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
Daily Home Spirometry: An Effective Tool for Detecting Progression in Idiopathic Pulmonary Fibrosis.
Anne-Marie Russell,Huzaifa Adamali,Philip L. Molyneaux,Philip L. Molyneaux,Pauline T. Lukey,Richard P. Marshall,Elisabetta A. Renzoni,Elisabetta A. Renzoni,Athol U. Wells,Athol U. Wells,Toby M. Maher,Toby M. Maher +11 more
TL;DR: Measurement of daily home spirometry in patients with IPF is highly clinically informative and is feasible to perform for most of these patients, and suggests that daily FVC may be of value as a primary endpoint in short proof-of-concept IPF studies.
Journal ArticleDOI
Idiopathic pulmonary fibrosis: Diagnosis, epidemiology and natural history
TL;DR: A deeper understanding of the mechanisms responsible for an accelerated course of the disease and the identification of biomarkers of progression would lead to a better stratification of the Disease, essential for delivering individualized therapeutic strategies.
Journal ArticleDOI
Inhibition of Myocardin-Related Transcription Factor/Serum Response Factor Signaling Decreases Lung Fibrosis and Promotes Mesenchymal Cell Apoptosis
Thomas H. Sisson,Iyabode O. Ajayi,Natalya Subbotina,Amos E. Dodi,Eva S. Rodansky,Lauren N. Chibucos,Kevin K. Kim,Venkateshwar G. Keshamouni,Eric S. White,Yong Zhou,Peter D.R. Higgins,Scott D. Larsen,Richard R. Neubig,Jeffrey C. Horowitz +13 more
TL;DR: In vivo studies examined the effect of therapeutically administered CCG-203971 on lung fibrosis in two distinct murine models of fibrosis induced by bleomycin or targeted type II alveolar epithelial injury and suggest that its inhibition can help resolve lung fibrot by promoting fibroblast apoptosis.
Journal ArticleDOI
Quantitative computed tomography imaging of interstitial lung diseases.
Brian J. Bartholmai,Sushravya Raghunath,Ronald A. Karwoski,Teng Moua,Srinivasan Rajagopalan,Fabien Maldonado,Paul A. Decker,Richard A. Robb +7 more
TL;DR: It is likely that quantitative analysis of HRCT can be used in clinical practice as a means to aid in identifying a probable diagnosis, stratifying prognosis in early disease, and consistently determining progression of the disease or response to therapy.
Journal ArticleDOI
Palliative Care and Location of Death in Decedents With Idiopathic Pulmonary Fibrosis
Kathleen O. Lindell,Zhan Liang,Leslie A. Hoffman,Margaret Rosenzweig,Melissa Saul,Joseph M. Pilewski,Kevin F. Gibson,Naftali Kaminski +7 more
TL;DR: The majority of patients with IPF died in a hospital setting and only a minority received a formal palliative care referral, indicating the need to study adequacy of end-of-life management in IPF and promote earlier discussion and referral to palliatives care.
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TL;DR: The Diagnostic Process Is Dynamic Clinical Evaluation Radiological Evaluation Role of Surgical Lung Biopsy Unclassifiable Interstitial Pneumonia Bronchoalveolar Lavage Fluid Evaluation Idiopathic Pulmonary Fibrosis.
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