Acute Exacerbation of Idiopathic Pulmonary Fibrosis. An International Working Group Report
Harold R. Collard,Christopher J. Ryerson,Tamera J. Corte,Gisli Jenkins,Y. Kondoh,David J. Lederer,Joyce S. Lee,Toby M. Maher,Athol U. Wells,Katerina M. Antoniou,Juergen Behr,Kevin K. Brown,Cottin,K. R. Flaherty,Junya Fukuoka,David M. Hansell,Takeshi Johkoh,Naftali Kaminski,Dong Soon Kim,Martin Kolb,David A. Lynch,J.L. Myers,Ganesh Raghu,Luca Richeldi,Hiroyuki Taniguchi,Fernando J. Martinez +25 more
TLDR
To better reflect the current state of knowledge and improve the feasibility of future research into its etiology and treatment, the working group proposes a new conceptual framework for acute respiratory deterioration in idiopathic pulmonary fibrosis and a revised definition and diagnostic criteria for acute exacerbation.Abstract:
Acute exacerbation of idiopathic pulmonary fibrosis has been defined as an acute, clinically significant, respiratory deterioration of unidentifiable cause. The objective of this international working group report on acute exacerbation of idiopathic pulmonary fibrosis was to provide a comprehensive update on the topic. A literature review was conducted to identify all relevant English text publications and abstracts. Evidence-based updates on the epidemiology, etiology, risk factors, prognosis, and management of acute exacerbations of idiopathic pulmonary fibrosis are provided. Finally, to better reflect the current state of knowledge and improve the feasibility of future research into its etiology and treatment, the working group proposes a new conceptual framework for acute respiratory deterioration in idiopathic pulmonary fibrosis and a revised definition and diagnostic criteria for acute exacerbation of idiopathic pulmonary fibrosis.read more
Citations
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Journal ArticleDOI
Diagnosis of Idiopathic Pulmonary Fibrosis. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline.
Ganesh Raghu,Martine Remy-Jardin,Jeffrey L. Myers,Luca Richeldi,Christopher J. Ryerson,David J. Lederer,Juergen Behr,Vincent Cottin,Sonye K. Danoff,Ferran Morell,Kevin R. Flaherty,Athol U. Wells,Fernando J. Martinez,Arata Azuma,Thomas Bice,Demosthenes Bouros,Kevin K. Brown,Harold R. Collard,Abhijit Duggal,Liam Galvin,Yoshikazu Inoue,R. Gisli Jenkins,Takeshi Johkoh,Ella A. Kazerooni,Masanori Kitaichi,Shandra L Knight,George Mansour,Andrew G. Nicholson,Sudhakar Pipavath,Ivette Buendía-Roldán,Moisés Selman,William D. Travis,Simon L.F. Walsh,Kevin C. Wilson +33 more
TL;DR: The guideline panel provided recommendations related to the diagnosis of IPF, including a conditional recommendation for multidisciplinary discussion and a strong recommendation against measurement of serum biomarkers for the sole purpose of distinguishing IPF from other ILDs.
Journal ArticleDOI
Nintedanib in Progressive Fibrosing Interstitial Lung Diseases
TL;DR: In patients with progressive fibrosing interstitial lung diseases, the annual rate of decline in the FVC was significantly lower among patients who received nintedanib than among those who received placebo.
Journal ArticleDOI
Idiopathic Pulmonary Fibrosis.
TL;DR: Idiopathic Pulmonary Fibrosis IdiopATHic pulmonary fibrosis appears to be increasing in incidence, and it requires early recognition and intervention with supportive care and pharmacologic agents to forestall its progression.
Journal ArticleDOI
Idiopathic pulmonary fibrosis
TL;DR: This disease has improved understanding of the mechanisms of lung fibrosis, and offers hope that similar approaches will transform the management of patients with other progressive fibrotic lung diseases.
Journal ArticleDOI
Idiopathic pulmonary fibrosis
Fernando J. Martinez,Harold R. Collard,Annie Pardo,Ganesh Raghu,Luca Richeldi,Moisés Selman,Jeffrey J. Swigris,Hiroyuki Taniguchi,Athol U. Wells +8 more
TL;DR: The biological processes underlying IPF are thought to reflect an aberrant reparative response to repetitive alveolar epithelial injury in a genetically susceptible ageing individual, although many questions remain on how to define susceptibility.
References
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Journal ArticleDOI
Acute respiratory distress syndrome: the Berlin Definition.
Ards Definition Task Force,V. Marco Ranieri,Gordon D. Rubenfeld,B. Taylor Thompson,Niall D. Ferguson,Ellen Caldwell,Eddy Fan,Luigi Camporota,Luigi Camporota,Arthur S. Slutsky +9 more
TL;DR: The updated and revised Berlin Definition for ARDS addresses a number of the limitations of the AECC definition and may serve as a model to create more accurate, evidence-based, critical illness syndrome definitions and to better inform clinical care, research, and health services planning.
Journal ArticleDOI
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
TL;DR: 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.
Journal ArticleDOI
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
TL;DR: In patients with idiopathic pulmonary fibrosis, nintedanib reduced the decline in FVC, which is consistent with a slowing of disease progression; nintinganib was frequently associated with diarrhea, which led to discontinuation of the study medication in less than 5% of patients.
Journal ArticleDOI
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
TL;DR: Pirfenidone, as compared with placebo, reduced disease progression, as reflected by lung function, exercise tolerance, and progression-free survival, in patients with idiopathic pulmonary fibrosis.
Idiopathic Pulmonary Fibrosis
TL;DR: The most common signs and symptoms of idiopathic pulmonary fibrosis are shortness of breath and a persistent dry, hacking cough; many affected individuals also experience a loss of appetite and gradual weight loss.
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