14 results on '"INTERSTITIAL LUNG-DISEASE"'
Search Results
2. Autoantibodies in idiopathic inflammatory myopathies: Clinical associations and laboratory evaluation by mono- and multispecific immunoassays
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Yves Allenbach, Jan Damoiseaux, L. Musset, Yehuda Shoenfeld, Ellen De Langhe, Chih Wei Tseng, Xavier Bossuyt, Yi Hsing Chen, Yves Piette, Anouk C.M. Platteel, Dörte Hamann, Ora Shovman, Jean Baptiste Vulsteke, and Carolien Bonroy
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SIGNAL RECOGNITION PARTICLE ,GENE 5 ,Immunology ,SCLEROSIS-ASSOCIATED ANTIBODIES ,INTERSTITIAL LUNG-DISEASE ,Malignancy ,RHEUMATOLOGY CLASSIFICATION CRITERIA ,Medicine and Health Sciences ,medicine ,Humans ,Immunology and Allergy ,PHASE CHEMILUMINESCENCE IMMUNOASSAY ,Myositis ,Autoantibodies ,Immunoassay ,MYOSITIS-SPECIFIC AUTOANTIBODIES ,biology ,business.industry ,Verification ,Interstitial lung disease ,Autoantibody ,ANTI-HMGCR ANTIBODIES ,2017 EUROPEAN LEAGUE ,Dermatomyositis ,medicine.disease ,DIAGNOSTIC PERFORMANCE ,Idiopathic inflammatory myopathies ,biology.protein ,Inclusion body myositis ,Antibody ,business - Abstract
Idiopathic inflammatory myopathies (IIM) are a group of diseases characterized by immune-mediated muscular lesions that may be associated with extra-muscular manifestations involving skin, lungs, heart or joints. Four main groups of IIM can be distinguished: dermatomyositis (DM), overlap myositis including mainly anti-synthetase syndrome (ASS), immune mediated necrotizing myopathy (IMNM), and inclusion body myositis (IBM). Myositis-specific autoantibodies (MSA) are increasingly recognized as valuable tools for diagnosis, classification and prognosis of IIM. For example, ASS is associated with anti-aminoacyl tRNA synthetase antibodies (anti-Jo-1, PL-7, PL-12, …), IMNM with anti-SRP and anti-HMGCR; IBM with anti-cytosolic 5'nucleotidase 1A (cN1A), and DM with anti-Mi-2, anti-MDA-5, anti-TIF-1γ, anti-NXP-2 and anti-SAE. Moreover, anti-MDA-5 is associated with amyopathic myositis and interstitial lung disease and anti-TIF-1γ and anti-NXP-2 with juvenile DM as well as malignancy in patients >40 years. Most MSA have initially been discovered by immunoprecipitation. In routine laboratories, however, MSA are screened for by indirect immunofluorescence and identified by (automated) monospecific immunoassays or by multispecific immunoassays (mainly line/dot immunoassays). Validation of these (multispecific) assays is a challenge as the antibodies are rare and the assays diverse. In this review, we give an overview of the (clinical) performance characteristics of monospecific assays as well as of multispecific assays for detection of MSA. Although most assays are clinically useful, there are differences between techniques and between manufacturers. We discuss that efforts are needed to harmonize and standardize detection of MSA. ispartof: AUTOIMMUNITY REVIEWS vol:18 issue:3 pages:293-305 ispartof: location:Netherlands status: published
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- 2019
3. Computed Tomographic Biomarkers in Idiopathic Pulmonary Fibrosis. The Future of Quantitative Analysis
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Jonathan G. Goldin, Ganesh Raghu, Stephen M. Humphries, Simon L.F. Walsh, Brian J. Bartholmai, Craig S Conoscenti, Joseph Jacob, James F. Gruden, Jan De Backer, Fernando J. Martinez, David Barber, Toby M. Maher, Kevin R. Flaherty, Luca Richeldi, Nicola Sverzellati, David A. Lynch, Athol U. Wells, Grace Kim, Eric A. Hoffman, Xiaoping Wu, Brian D. Ross, Margaret L. Salisbury, Rozsa Schlenker-Herceg, and Kevin K. Brown
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Pulmonary and Respiratory Medicine ,medicine.medical_specialty ,Vital capacity ,AUTOMATED QUANTIFICATION ,Respiratory System ,TREATMENT TRIALS ,Settore MED/10 - MALATTIE DELL'APPARATO RESPIRATORIO ,INTERSTITIAL LUNG-DISEASE ,Critical Care and Intensive Care Medicine ,Idiopathic pulmonary fibrosis ,Critical Care Medicine ,General & Internal Medicine ,END-POINTS ,medicine ,Humans ,Quantitative computed tomography ,Intensive care medicine ,Tomography ,Lung ,11 Medical and Health Sciences ,Pulmonologists ,Science & Technology ,medicine.diagnostic_test ,Surrogate endpoint ,business.industry ,Interstitial lung disease ,STAGING SYSTEM ,medicine.disease ,Idiopathic Pulmonary Fibrosis ,X-Ray Computed ,Clinical trial ,HIGH-RESOLUTION CT ,quantitative computed tomography ,IMAGING BIOMARKERS ,biomarker ,Biomarker (medicine) ,Tomography, X-Ray Computed ,business ,Life Sciences & Biomedicine ,FORCED VITAL CAPACITY ,HYPERSENSITIVITY PNEUMONITIS ,CLINICAL-TRIALS ,Biomarkers ,Forecasting - Abstract
Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease with great variability in disease severity and rate of progression. The need for a reliable, sensitive, and objective biomarker to track disease progression and response to therapy remains a great challenge in IPF clinical trials. Over the past decade, quantitative computed tomography (QCT) has emerged as an area of intensive research to address this need. We have gathered a group of pulmonologists, radiologists and scientists with expertise in this area to define the current status and future promise of this imaging technique in the evaluation and management of IPF. In this Pulmonary Perspective, we review the development and validation of six computer-based QCT methods and offer insight into the optimal use of an imaging-based biomarker as a tool for prognostication, prediction of response to therapy, and potential surrogate endpoint in future therapeutic trials.
