Summary for Clinicians: Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults
Evidence-based guidelines for the management of idiopathic pulmonary fibrosis (IPF) were updated in 2022 in a multidisciplinary discussion (MDD) by a panel of experts appointed by the American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society. Topics from earlier guidelines that were addressed included radiological and histopathological features of usual interstitial pneumonia (UIP), transbronchial lung cryobiopsy (TBLC), and genomic classifier (GC) testing for the diagnosis of IPF and the use of antacid medications and antireflux surgery for treatment of IPF.
Additionally, the term progressive pulmonary fibrosis (PPF) was defined to describe interstitial lung diseases (ILDs) other than IPF that progress to fibrosis, and treatment of PPF with antifibrotic medications was explored. Guidelines have different implications for patients, clinicians, and policymakers. This summary is intended to provide the practicing clinician with key points from the guideline.
Pulmonologists, critical care specialists, translational researchers, and clinicians
At the conclusion of this activity, learners should be able to:
- Effectively communicate the risks and benefits of medical antacid therapy and antireflux surgery to patients with idiopathic pulmonary fibrosis
- Differentiate between progressive and non-progressive forms of pulmonary fibrosis
- Develop new strategies to manage patients with progressive pulmonary fibrosis
The American Thoracic Society is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians.
Article Authorship Disclosures (as submitted to the ATS prior to article publication date)
Derrick D. Herman, M.D. (The Ohio State University Wexner Medical Center, Columbus, OH, USA) reported no financial relationships with ineligible companies.
Marya Ghazipura, M.S., PhD. (New York University Langone Health, New York, NY, USA) reported no financial relationships with ineligible companies.
Ganesh Raghu, M.D. (University of Washington Medical Center, Seattle, WA, USA) reported grants from NIH; served as a consultant for IPF and PF studies for Bellerophan, BMS, Fibrogen, Nitto, Novartis, Roche-Genentech, and Veracyte.
Luca Richeldi, M.D., PhD. (Università Cattolica del Sacro Cuore, Rome, Italy) reported grants from Roche and Boehringer Ingelheim; personal fees from Biogen, Roche, ImmuneWorks, Boehringer Ingelheim, Celgene, Nitto, Fibrogen, Promedior, Pliant Therapeutics, Asahl Kasel, Toray, BMS, RespiVant, and CSL Behring
Martine Remy-Jardin, M.D., PhD. (Hôpital Calmette, Université Lille Nord de France, Lille, France) reported no financial relationships with ineligible companies.
Joseph K. Ruminjo, M.D. (American Thoracic Society, New York, NY, USA) reported no financial relationships with ineligible companies.
Carey C. Thomson, M.D., M.P.H. (Mt. Auburn Hospital, Cambridge, MA, USA) reported personal fees from Median Technology, HealthMyne, Fulcrum Therapeutics, and UpToDate.
Off-Label Usage Disclosure
The off-label use of pirfenidone for treating progressive pulmonary fibrosis was discussed.
Disclosures of AnnalsATS CME Planners
Margaret M. Hayes, M.D.
Harvard Medical School, Boston MA, USA
Dr. Hayes reported receiving payments as an author for a chapter on heliox for UpToDate.
Silpa Krefft, M.D., M.P.H.
Video Editor, AnnalsATS
University of Colorado, Denver CO, USA
Dr. Krefft reported providing medicolegal consulting in the area of occupational lung disease.
Caroline Okorie, M.D., M.P.H.
Stanford University School of Medicine, Stanford CA, USA
Dr. Okorie reported no financial relationships with ineligible companies.
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