Integration and application of clinical practice guidelines for the diagnosis of idiopathic pulmonary fibrosis and fibrotic hypersensitivity pneumonitis.

Abstract

Recent clinical practice guidelines have addressed the diagnosis of idiopathic pulmonary fibrosis (IPF) and fibrotic hypersensitivity pneumonitis (fHP). These disease-specific guidelines were developed independently, without clear direction on how to concurrently apply their respective recommendations within a single patient, where discrimination between these two fibrotic interstitial lung diseases represents a frequent diagnostic challenge. The objective of this document, created by an international group of experts, was to suggest a pragmatic approach on how to apply existing guidelines to distinguish IPF and fHP. Key clinical, radiological, and pathological features described in previous guidelines are integrated in a set of diagnostic algorithms, which are then placed in the broader context of multidisciplinary discussion to guide the generation of a consensus diagnosis. While these algorithms necessarily reflect some uncertainty wherever strong evidence is lacking, they provide insight into the current approach favored by experts in the field based on currently available knowledge. The authors further identify priorities for future research to clarify ongoing uncertainties in the diagnosis of fibrotic interstitial lung diseases.