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
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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
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
TL;DR: This update is a supplement to the previous 2002 IIP classification document and outlines advances in the past decade and potential areas for future investigation.
Journal ArticleDOI
An official American thoracic society/European respiratory society statement: Key concepts and advances in pulmonary rehabilitation
Martijn A. Spruit,Sally J Singh,Chris Garvey,Richard ZuWallack,Linda Nici,Anne E Holland,Suzanne C. Lareau,Fabio Pitta,Louise Sewell,Jo Raskin,Jean Bourbeau,Rebecca Crouch,Frits M.E. Franssen,Richard Casaburi,Jan H. Vercoulen,Ioannis Vogiatzis,Rik Gosselink,Enrico Clini,Tanja Effing,Job van der Palen,Thierry Troosters,Daisy J.A. Janssen,Eileen G. Collins,Judith Garcia-Aymerich,Dina Brooks,Bonnie Fahy,Milo A. Puhan,Martine Hoogendoorn,Rachel Garrod,Annemie M. W. J. Schols,Brian W. Carlin,Roberto P. Benzo,Mike Morgan,Andrew L. Ries,Roger S. Goldstein,Claire A. Dowson,Jan Brozek,Claudio F. Donner,Emiel F.M. Wouters +38 more
TL;DR: The considerable growth in the science and application of pulmonary rehabilitation since 2006 adds further support for its efficacy in a wide range of individuals with chronic respiratory disease.
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
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.
References
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Journal ArticleDOI
Interstitial lung diseases: genetic predisposition and inherited interstitial lung diseases
Ganesh Raghu,Richard Hert +1 more
Journal Article
NOD2/CARD15 gene polymorphisms in idiopathic pulmonary fibrosis.
Michele Zorzetto,Ilaria Ferrarotti,Ilaria Campo,Rocco Trisolini,Poletti,Roberta Scabini,Michele Ceruti,Paola Mazzola,E. Crippa,Stefania Ottaviani,Carlo Agostini,Gianpietro Semenzato,Ernesto Pozzi,M. Luisetti +13 more
TL;DR: In this paper, the frequencies of the three major NOD2/CARD 15 gene mutations (R702W, G908R and 1007fsinC) in a series of 76 subjects affected by IPF were determined.
Journal Article
The 3' untranslated region of tumor necrosis factor-alpha is highly conserved in idiopathic pulmonary fibrosis (IPF).
TL;DR: It is thought that chronic inflammation contributes to the pathological process of IPF, and the inflammatory response is an essential component of host defence that is tightly regulated in vivo.
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Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis
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