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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Clinical features and natural history of interstitial pneumonia with autoimmune features: A single center experience
TL;DR: Patients with IPAF were predominately non-smoking women with high-resolution computed tomography scans that suggested NSIP, and their pulmonary physiology was stable, and during limited follow-up, no deaths were observed.
Journal ArticleDOI
Acute exacerbations of progressive-fibrosing interstitial lung diseases
Martin Kolb,Benjamin Bondue,Alberto Pesci,Yasunari Miyazaki,Jin Woo Song,Nitin Y. Bhatt,John T. Huggins,Justin M. Oldham,Maria Padilla,Jesse Roman,Shane Shapera +10 more
TL;DR: In this paper, the authors defined acute exacerbation as an acute, clinically significant respiratory deterioration, typically less than 1 month in duration, together with computerised tomography imaging showing new bilateral glass opacity and/or consolidation superimposed on a background pattern consistent with fibrosing ILDs.
Journal ArticleDOI
Fibroblast senescence in the pathology of idiopathic pulmonary fibrosis
David W Waters,Kaj E C Blokland,Kaj E C Blokland,Prabuddha S. Pathinayake,Janette K. Burgess,Steven E. Mutsaers,Cecilia M. Prêle,Michael Schuliga,Christopher Grainge,Darryl A. Knight +9 more
TL;DR: Activation of STAT3 has previously been shown to correlate with IPF progression and therefore is a potential molecular target to modify early-stage senescence and restore normal fibroblast function.
Journal ArticleDOI
Idiopathic Pulmonary Fibrosis: The Association between the Adaptive Multiple Features Method and Fibrosis Outcomes
Margaret L. Salisbury,David A. Lynch,Edwin J R van Beek,Ella A. Kazerooni,Junfeng Guo,Meng Xia,Susan Murray,Kevin J. Anstrom,Eric Yow,Fernando J. Martinez,Eric A. Hoffman,Kevin R. Flaherty +11 more
TL;DR: AMFM‐measured fibrosis is an automated adjunct to existing prognostic markers and may allow for study enrichment with subjects at increased disease progression risk and is independently associated with elevated hazard for disease progression.
Journal ArticleDOI
Pirfenidone attenuates bleomycin-induced pulmonary fibrosis in mice by regulating Nrf2/Bach1 equilibrium
TL;DR: Theraputic effects of PFD for IPF were involved in Nrf2/Bach1 equilibrium which regulated the capacity of oxidative stress, and provided new insights into the antioxidant mechanism of P FD.
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