Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management

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 sectionsondefinitionandepidemiology, 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 questionbased 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.

Keywords: idiopathic pulmonary fibrosis; usual interstitial pneumonia; evidence-based medicine, diagnosis, therapeutics

Idiopathic pulmonary fibrosis (IPF) is defined as a specific form of chronic, progressive fibrosing interstitial pneumonia of unknown cause, occurring primarily in older adults, and limited to the lungs. It is characterized by progressive worsening of dyspnea and lung function and is associated with a poor prognosis. The American Thoracic Society and European Respiratory Society (ATS/ERS), in collaboration with the American College of Chest Physicians (ACCP), published an international consensus statement in 2000 on the diagnosis and management of IPF (1). Importantly, the statement recognized IPF as a distinct clinical entity associated with the histologic appearance of usual interstitial pneumonia (UIP), and provided specific recommendations for clinicians regarding its diagnosis and management. Since the publication of the 2000 ATS/ERS statement, studies have used the ATS/ERS statement recommendations to further our understanding of the clinical manifestations and course of IPF. The accumulated data and observations made in these studies allow us to provide new guidelines for the diagnosis and management of IPF based on the best available evidence using ATS/ERS methodology.

OBJECTIVE

This document is an international evidence-based guideline on the diagnosis and management of IPF. The purpose of these guidelines is to analyze the additional evidence accumulated since the publication of the 2000 ATS/ERS consensus statement and to provide evidence-based recommendations for management, with an emphasis on diagnosis and treatment. This document is intended to replace the previous ATS/ERS IPF consensus statement, and will be updated when appropriate in accordance with the policy of the sponsoring societies.
The primary objective of this document is to provide recommendations based on a thorough review of the evidence published to date using the GRADE methodology (see below) to clinicians in a transparent manner. It is intended to empower clinicians to interpret these recommendations in the context of individual patient values and preferences, and to make appropriate decisions regarding all aspects of disease management, tailored to the patient with typical IPF.