Title

Idiopathic Pulmonary Fibrosis and Progressive Pulmonary Fibrosis in Adults

Authoring Organizations

American Thoracic Society

European Respiratory Society

Publication Month/Year

April 28, 2022

Last Updated Month/Year

October 4, 2024

Document Type

Guideline

Country of Publication

US

Document Objectives

This American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Asociación Latinoamericana de Tórax 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.

Target Patient Population

Adults with idiopathic pulmonary fibrosis (IPF)

Target Provider Population

Pulmonologists, critical care specialists and other allied providers caring for patients with pulmonary fibrosis

PICO Questions

  1. Should patients with progressive fibrotic NSIP be treated with pirfenidone?

  2. Should patients with progressive fibrotic sarcoidosis be treated with pirfenidone?

  3. Should patients with progressive fibrotic unclassifiable ILD (uILD) be treated with pirfenidone?

Inclusion Criteria

Male, Female, Adult, Older adult

Health Care Settings

Ambulatory, Hospital, Laboratory services, Outpatient

Intended Users

Nurse, nurse practitioner, physician, physician assistant

Scope

Diagnosis, Assessment and screening, Treatment, Management, Prevention

Diseases/Conditions (MeSH)

D054990 - Idiopathic Pulmonary Fibrosis, D017563 - Lung Diseases, Interstitial, D011658 - Pulmonary Fibrosis

Keywords

interstitial lung disease, Idiopathic pulmonary fibrosis (IPF), IPF, ILD, PPF, Progressive Pulmonary Fibrosis

Source Citation

Raghu G, Remy-Jardin M, Richeldi L, Thomson CC, Inoue Y, Johkoh T, Kreuter M, Lynch DA, Maher TM, Martinez FJ, Molina-Molina M, Myers JL, Nicholson AG, Ryerson CJ, Strek ME, Troy LK, Wijsenbeek M, Mammen MJ, Hossain T, Bissell BD, Herman DD, Hon SM, Kheir F, Khor YH, Macrea M, Antoniou KM, Bouros D, Buendia-Roldan I, Caro F, Crestani B, Ho L, Morisset J, Olson AL, Podolanczuk A, Poletti V, Selman M, Ewing T, Jones S, Knight SL, Ghazipura M, Wilson KC. Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med. 2022 May 1;205(9):e18-e47. doi: 10.1164/rccm.202202-0399ST. PMID: 35486072.