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- 2019
4. Endotypes of Prematurity and Phenotypes of Bronchopulmonary Dysplasia: Toward Personalized Neonatology
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Maria Pierro, Karen Van Mechelen, Elke van Westering-Kroon, Eduardo Villamor-Martínez, and Eduardo Villamor
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NITRIC-OXIDE ,EXTREMELY PRETERM INFANTS ,HYPERTENSION ,phenotype ,preterm birth ,Medicine (miscellaneous) ,CHILDREN ,INTERSTITIAL LUNG-DISEASE ,respiratory system ,DIAGNOSIS ,PULMONARY VEIN STENOSIS ,bronchopulmonary dysplasia ,mental disorders ,COMPUTED-TOMOGRAPHY ,endotype ,VASCULAR DEVELOPMENT ,TRACHEOBRONCHOMALACIA - Abstract
Bronchopulmonary dysplasia (BPD), the chronic lung disease of prematurity, is increasingly recognized as the consequence of a pathological reparative response of the developing lung to both antenatal and postnatal injury. According to this view, the pathogenesis of BPD is multifactorial and heterogeneous with different patterns of antenatal stress (endotypes) that combine with varying postnatal insults and might distinctively damage the development of airways, lung parenchyma, interstitium, lymphatic system, and pulmonary vasculature. This results in different clinical phenotypes of BPD. There is no clear consensus on which are the endotypes of prematurity but the combination of clinical information with placental and bacteriological data enables the identification of two main pathways leading to birth before 32 weeks of gestation: (1) infection/inflammation and (2) dysfunctional placentation. Regarding BPD phenotypes, the following have been proposed: parenchymal, peripheral airway, central airway, interstitial, congestive, vascular, and mixed phenotype. In line with the approach of personalized medicine, endotyping prematurity and phenotyping BPD will facilitate the design of more targeted therapeutic and prognostic approaches.
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- 2022
5. Idiopathic pulmonary fibrosis: Current knowledge, future perspectives and its importance in radiation oncology
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Stéphanie Peeters, Görkem Türkkan, Hester A. Gietema, Lizza E.L. Hendriks, Cristina Mitea, Rémy Mostard, Yves Willems, Lennart Conemans, and Dirk De Ruysscher
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medicine.medical_specialty ,Lung Neoplasms ,Pulmonary toxicity ,IMPACT ,medicine.medical_treatment ,Radiation induced lung injury ,Disease ,Pathogenesis ,MUC5B PROMOTER POLYMORPHISM ,INTERSTITIAL LUNG-DISEASE ,THERAPY ,030218 nuclear medicine & medical imaging ,03 medical and health sciences ,Idiopathic pulmonary fibrosis ,0302 clinical medicine ,medicine ,Humans ,Radiology, Nuclear Medicine and imaging ,Risk factor ,Intensive care medicine ,Contraindication ,Lung ,Radiotherapy ,Toxicity ,business.industry ,MORTALITY ,Cancer ,Hematology ,Lung Injury ,respiratory system ,medicine.disease ,CANCER ,humanities ,Idiopathic Pulmonary Fibrosis ,respiratory tract diseases ,Radiation therapy ,Oncology ,Radiation-induced lung injury ,ACUTE EXACERBATION ,030220 oncology & carcinogenesis ,SURVIVAL ,Radiation Oncology ,business - Abstract
Idiopathic pulmonary fibrosis (IPF) is a progressive, fibrotic lung disease with an unknown cause. Uncertainties still remain regarding the pathogenesis of IPF, and the prognosis of this disease is poor despite some recent improvements in treatment. Radiation induced lung injury (RILI) is a common complication and a dose-limiting toxicity of thoracic radiotherapy. Importantly, IPF is a crucial risk factor for pulmonary toxicity after thoracic radiotherapy. Although IPF is not universally accepted as a definite contraindication for thoracic radiotherapy at present, it has been shown that IPF can increase the risk of severe and fatal complications after thoracic radiotherapy. Proton beam therapy has shown promising results in reducing the incidence of thoracic radiotherapy related life-threatening complications in IPF patients, but the current evidence is not sufficient to recommend the standard use of it. Many similarities are noticeable between IPF and RILI in terms of pathogenesis and underlying mechanisms. Better understanding of the mechanisms of IPF and RILI may enable clinicians to provide safer and more effective thoracic radiotherapy treatments in cancer patients with IPF. In this review, we summarize the current knowledge of IPF, present the importance of IPF in radiation oncology practice, and highlight the similarities and relationship between IPF and RILI. (C) 2020 Elsevier B.V. All rights reserved.
