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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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An Official American Thoracic Society/European Respiratory Society Statement: Update of the International Multidisciplinary Classification of the Idiopathic Interstitial Pneumonias

TL;DR: This update is a supplement to the previous 2002 IIP classification document and outlines advances in the past decade and potential areas for future investigation.
References
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Journal ArticleDOI

Sildenafil for treatment of lung fibrosis and pulmonary hypertension: a randomised controlled trial

TL;DR: Sildenafil causes preferential pulmonary vasodilation and improves gas exchange in patients with severe lung fibrosis and secondary pulmonary hypertension, and ratio of pulmonary to systemic vascular resistance decreased only in individuals who received nitric oxide and sildanafil.
Journal ArticleDOI

Natural History and Treated Course of Usual and Desquamative Interstitial Pneumonia

TL;DR: It is concluded that the histologic classification of chronic interstitial pneumonia used here permits forecasts of prognosis and response to treatment that cannot be deduced from other data.
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Short telomeres are a risk factor for idiopathic pulmonary fibrosis

TL;DR: Short telomeres are a signature in IIPs and likely play a role in their age-related onset, and the clustering of cryptogenic liver cirrhosis with IPF suggests that the telomere shortening can contribute to what appears clinically as idiopathic progressive organ failure in the lung and the liver.
Journal ArticleDOI

Changes in clinical and physiologic variables predict survival in idiopathic pulmonary fibrosis.

TL;DR: Evaluated changes in clinical and physiological variables over time for survival time in 81 patients with biopsy-proven idiopathic pulmonary fibrosis found that changes in dyspnea score, total lung capacity, FVC, partial pressure of arterial oxygen, oxygen saturation, and alveolar-arterial oxygen gradient were predictive of survival time after adjustment for baseline values.
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American thoracic society/European respiratory society international multidisciplinary consensus classification of the idiopathic interstitial pneumonias