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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Radiological diagnosis in lung disease: factoring treatment options into the choice of diagnostic modality.
TL;DR: The choice of radiologicalal technique for the detection, staging, follow-up, and quantification of lung disease should be based on the individual clinical options, so that appropriate treatment can be provided without excessive use of diagnostic testing.
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Clinical, serological and radiological features of a prospective cohort of Interstitial Pneumonia with Autoimmune Features (IPAF) patients.
Gianluca Sambataro,Domenico Sambataro,Sebastiano Emanuele Torrisi,Ada Vancheri,Michele Colaci,Mauro Pavone,Francesca Pignataro,Nicoletta Del Papa,Stefano Palmucci,Carlo Vancheri +9 more
TL;DR: In comparison with IPF, IPAF patients showed younger age, better performances in Pulmonary Function Tests, less necessity of O2 support and predominance of female sex and NSIP pattern, and seem to have a less severe lung disease than IPF.
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Normal Human Lung Epithelial Cells Inhibit Transforming Growth Factor-β Induced Myofibroblast Differentiation via Prostaglandin E2.
Amali P. Epa,Thomas H. Thatcher,Stephen J. Pollock,Lindsay A. Wahl,Elizabeth Lyda,R.M. Kottmann,Richard P. Phipps,Patricia J. Sime +7 more
TL;DR: The first direct experimental evidence that lung epithelial cells inhibit TGF-β induced myofibroblast differentiation and pro-fibrotic phenotypes in fibroblasts is provided, not restricted by tissue origin, and is mediated, at least in part, by PGE2.
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Fibulin-1 Predicts Disease Progression in Patients With Idiopathic Pulmonary Fibrosis
Jade Jaffar,Jade Jaffar,Jade Jaffar,Sofia Unger,Tamera J. Corte,Michael Keller,Paul J. Wolters,Luca Richeldi,Stefania Cerri,Cecilia M. Prêle,Philip M. Hansbro,William Scott Argraves,Rema A. Oliver,Brian G. Oliver,Judith L. Black,Janette K. Burgess,Janette K. Burgess,Janette K. Burgess +17 more
TL;DR: Fibulin-1 is a novel potential biomarker for disease progression in IPF and raises the possibility that it could be used as a target for the development of new treatments.
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Peptide-mediated inhibition of mitogen-activated protein kinase-activated protein kinase-2 ameliorates bleomycin-induced pulmonary fibrosis
Ragini Vittal,Amanda J. Fisher,Hongmei Gu,Elizabeth A. Mickler,Alyssa Panitch,Cynthia Lander,Oscar W. Cummings,George E. Sandusky,David S. Wilkes +8 more
TL;DR: The peptide-mediated inhibition of MK2 affects both inflammatory and fibrotic responses, and thus may offer a promising therapeutic target for IPF.
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