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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

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 Article

Cigarette smoking: A risk factor for idiopathic pulmonary fibrosis

TL;DR: Although there was no clear exposure-response pattern with cumulative consumption of cigarettes, there was a trend for time since cessation of smoking, with the highest risk for those who had most recently quit.
Journal ArticleDOI

Idiopathic pulmonary fibrosis: a composite physiologic index derived from disease extent observed by computed tomography.

TL;DR: In this paper, the composite physiologic index (CPI) most closely reflecting the morphologic extent of pulmonary fibrosis was identified as a more accurate prognostic determinant in usual interstitial pneumonia than an individual pulmonary function test.
Journal ArticleDOI

The Clinical Course of Patients with Idiopathic Pulmonary Fibrosis

TL;DR: The data suggest that a gradual, progressive decline does not occur in many patients with idiopathic pulmonary fibrosis, thereby supporting the need for early referral for lung transplantation.
Journal ArticleDOI

Fibrotic idiopathic interstitial pneumonia: The prognostic value of longitudinal functional trends

TL;DR: It is concluded that at 12 months, serial pulmonary function trends have considerable prognostic value in UIP and NSIP, while their histologic distinction provides no additional prognostic information when pulmonary function Trends are clear cut or when functional impairment is severe.
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American thoracic society/European respiratory society international multidisciplinary consensus classification of the idiopathic interstitial pneumonias