TY - JOUR
T1 - Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline
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.
PY - 2022
Y1 - 2022
N2 - Abstract
Background: This American Thoracic Society, European
Respiratory Society, Japanese Respiratory Society, and
Asociacion Latinoamericana de T orax 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.
Keywords: idiopathic pulmonary fibrosis; progressive pulmonary
fibrosis; radiology; histopathology
AB - Abstract
Background: This American Thoracic Society, European
Respiratory Society, Japanese Respiratory Society, and
Asociacion Latinoamericana de T orax 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.
Keywords: idiopathic pulmonary fibrosis; progressive pulmonary
fibrosis; radiology; histopathology
KW - histopathology
KW - idiopathic pulmonary fibrosis
KW - progressive pulmonary fibrosis
KW - radiology
KW - histopathology
KW - idiopathic pulmonary fibrosis
KW - progressive pulmonary fibrosis
KW - radiology
UR - http://hdl.handle.net/10807/203454
U2 - 10.1164/rccm.202202-0399ST
DO - 10.1164/rccm.202202-0399ST
M3 - Article
SN - 1073-449X
VL - 205
SP - e18-e47-e47
JO - American Journal of Respiratory and Critical Care Medicine
JF - American Journal of Respiratory and Critical Care Medicine
ER -