An Official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-Based Guidelines for Diagnosis and Management
Ganesh Raghu,Harold R. Collard,Jim J. Egan,Fernando J. Martinez,Juergen Behr,Kevin K. Brown,Thomas V. Colby,Jean-François Cordier,Kevin R. Flaherty,Joseph A. Lasky,David A. Lynch,Jay H. Ryu,Jeffrey J. Swigris,Athol U. Wells,Julio Ancochea,Demosthenes Bouros,Carlos Roberto Ribeiro de Carvalho,Ulrich Costabel,Masahito Ebina,David M. Hansell,Takeshi Johkoh,Dong Soon Kim,Talmadge E. King,Yasuhiro Kondoh,Jeffrey L. Myers,Nestor L. Müller,Andrew G. Nicholson,Luca Richeldi,Moisés Selman,Rosalind F. Dudden,Barbara S. Griss,Shandra Protzko,Holger J. Schünemann +32 more
TLDR
This document represents the current state of knowledge regarding idiopathic pulmonary fibrosis, and contains sections on definition and epidemiology, risk factors, diagnosis, natural history, staging and prognosis, treatment, and monitoring disease course.Abstract:
This document is an international evidence-based guideline on the diagnosis and management of idiopathic pulmonary fibrosis, and is a collaborative effort of the American Thoracic Society, the European Respiratory Society, the Japanese Respiratory Society, and the Latin American Thoracic Association. It represents the current state of knowledge regarding idiopathic pulmonary fibrosis (IPF), and contains sections on definition and epidemiology, risk factors, diagnosis, natural history, staging and prognosis, treatment, and monitoring disease course. For the diagnosis and treatment sections, pragmatic GRADE evidence-based methodology was applied in a question-based format. For each diagnosis and treatment question, the committee graded the quality of the evidence available (high, moderate, low, or very low), and made a recommendation (yes or no, strong or weak). Recommendations were based on majority vote. It is emphasized that clinicians must spend adequate time with patients to discuss patients' values and preferences and decide on the appropriate course of action.read more
Citations
More filters
Journal ArticleDOI
Cell cycle dependence of ACE-2 explains downregulation in idiopathic pulmonary fibrosis
Bruce D. Uhal,MyTrang Dang,Vinh Dang,Roger Llatos,Esteban Cano,Amal Abdul-Hafez,Jonathan C. Markey,Christopher C. Piasecki,Maria Molina-Molina +8 more
TL;DR: Cell cycle-dependent regulation of this protective enzyme by a JNK-mediated transcriptional mechanism is demonstrated and explains the loss of ACE-2 in lung fibrosis.
Journal ArticleDOI
Pirfenidone for Idiopathic Pulmonary Fibrosis: A Systematic Review and Meta-Analysis
TL;DR: Pirfenidone treatment should be considered not only for its benefits in pulmonary function tests but also by its clinically relevant outcomes, such as reduction in all-cause mortality, IPF related mortality, worsening and exacerbation of IPF and PFS.
Journal ArticleDOI
German Guideline for Idiopathic Pulmonary Fibrosis – Update on Pharmacological Therapies 2017
Jürgen Behr,Andreas Günther,Francesco Bonella,K Geißler,Dirk Koschel,Michael Kreuter,Antje Prasse,Nicolas Schönfeld,H. Sitter,Joachim Müller-Quernheim,Ulrich Costabel +10 more
TL;DR: The German IPF guideline offered recommendations based on expert consensus for some open questions in the management of IPF patients for which firm evidence is lacking and antacid therapy as a general treatment for IPF was rated negative, based on conflicting results of recent analyses.
Journal ArticleDOI
Texture-Based Automated Quantitative Assessment of Regional Patterns on Initial CT in Patients With Idiopathic Pulmonary Fibrosis: Relationship to Decline in Forced Vital Capacity
TL;DR: RO of less than 22.05% in extent can accurately predict stable IPF at 1-year follow-up in terms of FVC on initial CT using texture-based automated quantification in patients with idiopathic pulmonary fibrosis.
Journal ArticleDOI
Idiopathic Pulmonary Fibrosis: Best Practice in Monitoring and Managing a Relentless Fibrotic Disease.
Wim A. Wuyts,Marlies S. Wijsenbeek,Benjamin Bondue,Demosthenes Bouros,P. Bresser,Carlos Robalo Cordeiro,Ole Hilberg,Jesper Magnusson,Effrosyni D. Manali,António Morais,Spyridon Papiris,Saher B. Shaker,Marcel Veltkamp,Elisabeth Bendstrup +13 more
TL;DR: Optimal management of IPF requires a multidimensional approach, including both pharmacological therapy to slow decline in lung function and supportive care to preserve patients’ quality of life.
