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An Official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-Based Guidelines for Diagnosis and Management

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.

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Citations
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Journal ArticleDOI

Daily Home Spirometry: An Effective Tool for Detecting Progression in Idiopathic Pulmonary Fibrosis.

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.
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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.
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Inhibition of Myocardin-Related Transcription Factor/Serum Response Factor Signaling Decreases Lung Fibrosis and Promotes Mesenchymal Cell Apoptosis

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.
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Quantitative computed tomography imaging of interstitial lung diseases.

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

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.
References
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Standardisation of spirometry

TL;DR: This research presents a novel and scalable approach called “Standardation of LUNG FUNCTION TESTing” that combines “situational awareness” and “machine learning” to solve the challenge of integrating nanofiltration into the energy system.
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Cancer statistics, 2007.

TL;DR: While the absolute number of cancer deaths decreased for the second consecutive year in the United States, much progress has been made in reducing mortality rates and improving survival, cancer still accounts for more deaths than heart disease in persons under age 85 years.
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Interpretative strategies for lung function tests

TL;DR: This section is written to provide guidance in interpreting pulmonary function tests (PFTs) to medical directors of hospital-based laboratories that perform PFTs, and physicians who are responsible for interpreting the results of PFTS most commonly ordered for clinical purposes.
Journal ArticleDOI

American thoracic society/European respiratory society international multidisciplinary consensus classification of the idiopathic interstitial pneumonias

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.
Journal ArticleDOI

Fleischner Society: Glossary of Terms for Thoracic Imaging

TL;DR: Members of the Fleischner Society compiled a glossary of terms for thoracic imaging that replaces previous glossaries published in 1984 and 1996 for Thoracic radiography and computed tomography, respectively.
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American thoracic society/European respiratory society international multidisciplinary consensus classification of the idiopathic interstitial pneumonias