About Interstitial Lung Disease (ILD)

Interstitial lung diseases (ILDs) encompass over 200 distinct pulmonary disorders characterized by varying degrees of inflammation and fibrosis affecting the lung interstitium, the area surrounding the alveoli. These conditions compromise gas exchange, causing symptoms such as progressively worsening shortness of breath on exertion, persistent dry cough, and eventual respiratory failure in advanced stages [1,2].

Idiopathic pulmonary fibrosis (IPF) represents the most prevalent and extensively studied fibrotic ILD. IPF is a chronic, progressive fibrosing lung disease of unknown origin, typically resulting in declining respiratory function. The disease course varies substantially among patients, with a median survival of approximately 2 to 5 years post-diagnosis. Early diagnosis and antifibrotic therapies can, however, slow disease progression and improve patient outcomes [3-5].

Accurate diagnosis of ILDs often requires multidisciplinary discussions (MDD) involving pulmonologists, radiologists, pathologists, rheumatologists, and other specialists. The diagnostic process integrates clinical assessment, high-resolution computed tomography (HRCT), pulmonary function tests, and occasionally bronchoalveolar lavage or lung biopsies [6].

Although fibrotic ILDs currently have no cure, antifibrotic therapies such as pirfenidone and nintedanib have significantly improved disease management. These treatments, initially approved for IPF, slow lung function decline and are being increasingly investigated or approved for other progressive fibrotic ILDs (e.g. progressive pulmonary fibrosis, PPF) [7-9].

Real-world evidence indicates variability in individual treatment responses to antifibrotic therapies. Current research is focused on discovering circulating and imaging biomarkers, improving patient stratification, and personalizing treatment approaches to maximize patient benefit [10-12].

Additionally, innovative technologies such as artificial intelligence (AI)-assisted imaging tools are being developed to facilitate earlier disease detection, enhance diagnostic accuracy, and improve prognosis assessment, potentially transforming patient care strategies in ILD management [13].

References

  1. Wijsenbeek M, Cottin V. Spectrum of Fibrotic Lung Diseases. N Engl J Med. 2020;383(10):958-68.
  2. Ryerson CJ, Adegunsoye A, Piciucchi S, Hariri LP, Khor YH, Wijsenbeek MS, et al. Update of the international multidisciplinary classification of the interstitial pneumonias: an ERS/ATS statement. Eur Respir J. 2025;66(6).
  3. Raghu G, Remy-Jardin M, Richeldi L, Thomson CC, Inoue Y, Johkoh T, 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.
  4. Koudstaal T, Funke-Chambour M, Kreuter M, Molyneaux PL, Wijsenbeek MS. Pulmonary fibrosis: from pathogenesis to clinical decision-making. Trends Mol Med. 2023;29(12):1076-87.
  5. Zheng  Q, Cox IA, Campbell JA. Mortality and survival in idiopathic pulmonary fibrosis: a systematic review and meta-analysis. Eur Respir Rev. 2022 8(1):00591-2021
  6. Cottin V, Martinez FJ, Smith V, Walsh S L F. Multidisciplinary teams in the clinical care of fibrotic interstitial lung disease: current perspectives. Eur Respir Rev. 2022 Sep 7;31(165):220003
  7. 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):2071–82. doi: 10.1056/NEJMoa1402584
  8. 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):2083–92. doi: 10.1056/NEJMoa1402582
  9. Flaherty KR, Wells AU, Cottin V, Devaraj A, Walsh SLF, Inoue Y, Richeldi L. Nintedanib in Progressive Fibrosing Interstitial Lung Diseases. N Engl J Med. 2019;381:1718-1727
  10. Kreuter M, Wijsenbeek M, Vasakova M, et al. Real-world effectiveness and safety of nintedanib in patients with idiopathic pulmonary fibrosis: Results from the European multicentre EMPIRE registry. Respir Res. 2021;22:305. doi: 10.1186/s12931-021-01884-0
  11. Bowman WS, Newton CA, Linderholm AL, Neely ML, Pugashetti JV, Kaul B, et al. Proteomic biomarkers of progressive fibrosing interstitial lung disease: a multicentre cohort analysis. Lancet Respir Med. 2022;10(6):593-602.
  12. Miceli V, Callari A, Calzolari E, Castelbuono S, Claudia C, Lanzarone N, et al. Circulating biomarkers in patients with progressive fibrosing interstitial lung disease treated with nintedanib: a pilot study. Sci Rep. 2025;15(1):27115.
  13. Walsh SLF, Calandriello L, Silva M, Sverzellati N. Deep learning for classifying fibrotic lung disease on high-resolution computed tomography: a case–cohort study. Lancet Respir Med. 2018;6(11):837–845. doi: 10.1016/S2213-2600(18)30256-1