References
More filters
Journal ArticleDOI
Standardisation of spirometry
Martin R. Miller,John L. Hankinson,Vito Brusasco,Felip Burgos,Richard Casaburi,Allan L. Coates,Robert O. Crapo,Paul L. Enright,C.P.M. van der Grinten,P. Gustafsson,R. Jensen,D.C. Johnson,Neil R. MacIntyre,Roy T. McKay,Daniel Navajas,O. F. Pedersen,Riccardo Pellegrino,Giovanni Viegi,J. Wanger +18 more
TL;DR: This research presents a novel and scalable approach called “Standardation of LUNG FUNCTION TESTing” that combines “situational awareness” and “machine learning” to solve the challenge of integrating nanofiltration into the energy system.
Journal ArticleDOI
Cancer statistics, 2007.
TL;DR: While the absolute number of cancer deaths decreased for the second consecutive year in the United States, much progress has been made in reducing mortality rates and improving survival, cancer still accounts for more deaths than heart disease in persons under age 85 years.
Journal ArticleDOI
Interpretative strategies for lung function tests
Riccardo Pellegrino,Giovanni Viegi,Vito Brusasco,Robert O. Crapo,Felip Burgos,Richard Casaburi,Allan L. Coates,C.P.M. van der Grinten,P. Gustafsson,John L. Hankinson,R. Jensen,D.C. Johnson,Neil R. MacIntyre,Roy T. McKay,Martin R. Miller,Daniel Navajas,O. F. Pedersen,J. Wanger +17 more
TL;DR: This section is written to provide guidance in interpreting pulmonary function tests (PFTs) to medical directors of hospital-based laboratories that perform PFTs, and physicians who are responsible for interpreting the results of PFTS most commonly ordered for clinical purposes.
Journal ArticleDOI
American thoracic society/European respiratory society international multidisciplinary consensus classification of the idiopathic interstitial pneumonias
William D. Travis,Talmadge E. King,Eric D. Bateman,David A. Lynch,Frédrique Capron,Thomas V. Colby,Jean-François Cordier,Roland M. Dubois,Jeffrey R. Galvin,Philippe Grenier,David M. Hansell,Gary W. Hunninghake,Masanori Kitaichi,Nestor L. Müller,Jeffrey L. Myers,Sonoko Nagai,Andrew G. Nicholson,Ganesh Raghu,Benoit Wallaert,Christian Brambilla,Kevin K. Brown,Andrew L. Cherniaev,Ulrich Costabel,David B. Coultas,Gerald S. Davis,Maurits G. Demedts,William W. Douglas,Jim J. Egan,Anders Eklund,Leonarda M. Fabbri,Craig A. Henke,Richard Hubbard,Y. Inoue,Takateru Izumi,H. M. Jansen,Ian Johnston,Dong Soon Kim,Nasreen Khalil,Fiona R. Lake,Giuseppe Lungarella,Joseph P. Lynch,Douglas W. Mapel,Fernando J. Martinez,Richard A. Matthay,Lee S. Newman,Paul W. Noble,Ken Ohta,Dario Olivieri,Luis A. Ortiz,Venerino Poletti,Robert Rodriguez-Roisin,William N. Rom,Jay Hoon Ryu,Paulo Hilário Nascimento Saldiva,Raúl H Sansores,Marvin L. Schwarz,Moisés Selman,Cecelia M. Smith,Zhaohui Tong,Zarir F Udwadia,Dominique Valeyre,Athol U. Wells,Robert A. Wise,Antonio Xaubet,Emilio Alvarez Fernandez,Elisabeth Brambilla,Vera Luiza Capelozzi,Andrew Cherniaev,Peter Dalquen,Gerhard Dekan,Philip S. Hasleton,James C. Hogg,N. A. Jambhekar,Anna Luise A Katzenstein,Michael Koss,Osamu Matsubara,Klaus Michael Müller,F. B.J.M. Thunnissen,James A. Waldron,Wei Hua Li,Paul J. Friedman,Martin Remy-Jardin,Theresa C. McLoud +82 more
TL;DR: The Diagnostic Process Is Dynamic Clinical Evaluation Radiological Evaluation Role of Surgical Lung Biopsy Unclassifiable Interstitial Pneumonia Bronchoalveolar Lavage Fluid Evaluation Idiopathic Pulmonary Fibrosis.
Journal ArticleDOI
Fleischner Society: Glossary of Terms for Thoracic Imaging
David M. Hansell,Alexander A. Bankier,Heber MacMahon,Theresa C. McLoud,Nestor L. Müller,J Remy +5 more
TL;DR: Members of the Fleischner Society compiled a glossary of terms for thoracic imaging that replaces previous glossaries published in 1984 and 1996 for Thoracic radiography and computed tomography, respectively.
