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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Hot of the breath: Mortality as a primary end-point in IPF treatment trials: the best is the enemy of the good
TL;DR: It is believed strongly that the adoption of the views of these authors by licensing bodies—with, by implication, a statistically significant mortality benefit a pre-requisite for drug registration—would set back progress in the treatment of IPF by a decade or more.
Journal Article
Treatment and outcome of lung cancer in idiopathic interstitial pneumonias.
Michael Kreuter,Svenja Ehlers-Tenenbaum,Miriam Schaaf,Ute Oltmanns,Karin Palmowski,Hans Hoffmann,Philipp A. Schnabel,CP Heußel,Michael Puderbach,Felix J.F. Herth,Arne Warth +10 more
TL;DR: LC is a frequent comorbidity in IIP, with a higher incidence and reduced survival in IPF compared to other IIPs, and interdisciplinary evaluation of therapeutic options in IIPs patients diagnosed with LC is mandatory.
Journal ArticleDOI
Predictors of diagnosis and survival in idiopathic pulmonary fibrosis and connective tissue disease-related usual interstitial pneumonia
TL;DR: Presenting radiologic and pathologic features alone are not predictive of underlying secondary cause or survival between the two groups, and only age and forced vital capacity corrected for a priori covariates were predictive of survival in CTD-UIP.
Journal Article
Mast cells and fibroblasts work in concert to aggravate pulmonary fibrosis: Role of transmembrane SCF and PAR-2/PKCα/Raf-1/p44/42 signaling pathway
Malgorzata Wygrecka,Bhola D. Dahal,Djuro Kosanovic,Frank Petersen,Brigitte Taborski,Susanne von Gerlach,Miroslava Didiasova,Dariusz Zakrzewicz,Klaus T. Preissner,Ralph T. Schermuly,Philipp Markart +10 more
TL;DR: Fibroblasts and MCs appear to work in concert to perpetuate fibrotic processes and so contribute to lung fibrosis progression.
Journal ArticleDOI
The psychometric properties of the St George's Respiratory Questionnaire (SGRQ) in patients with idiopathic pulmonary fibrosis: a literature review.
TL;DR: Although the SGRQ was not developed specifically for use with patients with IPF, its psychometric properties are adequate and suggest that it may be a useful measure of HRQL in this patient population, however, several questions remain unaddressed, and further research is needed to confirm the S GRQ’s utility in IPF.
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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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David M. Hansell,Alexander A. Bankier,Heber MacMahon,Theresa C. McLoud,Nestor L. Müller,J Remy +5 more
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