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Editorial
. 2025 Oct;211(10):1751-1752.
doi: 10.1164/rccm.202505-1152ED.

Progress in Progressive Pulmonary Fibrosis

Jesse Roman  1
Affiliations
Editorial

Progress in Progressive Pulmonary Fibrosis

Jesse Roman. Am J Respir Crit Care Med. 2025 Oct.
No abstract available

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Figures

Figure 1.
Figure 1.
Interstitial lung diseases (ILDs) expected to experience progression. Idiopathic pulmonary fibrosis (IPF) and non-IPF ILDs with usual interstitial pneumonia (UIP) pattern on histology or imaging (UIP ILDs) are expected to progress. Progressive pulmonary fibrosis (PPF) is observed in non-IPF ILDs showing relative decline in FVC ⩾10% or DlCO ⩾15% within a year. IPF, non-IPF UIP ILD, and PPF should be considered for antifibrotic therapy.
See this image and copyright information in PMC

Comment on

  • Performance Characteristics for Physiological Measures of Progressive Pulmonary Fibrosis.
    Newton CA, Thenappan A, Liu GY, Yazbeck L, Lee CT, Pugashetti JV, Wang JM, White E, Flaherty KR, Belloli EA, Sheth JS, Mohan N, Nazemi N, Yu AR, Ghodrati S, Johannson KA, Marcoux V, Fisher JH, Assayag D, Manganas H, Khalil N, Kolb M, Morisset J, Garcia CK, Chua F, Strek ME, Khor YH, Adegunsoye A, Ryerson CJ, Molyneaux PL, Oldham JM. Newton CA, et al. Am J Respir Crit Care Med. 2025 Oct;211(10):1867-1875. doi: 10.1164/rccm.202501-0317OC. Am J Respir Crit Care Med. 2025. PMID: 40530983 Free PMC article.

References

    1. Travis WD, Costabel U, Hansell DM, King TE, Jr, Lynch DA, Nicholson AG. et al. ATS/ERS Committee on Idiopathic Interstitial Pneumonias. An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med . 2013;188:733–748. - PMC - PubMed
    1. Daniil ZD, Gilchrist FC, Nicholson AG, Hansell DM, Harris J, Colby TV. et al. A histologic pattern of nonspecific interstitial pneumonia is associated with a better prognosis than usual interstitial pneumonia in patients with cryptogenic fibrosing alveolitis. Am J Respir Crit Care Med . 1999;160:899–905. - PubMed
    1. Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK. et al. ATS/ERS/JRS/ALAT Committee on Idiopathic Pulmonary Fibrosis. An official ATS/ERS/JRS/ALAT statement. Idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med . 2011;183:788–824. - PMC - PubMed
    1. Finnerty JP, Ponnuswamy A, Dutta P, Abdelaziz A, Kamil H. Efficacy of antifibrotic drugs, nintedanib and pirfenidone, in treatment of progressive pulmonary fibrosis in both idiopathic pulmonary fibrosis (IPF) and non-IPF: a systematic review and meta-analysis. BMC Pulm Med . 2021;21:411. - PMC - PubMed
    1. Cottin V, Hiani NA, Hotchkin DL, Nambiar AM, Ogura T, Otaola M. et al. Presentation, diagnosis and clinical course of the progressive-fibrosing interstitial lung diseases. Eur Resir Rev . 2018;27:180076. - PMC - PubMed

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