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- 2020
6. Influence of MUC5B gene on antisynthetase syndrome
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Fernanda Hernandez-Gonzalez, Verónica Mijares, Leticia Lera-Gómez, Albert Selva-O'Callaghan, Julia Martínez-Barrio, Francisco Javier López-Longo, Sergio Prieto-González, Alicia De Pablo Gafas, Antonio Mera-Varela, Javier Narváez, Santos Castañeda, Ignacio Grafia, Jaime Calvo-Alén, María Aránzazu Alfranca González, Virginia Pérez, Gema Bonilla, Sonia María Fernández Rozas, Víctor Manuel Mora Cuesta, Nair Pérez Gómez, José M. Cifrián, Raquel López-Mejías, María Piedad Usetti, Miguel A. González-Gay, Olga Sánchez-Pernaute, Laura Nuño, Lorenzo Cavagna, Rosalía Laporta, Sara Remuzgo-Martínez, Fredeswinda Romero-Bueno, Verónica Pulito-Cueto, Oreste Gualillo, Ernesto Trallero-Araguás, Alejandro Balsa, Fernanda Genre, Javier Llorca, David Iturbe Fernández, Norberto Ortego-Centeno, European Commission, Universidad de Cantabria, UAM. Departamento de Medicina, Instituto de Investigación Sanitaria Fundación Jiménez Díaz (ISS-FJD), Instituto de Investigación Sanitaria Hospital Universitario de La Princesa (IIS-IP), and Instituto de Investigación Sanitaria Fundación Jiménez Díaz (IIS-FJD)
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Male ,Autoimmune diseases ,lcsh:Medicine ,Antisynthetase syndrome ,pulmonary-fibrosis ,Gastroenterology ,Idiopathic pulmonary fibrosis ,Rheumatic diseases ,0302 clinical medicine ,Usual interstitial pneumonia ,Pulmonary fibrosis ,Medicine ,030212 general & internal medicine ,Promoter Regions, Genetic ,lcsh:Science ,Multidisciplinary ,pneumonias ,Malalties autoimmunitàries ,Incidence ,Interstitial lung disease ,Fibrosi pulmonar ,cohort ,Middle Aged ,respiratory system ,Mucin-5B ,3. Good health ,Rheumatoid arthritis ,Cohort ,Female ,Hypersensitivity pneumonitis ,Adult ,medicine.medical_specialty ,Medicina ,interstitial lung-disease ,pattern ,Polymorphism, Single Nucleotide ,behavioral disciplines and activities ,Article ,03 medical and health sciences ,Rheumatology ,systemic-sclerosis ,Internal medicine ,Humans ,features ,030203 arthritis & rheumatology ,Myositis ,business.industry ,lcsh:R ,mucins ,medicine.disease ,respiratory tract diseases ,promoter polymorphism ,body regions ,lcsh:Q ,Lung Diseases, Interstitial ,business ,Follow-Up Studies - Abstract
MUC5B rs35705950 (G/T) is strongly associated with idiopathic pulmonary fibrosis (IPF) and also contributes to the risk of interstitial lung disease (ILD) in rheumatoid arthritis (RA-ILD) and chronic hypersensitivity pneumonitis (CHP). Due to this, we evaluated the implication of MUC5B rs35705950 in antisynthetase syndrome (ASSD), a pathology characterised by a high ILD incidence. 160 patients with ASSD (142 with ILD associated with ASSD [ASSD-ILD+]), 232 with ILD unrelated to ASSD (comprising 161 IPF, 27 RA-ILD and 44 CHP) and 534 healthy controls were genotyped. MUC5B rs35705950 frequency did not significantly differ between ASSD-ILD+ patients and healthy controls nor when ASSD patients were stratified according to the presence/absence of anti Jo-1 antibodies or ILD. No significant differences in MUC5B rs35705950 were also observed in ASSD-ILD+ patients with a usual interstitial pneumonia (UIP) pattern when compared to those with a non-UIP pattern. However, a statistically significant decrease of MUC5B rs35705950 GT, TT and T frequencies in ASSD-ILD+ patients compared to patients with ILD unrelated to ASSD was observed. In summary, our study does not support a role of MUC5B rs35705950 in ASSD. It also indicates that there are genetic differences between ILD associated with and that unrelated to ASSD, This study was partially supported by grants from the Foundation for Research in Rheumatology (FOREUM). RL-M is a recipient of a Miguel Servet type I programme fellowship from the ‘Instituto de Salud Carlos III’ (ISCIII), cofunded by the European Social