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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Rheumatoid arthritis-associated lung disease
TL;DR: A comprehensive, up-to-date review of RA-associated lung diseases including pathogenesis and management is published.
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Mortality prediction in idiopathic pulmonary fibrosis: evaluation of computer-based CT analysis with conventional severity measures.
Joseph Jacob,Brian J. Bartholmai,Srinivasan Rajagopalan,Maria Kokosi,Arjun Nair,Ronald A. Karwoski,Simon L.F. Walsh,Athol U. Wells,David M. Hansell +8 more
TL;DR: CALIPER-derived parameters, in particular PVV, are more accurate prognostically than traditional visual CT scores and have the potential to improve staging systems in IPF.
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The European IPF registry (eurIPFreg): baseline characteristics and survival of patients with idiopathic pulmonary fibrosis
Andreas Guenther,Ekaterina Krauss,Silke Tello,Jasmin Wagner,Bettina Paul,Stefan Kuhn,Olga Maurer,Sabine Heinemann,Ulrich Costabel,María Asunción Nieto Barbero,Veronika Müller,Philippe Bonniaud,Carlo Vancheri,Athol U. Wells,Martina Vasakova,Alberto Pesci,Matteo Sofia,Walter Klepetko,Werner Seeger,Fotios Drakopanagiotakis,Bruno Crestani +20 more
TL;DR: The data provide important insights into baseline characteristics, diagnostic and management changes as well as outcome data in European IPF patients over time.
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Management of patients with idiopathic pulmonary fibrosis in clinical practice: the INSIGHTS-IPF registry
Jürgen Behr,Michael Kreuter,Marius M. Hoeper,Hubert Wirtz,Jens Klotsche,Dirk Koschel,Stefan Andreas,Martin Claussen,Christian Grohé,H Wilkens,Winfried Randerath,Dirk Skowasch,F. Joachim Meyer,Joachim Kirschner,Sven Gläser,Felix J.F. Herth,Tobias Welte,Rudolf M. Huber,Claus Neurohr,Martin Schwaiblmair,Martin Kohlhäufl,Gert Höffken,Matthias Held,Andrea Koch,Thomas Bahmer,David Pittrow +25 more
TL;DR: This largest published registry of IPF patients shows surprising disease severity and treatment variation http://ow.ly/JbWRn
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Nintedanib with Add-on Pirfenidone in Idiopathic Pulmonary Fibrosis. Results of the INJOURNEY Trial.
Carlo Vancheri,Michael Kreuter,Luca Richeldi,Christopher J. Ryerson,Dominique Valeyre,Jan C. Grutters,Sabrina Wiebe,Wibke Stansen,Manuel Quaresma,Manuel Quaresma,Susanne Stowasser,Wim A. Wuyts +11 more
TL;DR: Nintedanib with add‐on pirfenidone had a manageable safety and tolerability profile in patients with IPF, in line with the adverse event profiles of each drug.
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