Guidelines & Papers
Guidelines
Idiopathic Pulmonary Fibrosis/Progressive Pulmonary Fibrosis
- Raghu G, Remy-Jardin M, Richeldi L, et al. 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;205(9):e18–e47. doi:10.1164/rccm.202202-0399ST
Hypersensitivity Pneumonitis
Diagnosis of Hypersensitivity Pneumonitis in Adults: An Official ATS/JRS/ALAT Clinical Practice Guideline
- Raghu G, Remy-Jardin M, Ryerson CJ, et al. Diagnosis of Hypersensitivity Pneumonitis in Adults. An Official ATS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med. 2020;202(3):e36-e69. doi:10.1164/rccm.202005-2032ST.
- Koster MA, Raghu G. Diagnosis of hypersensitivity pneumonitis in adults, 2020 clinical practice guideline: summary for clinicians. Ann Am Thorac Soc. 2021;18(4):559-566. doi:10.1513/AnnalsATS.202009-1195CME
Interstitial Lung Abnormalities
- Podolanczuk AJ, Hunninghake GM, Wilson KC, et al. Approach to the evaluation and management of interstitial lung abnormalities. An official American Thoracic Society clinical statement. Am J Respir Crit Care Med. 2025;211(7):1132–1155. doi:10.1164/rccm.202405-0856ST.
Systemic Autoimmune Rheumatic Disease/Connective Tissue Diseases
- Johnson SR, Bernstein EJ, Bolster MB, et al. 2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) guideline for the treatment of interstitial lung disease in people with systemic autoimmune rheumatic diseases. Arthritis Care Res (Hoboken). 2024;76(8):1051-1069. doi:10.1002/acr.25348
ERS/EULAR clinical practice guidelines for connective tissue disease-associated interstitial lung disease
- Antoniou KM, Distler O, Gheorghiu AM, et al. ERS/EULAR clinical practice guidelines for connective tissue disease-associated interstitial lung disease. Eur Respir J. 2026;67(1):2402533. doi:10.1183/13993003.02533-2024.
- Raghu G, Montesi SB, Silver RM, et al. Treatment of systemic sclerosis–associated interstitial lung disease: evidence-based recommendations. An official American Thoracic Society clinical practice guideline. Am J Respir Crit Care Med. 2024;209(2):137–152. doi:10.1164/rccm.202306-1113ST
Additional Guidelines
- Ryerson CJ, Bankier A, Beasley MB, et al. Standardized clinical terms and definitions for interstitial lung disease: a consensus statement from the Fleischner Society. Am J Respir Crit Care Med. 2025;211(10):1756-1774. doi:10.1164/rccm.202505-1142SO
- Jacobs SS, Krishnan JA, Lederer DJ, et al. Home oxygen therapy for adults with chronic lung disease. Am J Respir Crit Care Med. 2021;202(10):e121–e141. doi:10.1164/rccm.202009-3608ST
Treatment Related Papers
Efficacy and Safety of Nintedanib in Idiopathic Pulmonary Fibrosis
Efficacy and Safety of Nintedanib in Idiopathic Pulmonary Fibrosis
- Richeldi L, du Bois RM, Raghu G, et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2071-2082. doi:10.1056/NEJMoa1402584
A Phase 3 Trial of Pirfenidone in Patients with Idiopathic Pulmonary Fibrosis
A Phase 3 Trial of Pirfenidone in Patients with Idiopathic Pulmonary Fibrosis
- King TE Jr, Bradford WZ, Castro-Bernardini S, et al. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2083-2092. doi:10.1056/NEJMoa14025
Nerandomilast in Patients with Idiopathic Pulmonary Fibrosis
Nerandomilast in Patients with Idiopathic Pulmonary Fibrosis
- Richeldi L, Azuma A, Cottin V, et al; FIBRONEER-IPF Investigators. Nerandomilast in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2025;392(22):2193-2202. doi:10.1056/NEJMoa2414108
Phase 3 Trials of Inhaled Treprostinil for Idiopathic Pulmonary Fibrosis
Phase 3 Trials of Inhaled Treprostinil for Idiopathic Pulmonary Fibrosis
- Nathan SD, Smith P, Deng C, et al; TETON-1 Trial Investigators. Phase 3 trials of inhaled treprostinil for idiopathic pulmonary fibrosis. N Engl J Med. Published online May 18, 2026. doi:10.1056/NEJMoa2501488.