Fund (ESF, ‘Investing in your future’) (grant CP16/00033). SR-M is supported by funds of the RETICS Program (RD16/0012/0009), co-funded by the European Regional Development Fund (ERDF). VP-C is supported by a pre-doctoral grant from IDIVAL (PREVAL 18/01). VM is supported by funds of a Miguel Servet type I programme (grant CP16/00033) (ISCIII, co-funded by ESF). LL-G is supported by funds of PI18/00042 (ISCIII, co-funded by ERDF). OG is Staff Personnel of Xunta de Galicia (Servizo Galego de Saude, SERGAS) through a research-staff stabilization contract (ISCIII/SERGAS). OG,is member of RETICS Programme, RD16/0012/0014 (RIER: Red de Investigación en Inflamación y Enfermedades Reumáticas) via Instituto de Salud Carlos III (ISCIII) and FEDER. The work of OG (PI17/00409), was funded by Instituto de Salud Carlos III and FEDER. OG is a beneficiary of a project funded by Research Executive Agency of the European Union in the framework of MSCA-RISE Action of the H2020 Programme (Project number 734899). OG is beneficiary of a grant funded by Xunta de Galicia, Consellería de Educación, Universidade e Formación Profesional and Consellería de Economía, Emprego e Industria (GAIN), GPC IN607B2019/10
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- 2020
7. Pharmacological treatments for SSc-ILD: Systematic review and critical appraisal of the evidence
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James R. Seibold, Vanessa Smith, Francesco Del Galdo, Petros P. Sfikakis, Madelon C. Vonk, and Maurizio Cutolo
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medicine.medical_specialty ,Combination therapy ,Immunology ,Interstitial lung disease ,INTERSTITIAL LUNG-DISEASE ,PLACEBO-CONTROLLED TRIAL ,chemistry.chemical_compound ,Tocilizumab ,QUALITY-OF-LIFE ,Medicine and Health Sciences ,medicine ,Humans ,Immunology and Allergy ,SCLERODERMA LUNG ,Intensive care medicine ,MYCOPHENOLATE-MOFETIL ,Cyclophosphamide ,ORAL CYCLOPHOSPHAMIDE ,Evidence ,Scleroderma, Systemic ,HIGH-DOSE PREDNISOLONE ,business.industry ,PULMONARY-FUNCTION ,STEM-CELL TRANSPLANTATION ,Evidence-based medicine ,INTRAVENOUS PULSE CYCLOPHOSPHAMIDE ,Treatment ,Clinical trial ,Critical appraisal ,Systematic review ,chemistry ,Inflammatory diseases Radboud Institute for Health Sciences [Radboudumc 5] ,Systemic sclerosis ,Nintedanib ,Observational study ,Antifibrotic Agents ,Lung Diseases, Interstitial ,business ,Immunosuppressive Agents - Abstract
Contains fulltext : 244673.pdf (Publisher’s version ) (Open Access) Many therapies have been investigated for systemic sclerosis-associated interstitial lung disease (SSc-ILD), including immunosuppressive therapies, antifibrotic agents, immunomodulators and monoclonal antibodies. There is a high unmet medical need to better understand the current evidence for treatment efficacy and safety. This systematic review aims to present the existing literature on different drug treatments investigated for SSc-ILD and to critically assess the level of evidence for these drugs. A systematic review was performed in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. A structured literature search was performed for clinical trials and observational studies on the treatment of SSc-ILD with pharmaceutical interventions from 1 January 1990 to 15 December 2020. The quality of each reference was assessed using the Grades of Recommendation, Assessment, Development, and Evaluation (GRADE) criteria. A total of 77 references were reviewed and 13 different treatments were identified. We found high-quality evidence for the use of cyclophosphamide, nintedanib, mycophenolate and tocilizumab. Therefore, we would posit that the clinical community has four valid options for treatment of SSc-ILD. Further research is mandatory to provide more evidence for the optimal treatment strategy in SSc-ILD, including the optimal time to initiate treatment, selection of patients for treatment and upfront combination therapy.
