TY - JOUR
T1 - Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults
T2 - An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline
AU - the American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Asociacion Latinoamericana de Torax
AU - Raghu, Ganesh
AU - Remy-Jardin, Martine
AU - Richeldi, Luca
AU - Thomson, Carey C.
AU - Antoniou, Katerina M.
AU - Bissell, Brittany D.
AU - Bouros, Demosthenes
AU - Buendia-Roldan, Ivette
AU - Caro, Fabian
AU - Crestani, Bruno
AU - Ewing, Thomas
AU - Ghazipura, Marya
AU - Herman, Derrick D.
AU - Ho, Lawrence
AU - Hon, Stephanie M.
AU - Hossain, Tanzib
AU - Inoue, Yoshikazu
AU - Johkoh, Takeshi
AU - Jones, Stephen
AU - Kheir, Fayez
AU - Khor, Yet H.
AU - Knight, Shandra L.
AU - Kreuter, Michael
AU - Lynch, David A.
AU - Macrea, Madalina
AU - Maher, Toby M.
AU - Mammen, Manoj J.
AU - Martinez, Fernando J.
AU - Molina-Molina, Maria
AU - Morisset, Julie
AU - Myers, Jeffrey L.
AU - Nicholson, Andrew G.
AU - Olson, Amy L.
AU - Podolanczuk, Anna
AU - Poletti, Venerino
AU - Ryerson, Christopher J.
AU - Selman, Moises
AU - Strek, Mary E.
AU - Troy, Lauren K.
AU - Wijsenbeek, Marlies
AU - Wilson, Kevin C.
N1 - Publisher Copyright:
© 2022 by the American Thoracic Society
PY - 2022/5/1
Y1 - 2022/5/1
N2 - 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.
AB - 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.
KW - histopathology
KW - idiopathic pulmonary fibrosis
KW - progressive pulmonary fibrosis
KW - radiology
UR - https://www.scopus.com/pages/publications/85129135772
U2 - 10.1164/rccm.202202-0399ST
DO - 10.1164/rccm.202202-0399ST
M3 - Article
C2 - 35486072
AN - SCOPUS:85129135772
SN - 1073-449X
VL - 205
SP - E18-E47
JO - American Journal of Respiratory and Critical Care Medicine
JF - American Journal of Respiratory and Critical Care Medicine
IS - 9
ER -