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Open AccessJournal ArticleDOI

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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Mode of action of nintedanib in the treatment of idiopathic pulmonary fibrosis

TL;DR: Data from in vitro studies have shown that nintedanib interferes with processes active in fibrosis such as fibroblast proliferation, migration and differentiation, and the secretion of ECM, providing a strong rationale for the clinical efficacy of nintinganib in patients with IPF.
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Global incidence and mortality of idiopathic pulmonary fibrosis: a systematic review

TL;DR: The incidence of idiopathic pulmonary fibrosis is increasing worldwide and rates are coming together across countries, and current data suggest incidence is similar to that of conditions such as stomach, liver, testicular and cervical cancers.
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Pathogenesis of Idiopathic Pulmonary Fibrosis

TL;DR: A focus on genetic predisposition to IPF and how genetic changes, which occur primarily in epithelial cells, lead to activation of profibrotic pathways in epithelium is focused on.
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Therapy for Fibrotic Diseases: Nearing the Starting Line

TL;DR: An emerging consensus indicates that fibrotic diseases in lung, liver, and kidney exhibit common underlying mechanisms, which can be targeted therapeutically, and ongoing efforts to develop effective therapies are described, including a discussion of obstacles to progress.
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Acellular Normal and Fibrotic Human Lung Matrices as a Culture System for In Vitro Investigation

TL;DR: A culture system using acellular human normal and fibrotic lungs is developed to better address how ECM influences fibroblast phenotype in a disease-specific manner and allows investigation of important ECM-based hypotheses in human tissues.
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.
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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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