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- 2021
8. Clinical practice guidelines for laboratory diagnosis of epidermolysis bullosa
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Maria C. Bolling, K. Wertheim‐Tysarowska, P. C. van den Akker, G. Pohla-Gubo, M. El Hachem, Giovanna Zambruno, S. Büchel, Cristina Has, R. Hiremagalore, María José Escámez, Ignacia Fuentes, A. Charlesworth, Lin Liu, and Translational Immunology Groningen (TRIGR)
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medicine.medical_specialty ,CHILEAN PATIENTS ,Genetic counseling ,Dermatology ,Immunofluorescence Microscopy ,INTERSTITIAL LUNG-DISEASE ,MOLECULAR DIAGNOSIS ,Skin fragility ,SEQUENCE VARIANTS ,Medicine ,Humans ,PRENATAL-DIAGNOSIS ,Biología y Biomedicina ,CAUSES KINDLER SYNDROME ,Ingeniería Mecánica ,GLYCINE SUBSTITUTION ,TRANSMISSION ELECTRON-MICROSCOPY ,business.industry ,Clinical Laboratory Techniques ,Disease classification ,medicine.disease ,IMMUNOFLUORESCENCE MICROSCOPY ,Clinical Practice ,EXTRACUTANEOUS MANIFESTATIONS ,Epidermolysis bullosa ,business ,Genetic diagnosis ,Epidermolysis Bullosa - Abstract
This guideline were initiated by DEBRA International; financial support was provided by DEBRA Austria. The generous assistance of Rebecca Bodan, Lisa Brains, Sharon Cassidy and Kelsey Townsend-Miller is gratefully acknowledged in providing patient or lay input into this guideline. The authors acknowledge the guidance of Kattya Mayre-Chilton (DEDRA International). Johann Bauer (Paracelsus University and EB House, Salzburg, Austria), Christine Bodemer (Hôpital Universitaire Necker, Paris, France), Judith Fischer (Institute of Human Genetics, University of Freiburg, Germany), Jemima Mellerio (St John's Institute of Dermatology, Guy's and St Thomas' NHS Foundation Trust, London, U.K.), Francis Palisson (Universidad del Desarrollo and DEBRA, Chile), Eli Sprecher (Department of Dermatology, Tel Aviv Sourasky Medical Center, Israel) and Jouni Uitto, Leila Youssefian and Hassan Vahidnezhad (all from the Department of Dermatology and Cutaneous Biology, Thomas Jefferson University, Philadelphia, PA, U.S.A.) are acknowledged as reviewers.
- Published
- 2019
9. Gaps in care of patients living with pulmonary fibrosis: a joint patient and expert statement on the results of a Europe-wide survey
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Francesco Bonella, Antje Prasse, Elisabetta Balestro, Ron Flewett, Michel Viegas, Marlies S. Wijsenbeek, Davide Biondini, Guenther Wanke, Wim A. Wuyts, Helmut Prosch, Benjamin Bondue, Maria Molina-Molina, Steve Jones, Vincent Cottin, Catharina C. Moor, Michael Kreuter, Lurdes Planas-Cerezales, Liam Galvin, Anne-Marie Russell, and Pulmonary Medicine
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Pulmonary and Respiratory Medicine ,medicine.medical_specialty ,IMPACT ,Respiratory System ,Psychological intervention ,MEDLINE ,lcsh:Medicine ,INTERSTITIAL LUNG-DISEASE ,NEEDS ,Interstitial Lung Disease ,Unmet needs ,PALLIATIVE CARE ,Idiopathic pulmonary fibrosis ,Quality of life ,STAGE ,Pulmonary fibrosis ,Patient experience ,medicine ,Intensive care medicine ,Science & Technology ,business.industry ,lcsh:R ,OF-LIFE ,Interstitial lung disease ,EARLY-DIAGNOSIS ,Original Articles ,respiratory system ,Sciences bio-médicales et agricoles ,medicine.disease ,respiratory tract diseases ,IPF ,CAREGIVERS ,business ,BURDEN ,Life Sciences & Biomedicine - Abstract
Introduction Pulmonary fibrosis (PF) and its most common form, idiopathic pulmonary fibrosis (IPF), are chronic, progressive diseases resulting in increasing loss of lung function and impaired quality of life and survival. The aim of this joint expert and patient statement was to highlight the most pressing common unmet needs of patients with PF/IPF, putting forward recommendations to improve the quality of life and health outcomes throughout the patient journey. Methods Two online surveys for patients and healthcare professionals (HCPs) were conducted by the European Idiopathic Pulmonary Fibrosis and Related Disorders Federation (EU-IPFF) in 14 European countries. Results The surveys were answered by 286 patients and 69 HCPs, including physicians and nurses. Delays in diagnosis and timely access to interstitial lung disease specialists and pharmacological treatment have been identified as important gaps in care. Additionally, patients and HCPs reported that a greater focus on symptom-centred management, adequate information, trial information and increasing awareness of PF/IPF is required. Conclusions The surveys offer important insights into the current unmet needs of PF/IPF patients. Interventions at different points of the care pathway are needed to improve patient experience., This joint expert and patient statement highlights the most pressing common unmet needs of patients with pulmonary fibrosis, and puts forward recommendations to improve the quality of life and health outcomes throughout the patient journey http://bit.ly/34cTOeo
