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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Idiopathic Pulmonary Fibrosis: Gender-Age-Physiology Index Stage for Predicting Future Lung Function Decline
Margaret L. Salisbury,Meng Xia,Yueren Zhou,Susan Murray,Nabihah Tayob,Kevin K. Brown,Athol U. Wells,Shelley L. Schmidt,Fernando J. Martinez,Kevin R. Flaherty +9 more
TL;DR: Baseline GAP stage predicted death or lung transplantation but not the rate of future pulmonary function decline, and over a 2-year period, Gap stage was not associated with differences in yearly lung function decline.
Journal ArticleDOI
JNK inhibition reduces lung remodeling and pulmonary fibrotic systemic markers
Jos van der Velden,Ying Ye,James D. Nolin,Sidra M. Hoffman,David G. Chapman,Karolyn G. Lahue,Sarah Abdalla,Peng Chen,Yong Liu,Brydon L. Bennett,Nasreen Khalil,Donna Sutherland,William T. Smith,Gerald Horan,Mahmoud Assaf,Zebulun Horowitz,Rajesh Chopra,R. Stevens,Maria Palmisano,Yvonne M. W. Janssen-Heininger,Peter H. Schafer +20 more
TL;DR: Results illustrate JNK enzymatic activity involvement during pulmonary fibrosis, and support systemic biomarker use for tracking disease progression and the potential clinical benefit of this novel intervention in IPF.
Journal ArticleDOI
Reducing protein oxidation reverses lung fibrosis
Vikas Anathy,Karolyn G. Lahue,David G. Chapman,Shi B. Chia,Dylan T. Casey,Reem Aboushousha,Jos van der Velden,Evan Elko,Sidra M. Hoffman,David H. McMillan,Jane T. Jones,James D. Nolin,Sarah Abdalla,Robert W. Schneider,David J. Seward,Elle C. Roberson,Matthew D. Liptak,Morgan E. Cousins,Kelly J. Butnor,Douglas J. Taatjes,Ralph C. Budd,Charles G. Irvin,Ye-Shih Ho,Razq Hakem,Kevin K. Brown,Reiko Matsui,Markus Bachschmid,Jose L. Gomez,Naftali Kaminski,Albert van der Vliet,Yvonne M. W. Janssen-Heininger +30 more
TL;DR: It is shown that endogenous GLRX was inactivated through an oxidative mechanism and that direct administration of the Glrx protein into airways augmented Glrx activity and reversed increases in collagen in mice with TGFB1- or bleomycin-induced fibrosis, even when administered to fibrotic, aged animals.
Journal ArticleDOI
Outcomes in idiopathic pulmonary fibrosis: A meta-analysis from placebo controlled trials
TL;DR: Mortality was lower in trials that recruited patients with mild-moderate disease severities only, as compared to trials where patients with severe disease were allowed, and infections were high, both with and without immunosuppression, and were higher in severe disease.
Journal ArticleDOI
Autoimmunity to Vimentin Is Associated with Outcomes of Patients with Idiopathic Pulmonary Fibrosis.
Fu Jun Li,Ranu Surolia,Huashi Li,Zheng Wang,Tejaswini Kulkarni,Gang Liu,Joao A. de Andrade,Joao A. de Andrade,Daniel J. Kass,Victor J. Thannickal,Victor J. Thannickal,Steven R. Duncan,Veena B. Antony +12 more
TL;DR: The data show that anti-vimentin autoreactivity is prevalent in IPF patients and is strongly associated with disease manifestations, and raise the possibility that therapies specifically directed at these autoimmune processes could have therapeutic efficacy.
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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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