Related Papers (5)
Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis
Luca Richeldi,Roland M. du Bois,Ganesh Raghu,Arata Azuma,Kevin K. Brown,Ulrich Costabel,Vincent Cottin,Kevin R. Flaherty,David M. Hansell,Yoshikazu Inoue,Dong Soon Kim,Martin Kolb,Andrew G. Nicholson,Paul W. Noble,Moisés Selman,Hiroyuki Taniguchi,Michèle Brun,Florence Le Maulf,Mannaig Girard,Susanne Stowasser,Rozsa Schlenker-Herceg,Bernd Disse,Harold R. Collard +22 more
A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis
Talmadge E. King,Williamson Z. Bradford,Socorro Castro-Bernardini,Elizabeth A. Fagan,Ian Glaspole,Marilyn K. Glassberg,Eduard Gorina,Peter Hopkins,David Kardatzke,Lisa Lancaster,David J. Lederer,Steven D. Nathan,Carlos Alberto de Castro Pereira,Steven A. Sahn,Robert Sussman,Jeffrey J. Swigris,Paul W. Noble +16 more
An Official American Thoracic Society/European Respiratory Society Statement: Update of the International Multidisciplinary Classification of the Idiopathic Interstitial Pneumonias
William D. Travis,Ulrich Costabel,David M Hansell,Talmadge E. King,David A. Lynch,Andrew G. Nicholson,Christopher J. Ryerson,Jay H. Ryu,Moisés Selman,Athol U. Wells,Jurgen Behr,Demosthenes Bouros,Kevin K. Brown,Thomas V. Colby,Harold R. Collard,Carlos Robalo Cordeiro,Vincent Cottin,Bruno Crestani,Marjolein Drent,Rosalind F. Dudden,Jim Egan,Kevin R. Flaherty,Cory M. Hogaboam,Yoshikazu Inoue,Takeshi Johkoh,Dong Soon Kim,M Kitaichi,James E. Loyd,Fernando J. Martinez,Jeffrey L. Myers,Shandra Protzko,Ganesh Raghu,Luca Richeldi,Nicola Sverzellati,Jeffrey J. Swigris,Dominique Valeyre +35 more
American thoracic society/European respiratory society international multidisciplinary consensus classification of the idiopathic interstitial pneumonias
William D. Travis,Talmadge E. King,Eric D. Bateman,David A. Lynch,Frédrique Capron,Thomas V. Colby,Jean-François Cordier,Roland M. Dubois,Jeffrey R. Galvin,Philippe Grenier,David M. Hansell,Gary W. Hunninghake,Masanori Kitaichi,Nestor L. Müller,Jeffrey L. Myers,Sonoko Nagai,Andrew G. Nicholson,Ganesh Raghu,Benoit Wallaert,Christian Brambilla,Kevin K. Brown,Andrew L. Cherniaev,Ulrich Costabel,David B. Coultas,Gerald S. Davis,Maurits G. Demedts,William W. Douglas,Jim J. Egan,Anders Eklund,Leonarda M. Fabbri,Craig A. Henke,Richard Hubbard,Y. Inoue,Takateru Izumi,H. M. Jansen,Ian Johnston,Dong Soon Kim,Nasreen Khalil,Fiona R. Lake,Giuseppe Lungarella,Joseph P. Lynch,Douglas W. Mapel,Fernando J. Martinez,Richard A. Matthay,Lee S. Newman,Paul W. Noble,Ken Ohta,Dario Olivieri,Luis A. Ortiz,Venerino Poletti,Robert Rodriguez-Roisin,William N. Rom,Jay Hoon Ryu,Paulo Hilário Nascimento Saldiva,Raúl H Sansores,Marvin L. Schwarz,Moisés Selman,Cecelia M. Smith,Zhaohui Tong,Zarir F Udwadia,Dominique Valeyre,Athol U. Wells,Robert A. Wise,Antonio Xaubet,Emilio Alvarez Fernandez,Elisabeth Brambilla,Vera Luiza Capelozzi,Andrew Cherniaev,Peter Dalquen,Gerhard Dekan,Philip S. Hasleton,James C. Hogg,N. A. Jambhekar,Anna Luise A Katzenstein,Michael Koss,Osamu Matsubara,Klaus Michael Müller,F. B.J.M. Thunnissen,James A. Waldron,Wei Hua Li,Paul J. Friedman,Martin Remy-Jardin,Theresa C. McLoud +82 more