Integration and Application of Clinical Practice Guidelines for the Diagnosis of Idiopathic Pulmonary Fibrosis and Fibrotic Hypersensitivity Pneumonitis

Daniel Costin Marinescu*, Ganesh Raghu, Martine Remy-Jardin, William D. Travis, Ayodeji Adegunsoye, Mary Beth Beasley, Jonathan H. Chung, Andrew Churg, Vincent Cottin, Ryoko Egashira, Evans R. Fernández Pérez, Yoshikazu Inoue, Kerri A. Johannson, Ella A. Kazerooni, Yet H. Khor, David A. Lynch, Nestor L. Müller, Jeffrey L. Myers, Andrew G. Nicholson, Sujeet RajanRyoko Saito-Koyama, Lauren Troy, Simon L.F. Walsh, Athol U. Wells, Marlies S. Wijsenbeek, Joanne L. Wright, Christopher J. Ryerson

*Corresponding author for this work

Research output: Contribution to journalReview articleAcademicpeer-review

21 Citations (Scopus)


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 apply their respective recommendations concurrently within a single patient, where discrimination between these two fibrotic interstitial lung diseases represents a frequent diagnostic challenge. The objective of this review, 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, radiologic, and pathologic features described in previous guidelines are integrated in a set of diagnostic algorithms, which then are placed in the broader context of multidisciplinary discussion to guide the generation of a consensus diagnosis. Although 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.

Original languageEnglish
Pages (from-to)614-629
Number of pages16
Issue number3
Early online date19 Jun 2022
Publication statusPublished - 1 Sept 2022

Bibliographical note

Funding Information: Funding/support: The authors have reported to CHEST that no funding was received for this study. Financial/nonfinancial disclosures: None declared.

Publisher Copyright:
© 2022 American College of Chest Physicians


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