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Introduction
Part I. Update on the Diagnosis and Treatment of IPF
➤ We suggest transbronchial lung cryobiopsy (TBLC) be regarded
as an acceptable alternative to surgical lung biopsy for making a
histopathological diagnosis in patients with ILD of undetermined type
in medical centers with experience performing and interpreting TBLC
(conditional recommendation, very low-quality evidence).
➤ We make no recommendation for or against the addition of genomic
classifier testing (GCT) for the purpose of diagnosing usual interstitial
pneumonia (UIP) in patients with ILD of undetermined type who
are undergoing transbronchial forceps biopsy, due to insufficient
agreement among the committee members.
Remarks: There was consensus that genomic classifier testing should be reconsidered
once additional studies are published.
➤ We suggest not treating patients with IPF with antacid medication
for the purpose of improving respiratory outcomes (conditional
recommendation, very low-quality evidence).
Remarks: Antacid medication and other interventions may be appropriate for
patients with both IPF and symptoms of gastroesophageal reflux disease (GERD)
for the purpose of improving gastroesophageal reflux (GER) -related outcomes in
accordance with GER-specific guidelines.
➤ We suggest not referring patients with IPF for anti-reflux surgery
for the purpose of improving respiratory outcomes (conditional
recommendation, very low-quality evidence).
Remarks: Anti-reflux surgery may be appropriate for patients with both IPF and
symptoms of GERD for the purpose of improving GER-related outcomes in accordance
with GER-specific guidelines.
➤ Progress in the diagnosis and treatment of idiopathic pulmonary
fibrosis (IPF) and other interstitial lung diseases (ILDs) manifesting
progressive pulmonary fibrosis (PPF) has generated the need for
updating guidelines.
➤ These recommendations are not mandates because they cannot
account for all unique clinical circumstances and should be revisited as
new evidence is published.
Recommendations
