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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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Journal ArticleDOI

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

A controlled trial of sildenafil in advanced idiopathic pulmonary fibrosis

TL;DR: This study tested the hypothesis that treatment with sildenafil would improve walk distance, dyspnea, and quality of life in patients with advanced idiopathic pulmonary fibrosis, defined as a carbon monoxide diffusion capacity of less than 35% of the predicted value.
Journal ArticleDOI

Incidence and mortality of idiopathic pulmonary fibrosis and sarcoidosis in the UK

TL;DR: The incidence of Idiopathic pulmonary fibrosis has more than doubled between 1990 and 2003; this is not due to the ageing of the UK population or an increased ascertainment of milder cases.
Journal ArticleDOI

Prognosis of fibrotic interstitial pneumonia: idiopathic versus collagen vascular disease-related subtypes.

TL;DR: The data suggest that the better prognosis of patients in the CVD group is not solely due to the predominance of the NSIP pattern, despite the same pathologic pattern.
Journal ArticleDOI

Increased prevalence of gastroesophageal reflux in patients with idiopathic pulmonary fibrosis.

TL;DR: Patients with IPF have a high prevalence of increased esophageal acid exposure, usually without typical GER symptoms, and acid reflux may be a contributing factor in the pathogenesis of IPF.
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American thoracic society/European respiratory society international multidisciplinary consensus classification of the idiopathic interstitial pneumonias