Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline

Ganesh Raghu*, Martine Remy-Jardin, the American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Asociacion Latinoamericana de Torax, Luca Richeldi, Carey C. Thomson, Katerina M. Antoniou, Brittany D. Bissell, Demosthenes Bouros, Ivette Buendia-Roldan, Fabian Caro, Bruno Crestani, Thomas Ewing, Marya Ghazipura, Derrick D. Herman, Lawrence Ho, Stephanie M. Hon, Tanzib Hossain, Yoshikazu Inoue, Takeshi Johkoh, Stephen JonesFayez Kheir, Yet H. Khor, Shandra L. Knight, Michael Kreuter, David A. Lynch, Madalina Macrea, Toby M. Maher, Manoj J. Mammen, Fernando J. Martinez, Maria Molina-Molina, Julie Morisset, Jeffrey L. Myers, Andrew G. Nicholson, Amy L. Olson, Anna Podolanczuk, Venerino Poletti, Christopher J. Ryerson, Moises Selman, Mary E. Strek, Lauren K. Troy, Marlies Wijsenbeek, Kevin C. Wilson

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

898 Citations (Scopus)

Abstract

Background: This American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Asociacion Latinoamericana de Torax guideline updates prior idiopathic pulmonary fibrosis (IPF) guidelines and addresses the progression of pulmonary fibrosis in patients with interstitial lung diseases (ILDs) other than IPF. Methods: A committee was composed of multidisciplinary experts in ILD, methodologists, and patient representatives. 1) Update of IPF: Radiological and histopathological criteria for IPF were updated by consensus. Questions about transbronchial lung cryobiopsy, genomic classifier testing, antacid medication, and antireflux surgery were informed by systematic reviews and answered with evidence-based recommendations using the Grading of Recommendations, Assessment, Development and Evaluation (GRADE) approach. 2) Progressive pulmonary fibrosis (PPF): PPF was defined, and then radiological and physiological criteria for PPF were determined by consensus. Questions about pirfenidone and nintedanib were informed by systematic reviews and answered with evidence-based recommendations using the GRADE approach. Results: 1) Update of IPF: A conditional recommendation was made to regard transbronchial lung cryobiopsy as an acceptable alternative to surgical lung biopsy in centers with appropriate expertise. No recommendation was made for or against genomic classifier testing. Conditional recommendations were made against antacid medication and antireflux surgery for the treatment of IPF. 2) PPF: PPF was defined as at least two of three criteria (worsening symptoms, radiological progression, and physiological progression) occurring within the past year with no alternative explanation in a patient with an ILD other than IPF. A conditional recommendation was made for nintedanib, and additional research into pirfenidone was recommended. Conclusions: The conditional recommendations in this guideline are intended to provide the basis for rational, informed decisions by clinicians.

Original languageEnglish
Pages (from-to)E18-E47
JournalAmerican Journal of Respiratory and Critical Care Medicine
Volume205
Issue number9
DOIs
Publication statusPublished - 1 May 2022

Bibliographical note

Funding Information:
Supported by the American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Asociacion Latinoamericana de Torax.

Funding Information:
Supported by the American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Asociación Latinoamericana de Tórax.

Publisher Copyright:
© 2022 by the American Thoracic Society

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