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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ADAM10-mediated ephrin-B2 shedding promotes myofibroblast activation and organ fibrosis.
David Lagares,Parisa Ghassemi-Kakroodi,Caroline Tremblay,Alba Santos,Clemens K. Probst,Alicia Franklin,Daniela M. Santos,Paula Grasberger,Neil Ahluwalia,Sydney B. Montesi,Barry S. Shea,Katharine E. Black,Rachel S. Knipe,Meryem Blati,Murray Baron,Brian Wu,Hassan Fahmi,Rajiv Gandhi,Annie Pardo,Moisés Selman,Jiangping Wu,Jean-Pierre Pelletier,Johanne Martel-Pelletier,Andrew M. Tager,Mohit Kapoor +24 more
TL;DR: This work provides molecular, functional and translational evidence that the ectodomain of membrane-bound ephrin-B2 is shed from fibroblasts into the alveolar airspace after lung injury and uncovers a new molecular mechanism of tissue fibrogenesis.
Journal ArticleDOI
MicroRNAs as potential targets for progressive pulmonary fibrosis.
TL;DR: Specific patterns of dysregulated miRNAs in patients with IPF are discussed, including involvement in regulating lung inflammation, TGF-β1-mediated EMT and fibroblast differentiation processes, ECM genes expression, and in the progression of lung fibrosis.
Journal ArticleDOI
Computer-Aided Diagnosis of Pulmonary Fibrosis Using Deep Learning and CT Images.
Andreas Christe,Alan A. Peters,Dionysios Drakopoulos,Johannes T. Heverhagen,Thomas Geiser,Thomai Stathopoulou,Stergios Christodoulidis,Marios Anthimopoulos,Stavroula Mougiakakou,Lukas Ebner +9 more
TL;DR: It is found that a computer-aided detection algorithm based on machine learning was able to classify idiopathic pulmonary fibrosis with similar accuracy to a human reader.
Journal ArticleDOI
PVDOMICS: A Multi-Center Study to Improve Understanding of Pulmonary Vascular Disease Through Phenomics
Anna R. Hemnes,Gerald J. Beck,John H. Newman,Aiden Abidov,Micheala A. Aldred,John Barnard,Erika B. Rosenzweig,Barry A. Borlaug,Wendy K. Chung,Suzy A. A. Comhair,Serpil C. Erzurum,Robert P. Frantz,Michael P. Gray,Gabriele Grunig,Paul M. Hassoun,Nicholas S. Hill,Evelyn M. Horn,Bo Hu,Jason K. Lempel,Bradley A. Maron,Bradley A. Maron,Stephen C. Mathai,Stephen C. Mathai,Mitchell A. Olman,Franz Rischard,David M. Systrom,W.H. Wilson Tang,Aaron B. Waxman,Lei Xiao,Jason X.-J. Yuan,Jane A. Leopold +30 more
TL;DR: The National Institutes of Health (NIH) and National Heart, Lung and Blood institute (NHLBI) launched an initiative, PVDOMICS, that aims to augment the current pulmonary hypertension (PH) classification based on shared biological features, with an updated, molecular classification of pulmonary vascular disease (PVD).
Journal ArticleDOI
Matrix Remodeling in Pulmonary Fibrosis and Emphysema
Tejaswini Kulkarni,Philip J. O'Reilly,Veena B. Antony,Amit Gaggar,Amit Gaggar,Victor J. Thannickal,Victor J. Thannickal +6 more
TL;DR: It is proposed that regulation of mesenchymal cells under the influence of soluble factors, in particular transforming growth factor-β1, and the extracellular matrix determine the divergent tissue remodeling responses seen in pulmonary fibrosis and emphysema.
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