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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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Citations
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Measuring diffusion limitation with a perfusion-limited gas--hyperpolarized 129Xe gas-transfer spectroscopy in patients with idiopathic pulmonary fibrosis.

TL;DR: This work hypothesized that in patients with idiopathic pulmonary fibrosis (IPF), the ratio of (129)Xe spectroscopic signal in the RBCs vs. barrier would diminish as diffusion-limitation delayed replenishment of ( 129) Xe magnetization in RBC’s.
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Pulmonary hypertension in chronic interstitial lung diseases.

TL;DR: Pulmonary hypertension is a common complication of interstitial lung diseases, particularly in idiopathic pulmonary fibrosis and ILD associated with connective tissue disease, and right heart catheterisation remains the current gold standard for diagnosis.
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Fasudil, a Rho-Kinase Inhibitor, Attenuates Bleomycin-Induced Pulmonary Fibrosis in Mice

TL;DR: Fasudil, a selective ROCK inhibitor, is suggested to be a potential therapeutic candidate for the treatment of pulmonary fibrosis through its beneficial effects and mechanisms.
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Safety and efficacy of pirfenidone in idiopathic pulmonary fibrosis in clinical practice.

TL;DR: Pirfenidone was well-tolerated and had beneficial effects in patients with mild-to-severe and/or progressive IPF, and the most frequent adverse event was anorexia.
Journal ArticleDOI

Idiopathic Pulmonary Fibrosis: CT and Risk of Death

TL;DR: CT fibrosis score can replace diffusioncapacity of carbon monoxide test results in a modified GAP gender, age, and physiology model with comparable performance and may be a useful alternative model in situations where CT scoring is more reliable and available than diffusion capacity of carbon Monoxide.
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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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