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- 2019
10. Cough in idiopathic pulmonary fibrosis
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Surinder S. Birring, Mirjam J.G. van Manen, Anne-Marie Russell, Vincent Cottin, Carlo Vancheri, Marlies S. Wijsenbeek, Elisabetta A. Renzoni, and Pulmonary Medicine
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Pulmonary and Respiratory Medicine ,medicine.medical_specialty ,PROTEIN EXPRESSION ,PATHOGENESIS ,Comorbidity ,INTERSTITIAL LUNG-DISEASE ,CONTROLLED-TRIAL ,GASTROESOPHAGEAL-REFLUX ,PERSISTENT COUGH ,03 medical and health sciences ,Idiopathic pulmonary fibrosis ,0302 clinical medicine ,Quality of life ,Risk Factors ,URGE-TO-COUGH ,OBSTRUCTIVE SLEEP-APNEA ,medicine ,Animals ,Humans ,030212 general & internal medicine ,Intensive care medicine ,OBSTRUCTIVE SLEEP-APNEA, INTERSTITIAL LUNG-DISEASE ,REFRACTORY CHRONIC COUGH, URGE-TO-COUGH, GASTROESOPHAGEAL-REFLUX, PROTEIN EXPRESSION, PERSISTENT COUGH, CONTROLLED-TRIAL, PATHOGENESIS, PIRFENIDONE ,lcsh:RC705-779 ,business.industry ,PIRFENIDONE ,lcsh:Diseases of the respiratory system ,respiratory system ,Prognosis ,medicine.disease ,Idiopathic Pulmonary Fibrosis ,REFRACTORY CHRONIC COUGH ,respiratory tract diseases ,Clinical trial ,Antitussive Agents ,Chronic cough ,Chronic disease ,Cough ,030228 respiratory system ,Chronic Disease ,Quality of Life ,Physical therapy ,medicine.symptom ,business - Abstract
Many patients with idiopathic pulmonary fibrosis (IPF) complain of chronic refractory cough. Chronic cough is a distressing and disabling symptom with a major impact on quality of life. During recent years, progress has been made in gaining insight into the pathogenesis of cough in IPF, which is most probably “multifactorial” and influenced by mechanical, biochemical and neurosensory changes, with an important role for comorbidities as well. Clinical trials of cough treatment in IPF are emerging, and cough is increasingly included as a secondary end-point in trials assessing new compounds for IPF. It is important that such studies include adequate end-points to assess cough both objectively and subjectively. This article summarises the latest insights into chronic cough in IPF. It describes the different theories regarding the pathophysiology of cough, reviews the different methods to assess cough and deals with recent and future developments in the treatment of cough in IPF.
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- 2016
11. Systemic sclerosis: state of the art on clinical practice guidelines
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Jeska K de Vries-Bouwstra, Jacob M van Laar, Ronald F van Vollenhoven, Els Vandecasteele, Alexandre E. Voskuyl, Charissa Frank, Tobias Alexander, Gemma Lepri, Fonseca João Eurico, Eric Hachulla, Angela Tincani, Alexis Mathian, Elisabetta Zanatta, Veronica Codullo, Alberto Sulli, Luc Mouthon, Marco Matucci-Cerinic, Vanessa Smith, Amber Vanhaecke, Gerd R Burmester, Marie Vanthuyne, Frank J. A. van den Hoogen, D. Launay, Matthias Schneider, Maurizio Cutolo, Rosaria Talarico, Yannick Allanore, Ilaria Galetti, Frédéric Houssiau, Virgil A. S. H. Dalm, Alessandra Della Rossa, Cosimo Bruni, Carlo Alberto Scirè, Ulf Mueller-Ladner, Oliver Distler, Stefano Bombardieri, Paolo Airò, Barbara Ruaro, Marta Mosca, Ana Rita Vieira, Immunology, Internal Medicine, Smith, V, Scire, C, Talarico, R, Airo, P, Alexander, T, Allanore, Y, Bruni, C, Codullo, V, Dalm, V, De Vries-Bouwstra, J, Della Rossa, A, Distler, O, Galetti, I, Launay, D, Lepri, G, Mathian, A, Mouthon, L, Ruaro, B, Sulli, A, Tincani, A, Vandecasteele, E, Vanhaecke, A, Vanthuyne, M, Van Den Hoogen, F, Van Vollenhoven, R, Voskuyl, A, Zanatta, E, Bombardieri, S, Burmester, G, Eurico, F, Frank, C, Hachulla, E, Houssiau, F, Mueller-Ladner, U, Schneider, M, Van Laar, J, Vieira, A, Cutolo, M, Mosca, M, Matucci-Cerinic, M, Scirè, Ca, Van den Hoogen, F, Voskuyl, Ae, Eurico, Fj, van Laar, Jm, and Matucci-Cerinic, M.
