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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Acute Exacerbation in Interstitial Lung Disease.
Gabriela Leuschner,Jürgen Behr +1 more
TL;DR: The etiology of AE-ILD is not fully understood, but there are distinct risk factors and triggers like infection, mechanical stress, and microaspiration and it is associated with a high mortality within 6–12 months.
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Pirfenidone in Idiopathic Pulmonary Fibrosis: Real-Life Experience from a German Tertiary Referral Center for Interstitial Lung Diseases
Ute Oltmanns,Nicolas Kahn,Karin Palmowski,Annette Träger,Heinrich Wenz,Claus Peter Heussel,Philipp A. Schnabel,Michael Puderbach,M. Wiebel,Svenja Ehlers-Tenenbaum,Arne Warth,Felix J.F. Herth,Michael Kreuter +12 more
TL;DR: In this heterogeneous patient group, a nonsignificant effect of pirfenidone treatment on pulmonary function was seen, underlining the need for more data on patient selection criteria and efficacy of pirenidone, particularly in patients with coexistent emphysema and concomitant NAC/CCS treatment.
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Alveolar derecruitment and collapse induration as crucial mechanisms in lung injury and fibrosis.
Dennis Lutz,Amiq Gazdhar,Elena Lopez-Rodriguez,Clemens Ruppert,Poornima Mahavadi,Andreas Günther,Walter Klepetko,Jason H. T. Bates,Bradford J. Smith,Thomas Geiser,Matthias Ochs,Lars Knudsen +11 more
TL;DR: The authors' ultrastructural observations suggest that collapse induration is important in human IPF, and surfactant inactivation leading to alveolar collapse and subsequent collapse indurations might be the primary pathway for the loss of alveoli in this animal model.
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Mechanisms and consequences of oxidative stress in lung disease: therapeutic implications for an aging populace.
TL;DR: This review will discuss the consequences of aging on lung morphology and physiology, and how redox imbalance with aging contributes to lung disease susceptibility, and the development of more effective therapeutic strategies for lung diseases that disproportionately afflict the elderly.
Idiopathic pulmonary fi brosis
TL;DR: Recent data on the clinical course, therapeutic options, and underlying mechanisms thought to be involved in the pathogenesis of idiopathic pulmonary fi brosis are reviewed.
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