure, Survival rate
Abstract

Background Pulmonary arterial hypertension (PAH) is a rare complication of systemic lupus erythematosus (SLE). Methods We identified all patients with SLE and PAH (SLE-PAH) who were enrolled in the French Pulmonary Hypertension Registry with a diagnosis confirmed by right heart catheterization (RHC). A control group of 101 patients with SLE without known PAH was selected from SLE expert centers participating in the Pulmonary Hypertension Registry. Survival was estimated by the Kaplan-Meier method. Hazard ratios associated with potential predictors of death were estimated using Cox proportional hazard models. Results Of the 69 patients with SLE-PAH identified in the French Pulmonary Hypertension Registry, 51 were included in the study. They did not differ from the control group regarding age, sex, or duration of SLE at the time of the analysis but had a higher frequency of anti-SSA and anti-SSB antibodies. The delay between SLE diagnosis and PAH diagnosis was 4.9 years (range, 2.8-12.9) years. The 3- and 5-year overall survival rates were 89.4% (95% CI, 76.2%-96.5%) and 83.9% (95% CI, 68.8%-92.1%), respectively. The survival rate was significantly better in patients with anti-U1-RNP antibodies ( P = .04). Conclusions Patients with SLE-PAH have an overall 5-year survival rate of 83.9% after the PAH diagnosis. Anti-SSA/SSB antibodies may be a risk factor for PAH, and the presence of anti-U1-RNP antibodies appears to be a protective factor regarding survival.

Published
2018

49. External validation of a refined four-stratum risk assessment score from the French pulmonary hypertension registry

Author

Mitja Jevnikar, Laurent Savale, Grégoire Prévot, Xavier Jaïs, Arnaud Bourdin, Antoine Beurnier, David Montani, Vincent Cottin, Jason Weatherald, Olivier Sitbon, Marc Humbert, Pascal de Groote, Gérald Simonneau, François Picard, Ari Chaouat, Etienne-Marie Jutant, Athénaïs Boucly, and Delphine Horeau-Langlard

Subjects
Pulmonary and Respiratory Medicine, medicine.medical_specialty, Portopulmonary hypertension, Heart disease, business.industry, medicine.drug_class, Walk distance, External validation, Risk management tools, medicine.disease, Pulmonary hypertension, 3. Good health, 03 medical and health sciences, 0302 clinical medicine, 030228 respiratory system, Internal medicine, medicine, Natriuretic peptide, 030212 general & internal medicine, Risk assessment, business
Abstract

IntroductionContemporary risk assessment tools categorise patients with pulmonary arterial hypertension (PAH) as low, intermediate or high risk. A minority of patients achieve low risk status with most remaining intermediate risk. Our aim was to validate a four-stratum risk assessment approach categorising patients as low, intermediate-low, intermediate-high or high risk, as proposed by the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA) investigators.MethodsWe evaluated incident patients from the French PAH Registry and applied a four-stratum risk method at baseline and at first reassessment. We applied refined cut-points for three variables: World Health Organization functional class, 6-min walk distance and N-terminal pro-brain natriuretic peptide. We used Kaplan–Meier survival analyses and Cox proportional hazards regression to assess survival according to three-stratum and four-stratum risk approaches.ResultsAt baseline (n=2879), the four-stratum approach identified four distinct risk groups and performed slightly better than a three-stratum method for predicting mortality. Four-stratum model discrimination was significantly higher than the three-stratum method when applied during follow-up and refined risk categories among subgroups with idiopathic PAH, connective tissue disease-associated PAH, congenital heart disease and portopulmonary hypertension. Using the four-stratum approach, 53% of patients changed risk category from baseline compared to 39% of patients when applying the three-stratum approach. Those who achieved or maintained a low risk status had the best survival, whereas there were more nuanced differences in survival for patients who were intermediate-low and intermediate-high risk.ConclusionsThe four-stratum risk assessment method refined risk prediction, especially within the intermediate risk category of patients, performed better at predicting survival and was more sensitive to change than the three-stratum approach.

Published
2021

50. Impact of pulmonary perfusion defects by scintigraphy on pulmonary vascular resistances, functional capacity and right ventricular systolic function in chronic thromboembolic pulmonary hypertension

Author

I. Berry, E. Noel-Savina, Clément Karsenty, Olivier Lairez, Eve Cariou, Didier Carrié, Stéphanie Cazalbou, Grégoire Prévot, Yoan Lavie-Badie, Pierre Pascal, and D. Eyharts

Subjects
medicine.medical_specialty, medicine.diagnostic_test, business.industry, Hemodynamics, Systolic function, medicine.disease, Scintigraphy, Pulmonary hypertension, Internal medicine, medicine, Cardiology, Chronic thromboembolic pulmonary hypertension, Respiratory system, Cardiology and Cardiovascular Medicine, business, Perfusion, Emission computed tomography
Abstract

Background Chronic thromboembolic pulmonary hypertension (CTEPH) is a specific subtype of pulmonary hypertension. Pulmonary ventilation/perfusion single-photon emission computed tomography (V/Q SPECT) is the reference diagnostic examination of CTPEH showing mismatch in at least one segment or two sub-segments. Purpose To investigate the relationship between the extent of pulmonary perfusion defects and hemodynamic, echocardiographic, biological and functional parameters. Methods Between 2012 and 2019, 46 patients with CTEPH were retrospectively enrolled in the study. The diagnosis of pulmonary hypertension was made by the referral team of the expert centre according European Society of Cardiology and European Respiratory Society guidelines. All patients underwent pulmonary V/Q SPECT, right heart catheterisation, transthoracic echocardiography (TTE), functional tests and natriuretic peptides assays during the same stay. Results There was a slight correlation between the extent of pulmonary perfusion defects and pulmonary vascular resistances (r = 0.510, P Fig. 1 ). However, there was no correlation between the extent of pulmonary perfusion defects or hemodynamic measurements and right ventricular function assessed by TTE, the functional parameters (6 minutes walk distance [6MWD], NYHA class and NT-proBNP level). In contrast, there was a correlation between right ventricular function assessed in TTE (i.e. tricuspid antero-posterior systolic excursion and tricuspid annular S wave) and 6MWD (r = 0.586, P Conclusions Pulmonary perfusion defects extension by V/Q SPECT can provide information on pulmonary vascular resistances. However, it is not correlated with right ventricular function and functional parameters.

Published
2021

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