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ERN ReCONNET ,European reference networks ,clinical practice guidelines ,nailfold videocapillaroscopy ,systemic sclerosis ,unmet needs ,European reference network ,Disease ,INTERSTITIAL LUNG-DISEASE ,RECOMMENDATIONS ,DEVELOPING CRITERIA ,PRACTICE PATHWAY ,High morbidity ,0302 clinical medicine ,Fibrosis ,Medicine and Health Sciences ,EXPERT CONSENSUS ,Immunology and Allergy ,030212 general & internal medicine ,skin and connective tissue diseases ,integumentary system ,Interstitial lung disease ,unmet need ,Clinical Practice ,medicine.symptom ,systemic sclerosi ,clinical practice guideline ,medicine.medical_specialty ,Immunology ,Systemic Sclerosis ,NO ,Pharmacological treatment ,Unmet needs ,03 medical and health sciences ,Rheumatology ,medicine ,SIMPLE CAPILLAROSCOPIC DEFINITIONS ,Intensive care medicine ,030203 arthritis & rheumatology ,business.industry ,STEM-CELL TRANSPLANTATION ,medicine.disease ,Sexual dysfunction ,SKIN ULCERS ,FUNCTIONAL DISABILITY ,clinical practice guidelines, ERN ReCONNET, European reference networks, nailfold videocapillaroscopy, systemic sclerosis, unmet needs ,POINTS ,business - Abstract
Systemic sclerosis (SSc) is an orphan disease characterised by autoimmunity, fibrosis of the skin and internal organs, and vasculopathy. SSc may be associated with high morbidity and mortality. In this narrative review we summarise the results of a systematic literature research, which was performed as part of the European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases project, aimed at evaluating existing clinical practice guidelines or recommendations. Only in the domains ‘Vascular & Ulcers’ (ie, non-pharmacological approach to digital ulcer), ‘PAH’ (ie, screening and treatment), ‘Treatment’ and ‘Juveniles’ (ie, evaluation of juveniles with Raynaud’s phenomenon) evidence-based and consensus-based guidelines could be included. Hence there is a preponderance of unmet needs in SSc referring to the diagnosis and (non-)pharmacological treatment of several SSc-specific complications. Patients with SSc experience significant uncertainty concerning SSc-related taxonomy, management (both pharmacological and non-pharmacological) and education. Day-to-day impact of the disease (loss of self-esteem, fatigue, sexual dysfunction, and occupational, nutritional and relational problems) is underestimated and needs evaluation.
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- 2018
12. Treatment of idiopathic pulmonary fibrosis: a position paper from a Nordic expert group
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G. H. Gudmundsson, Tone Sjåheim, C. M. Skold, Ole Hilberg, Alan Altraja, Elisabeth Bendstrup, Marjukka Myllärniemi, Gerardo Ferrara, and Riitta Kaarteenaho
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Indoles ,medicine.medical_treatment ,Placebo-controlled study ,GASTROESOPHAGEAL-REFLUX THERAPY ,INTERSTITIAL LUNG-DISEASE ,PLACEBO-CONTROLLED TRIAL ,law.invention ,chemistry.chemical_compound ,Idiopathic pulmonary fibrosis ,DOUBLE-BLIND ,0302 clinical medicine ,Randomized controlled trial ,law ,nintedanib ,idiopathic interstitial pneumonias ,lung transplantation ,pirfenidone ,pulmonary rehabilitation ,Internal Medicine ,030212 general & internal medicine ,Anti-Inflammatory Agents, Non-Steroidal ,Interstitial lung disease ,Pirfenidone ,respiratory system ,RANDOMIZED CONTROLLED-TRIAL ,3. Good health ,Nintedanib ,Algorithms ,medicine.drug ,medicine.medical_specialty ,LONG-TERM ,Pyridones ,INHALED N-ACETYLCYSTEINE ,INTERNATIONAL SOCIETY ,03 medical and health sciences ,medicine ,Humans ,Pulmonary rehabilitation ,GENOME-WIDE ASSOCIATION ,Intensive care medicine ,business.industry ,medicine.disease ,Idiopathic Pulmonary Fibrosis ,respiratory tract diseases ,Clinical trial ,030228 respiratory system ,chemistry ,Physical therapy ,business ,FORCED VITAL CAPACITY - Abstract
Idiopathic pulmonary fibrosis (IPF) is a fatal progressive lung disease occurring in adults. In the last decade, the results of a number of clinical trials based on the updated disease classification have been published. The registration of pirfenidone and nintedanib, the first two pharmacological treatment options approved for IPF, marks a new chapter in the management of patients with this disease. Other nonpharmacological treatments such as lung transplantation, rehabilitation and palliation have also been shown to be beneficial for these patients. In this review, past and present management is discussed based on a comprehensive literature search. A treatment algorithm is presented based on available evidence and our overall clinical experience. In addition, unmet needs with regard to treatment are highlighted and discussed. We describe the development of various treatment options for IPF from the first consensus to recent guidelines based on evidence from large-scale, multinational, randomized clinical trials, which have led to registration of the first drugs for IPF.