Nintedanib in Progressive Fibrosing Interstitial Lung Diseases
Nintedanib in Progressive Fibrosing Interstitial Lung Diseases
- Flaherty KR, Wells AU, Brown KK, et al. Nintedanib in progressive fibrosing interstitial lung diseases. N Engl J Med. 2019;381(18):1718-1727. doi:10.1056/NEJMoa1908681
Nintedanib for Systemic Sclerosis–Associated Interstitial Lung Disease
Nintedanib for Systemic Sclerosis–Associated Interstitial Lung Disease
- Distler O, Highland KB, Gahlemann M, et al; SENSCIS Trial Investigators. Nintedanib for systemic sclerosis–associated interstitial lung disease. N Engl J Med. 2019;380(26):2518-2528. doi:10.1056/NEJMoa1903076
Nerandomilast in Patients with Progressive Pulmonary Fibrosis
Nerandomilast in Patients with Progressive Pulmonary Fibrosis
- Maher TM, Assassi S, Azuma A, et al; FIBRONEER‑IPF Investigators. Nerandomilast in patients with progressive pulmonary fibrosis. N Engl J Med. 2025;392(22):2203‑2214. doi:10.1056/NEJMoa2503643
Cyclophosphamide v. Placebo in Scleroderma Lung Disease
Cyclophosphamide versus Placebo in Scleroderma Lung Disease
- Tashkin DP, Elashoff R, Clements PJ, et al; Scleroderma Lung Study Research Group. Cyclophosphamide versus placebo in scleroderma lung disease. New England Journal of Medicine. 2006;354(25):2655-2666. doi:10.1056/NEJMoa055120
Mycophenolate Mofetil versus Oral Cyclophosphamide in Scleroderma-related Interstitial Lung Disease: Scleroderma Lung Study II (SLS-II), a double-blind, parallel group, randomised controlled trial
- Tashkin DP, Roth MD, Clements PJ, et al; Scleroderma Lung Study II Investigators. Mycophenolate mofetil versus oral cyclophosphamide in scleroderma-related interstitial lung disease (SLS II): a randomized controlled, double-blind, parallel group trial. Lancet Respir Med. 2016;4(9):708-719. doi:10.1016/S2213-2600(16)30152-7
Tocilizumab in systemic sclerosis: a randomised, double-blind, placebo-controlled, phase 3 trial
Tocilizumab in systemic sclerosis: a randomised, double-blind, placebo-controlled, phase 3 trial
- Khanna D, Lin CJF, Furst DE, et al; focuSSced investigators. Tocilizumab in systemic sclerosis: a randomised, double-blind, placebo-controlled, phase 3 trial. Lancet Respir Med. 2020;8(10):963-974. doi:10.1016/S2213-2600(20)30318-0
Working Group Publications
Rural Health
- DeDent AM, Shore JE, Bascom R, et al. Caring for rural patients with interstitial lung disease: perspectives of Pulmonary Fibrosis Foundation Care Center Network nurses and providers. CHEST Pulmonary. 2025;3(3):100136. doi:10.1016/j.chpulm.2025.100136
ILD Fellows Curriculum Working Group
- King SI, Neumeier A, Graney B, et al. Defining interstitial lung disease education in pulmonary fellowship: a mixed methods study. Chest. 2025;168(4):967–979. doi:10.1016/j.chest.2025.04.034
Advancing practitioner knowledge in interstitial lung disease: a review of free, online resources
- Hwang J, Cadden GC, Huie TJ, et al. Advancing practitioner knowledge in interstitial lung disease: a review of free, online resources. ATS Scholar. 2026;7(1):74–85. doi:10.1093/atsscholar/aapaf006
PFF Position Statements
Contact us
Please share with us any papers or guidelines that should be added to this resource at CCN@pulmonaryfibrosis.org