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
Long-term clinical and real-world experience with pirfenidone in the treatment of idiopathic pulmonary fibrosis.
Vincent Cottin,Toby M. Maher +1 more
TL;DR: Educating patients about the potential for these adverse events to occur and providing instructions prior to treatment to avoid adverse drug reactions are an important means of ensuring patients may derive the important benefits provided by long-term treatment with pirfenidone.
Journal ArticleDOI
Discovery of CC-930, an orally active anti-fibrotic JNK inhibitor.
Veronique Plantevin Krenitsky,Lisa Nadolny,Mercedes Delgado,Leticia Ayala,Steven S. Clareen,Robert Hilgraf,Ronald Albers,Sayee G. Hegde,Neil R. D'sidocky,John Sapienza,Jonathan Wright,Meg Mccarrick,Sogole Bahmanyar,Philip P Chamberlain,Silvia L. Delker,Jeff Muir,David Giegel,Li Xu,Maria Celeridad,Jeff Lachowitzer,Brydon L. Bennett,Mehran F. Moghaddam,Oleg Khatsenko,Jason Katz,Rachel Fan,April Bai,Yang Tang,Michael A. Shirley,Brent Benish,Tracey Bodine,Kate Blease,Heather Raymon,Brian E. Cathers,Yoshitaka Satoh +33 more
TL;DR: This Letter describes the discovery of potent, selective, and orally active aminopurine JNK inhibitors and the identification of 1 (CC-930) as a development candidate, which is currently in Phase II clinical trial for IPF.
Journal ArticleDOI
A novel formulation of inhaled sodium cromoglicate (PA101) in idiopathic pulmonary fibrosis and chronic cough: a randomised, double-blind, proof-of-concept, phase 2 trial
Surinder S. Birring,Marlies S. Wijsenbeek,Sanjay Agrawal,Jan W.K. van den Berg,Helen Stone,Toby M. Maher,Ahmet Tutuncu,Alyn H. Morice +7 more
TL;DR: Inhaled PA101 could be a treatment option for chronic cough in patients with IPF and warrants further investigation, and the mechanism of cough in IPF might be disease specific.
Journal ArticleDOI
Biomarkers in idiopathic pulmonary fibrosis.
TL;DR: The bacterial signature in IPF lungs, as shown from microbiome analyses, as well as mitochondrial DNA seem to have promising roles as biomarkers, and combination of multiple biomarkers may identify comprehensive biomarker signatures in IPf patients.
Journal ArticleDOI
Accuracy and Reliability of Internet Resources for Information on Idiopathic Pulmonary Fibrosis.
TL;DR: Patient-directed online information on IPF is frequently incomplete, inaccurate, and outdated and there is no reliable method for patients to identify sites that provide appropriate information onIPF.
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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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