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- 2016
13. Clinical Genetics for the Pulmonologist: Introduction
- Author
-
Vincent Cottin, Rétrovirus et Pathologie Comparée (RPC), Institut National de la Recherche Agronomique (INRA)-École pratique des hautes études (EPHE), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Ecole Nationale Vétérinaire de Lyon (ENVL), and Centre Hospitalier Universitaire de Lyon
- Subjects
Pulmonary and Respiratory Medicine ,medicine.medical_specialty ,Genetics, Medical ,[SDV]Life Sciences [q-bio] ,Respiratory Tract Diseases ,INTERSTITIAL LUNG-DISEASE ,SUSCEPTIBILITY ,03 medical and health sciences ,0302 clinical medicine ,PULMONARY ARTERIOVENOUS-MALFORMATIONS ,Pulmonary Medicine ,Humans ,FIBROSIS ,Medicine ,Genetic Predisposition to Disease ,030212 general & internal medicine ,Intensive care medicine ,MUTATION ,ComputingMilieux_MISCELLANEOUS ,ALPHA(1)-ANTITRYPSIN ,IMMUNODEFICIENCY ,RECEPTOR ,business.industry ,Pulmonologist ,POLYMORPHISM ,DEFICIENCY ,030228 respiratory system ,Evolutionary biology ,Medical genetics ,business - Abstract
International audience
- Published
- 2005
14. Clinical evaluation of lymphocyte sub-populations and oxygen radical production in sarcoidosis and idiopathic pulmonary fibrosis
- Author
-
R. Aalbers, D. S. Postma, T.W. van der Mark, Gh Koeter, Hendricus Groen, and M. Hamstra
- Subjects
Adult ,Male ,INTERFERON ,Pulmonary and Respiratory Medicine ,Pathology ,medicine.medical_specialty ,Free Radicals ,SUPEROXIDE ANION ,Lymphocytosis ,Neutrophils ,Pulmonary Fibrosis ,Vital Capacity ,PATHOGENESIS ,INTERSTITIAL LUNG-DISEASE ,METABOLISM ,BRONCHOALVEOLAR LAVAGE ,ACTIVATION ,Leukocyte Count ,Idiopathic pulmonary fibrosis ,Sarcoidosis, Pulmonary ,Forced Expiratory Volume ,Humans ,Medicine ,Lung volumes ,CELL ,ALVEOLAR MACROPHAGES ,Lung ,medicine.diagnostic_test ,business.industry ,Total Lung Capacity ,Respiratory disease ,Interstitial lung disease ,Middle Aged ,GAMMA ,respiratory system ,medicine.disease ,Lymphocyte Subsets ,respiratory tract diseases ,Oxygen ,Bronchoalveolar lavage ,medicine.anatomical_structure ,Female ,Sarcoidosis ,medicine.symptom ,business ,Bronchoalveolar Lavage Fluid - Abstract
The purpose of this study was to investigate the relationship between bronchoalveolar lavage (BAL)-derived parameters of interstitial lung disease and clinical and lung function parameters in 34 patients with sarcoidosis and 23 patients with idiopathic pulmonary fibrosis (IPF). BAL findings of healthy individuals served as controls. Cell content and differentiation of BAL fluid were determined. Oxygen radical (O2-) production of BAL cells and of blood polymorphonuclear (PMN) cells was measured. Phenotypes of lung and blood lymphocytes were determined by immunoperoxidase staining. In addition, lung function was assessed, chest X-rays were made and serum ACE was measured. Lymphocyte alveolitis in sarcoidosis was associated with increased alveolar macrophage (AM) O2- production (P < 0.025 vs. sarcoidosis with normal lymphocyte counts). Patients with extrapulmonary sarcoidosis had higher CD4/CD8 ratios in BAL (P < 0.025) and shorter disease duration (P < 0.01) than those with strictly pulmonary sarcoidosis. Disease duration in sarcoidosis correlated inversely with the number of BAL cells (r = -0.38, P < 0.05), the relative and absolute number of lymphocytes in BAL fluid (r = -0.34, P < 0.05 and r = -0.44, P < 0.01, respectively) and the percentage of CD4-positive cells and the CD4/CD8 ratio (r = -0.43, P < 0.05 and r = -0.48, P < 0.025, respectively). Although significant increases in O2- production by BAL cells were observed in both IPF and sarcoidosis, only in sarcoidosis was a higher AM O2- production associated with a significantly lower total lung capacity (r = -0.67, P < 0.005) and pulmonary diffusing capacity TLCO (r = -0.50, P < 0.05). In conclusion, our findings show that lung lymphocyte phenotypes differ among patients with pulmonary and extrapulmonary sarcoidosis and that O2- production is upregulated in active sarcoidosis. In addition, our findings suggest that different relationships between BAL data and lung function in patients with sarcoidosis and IPF may be explained by differences in disease duration. In IPF, disease duration is likely to be underestimated because of its insidious onset. In sarcoidosis, the presence of extrapulmonary symptoms, helpful to establish an early diagnosis, is associated with significant BAL lymphocytosis.
- Published
- 1994
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