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Recognize and refer suspected pulmonary fibrosis

Early referral to an interstitial lung disease (ILD) specialist can help minimize the potential negative impact of delay in fibrotic ILD diagnosis1–4

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PROMPTLY REFER

Good reason to promptly refer is that a delay in identification of ILD can leave pulmonary fibrosis unchecked and shorten your patient’s life4–7

Delayed referral could negatively impact survival in patients with IPF, a well-known example of a progressive fibrotic ILD3,4

Survival in IPF patients decreases as the delay in referral increases*3

survival ipf patient decrease

*IPF is a well-known example of a fibrotic ILD that is always progressive.8 Survival from the time of evaluation at a speciality care center adjusted for age and lung function across quartiles of delay. Prospective observational study of 129 adults referred to a single specialist care unit.3 
Reprinted with permission of the American Thoracic Society. Copyright © 2018 American Thoracic Society.
Lamas D, Kawut SM, Bagiella E, et al. Delayed access and survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011;184(7):842–847.
The American Journal of Respiratory and Critical Care Medicine is an official journal of the American Thoracic Society.

MANAGEMENT OPTIONS

Although there is no cure, management options are available for fibrotic ILDs that can slow disease progression, making early identification imperative1,2,8,9

Early identification of fibrotic ILD may reduce the risk of mortality and help maintain patients’ quality of life1–4,10

  • Early referral can enable early access to HRCT and multidisciplinary evaluation, the key to early and accurate diagnosis of fibrotic ILDs1,2,11–13

Prompt referral of suspected fibrotic ILD can enable early access to the appropriate treatment1,2

  • A delay in treatment can keep a patient with ILD at a higher risk of an unpredictable life-threatening acute exacerbation14
  • Early access to appropriate treatment is key to helping slow progression and improve survival in patients with IPF, which can rapidly progress and lead to early mortality15–22

Time is of the essence in fibrotic ILDs… You play a crucial role in promptly recognizing and referring patients with suspected ILD1,2

Abbreviations

HRCT, high-resolution computed tomography; ILD, interstitial lung disease; IPF, idiopathic pulmonary fibrosis.

  1. Zibrak JD, Price D. Interstitial lung disease: raising the index of suspicion in primary care. NPJ Prim Care Respir Med. 2014;24:14054.
  2. Molina-Molina M, Aburto M, Acosta O, et al. Importance of early diagnosis and treatment in idiopathic pulmonary fibrosis. Exp Rev Resp Med. 2018;12(7):537–539.
  3. Lamas D, Kawut SM, Bagiella E, et al. Delayed access and survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011;184(7):842–84.
  4. Cosgrove GP, Bianchi P, Danese S, Lederer DJ. Barriers to timely diagnosis of interstitial lung disease in the real world: the INTENSITY survey. BMC Pulm Med. 2018;18(1):9.
  5. Cottin V, Richeldi L. Neglected evidence in idiopathic pulmonary fibrosis and the importance of early diagnosis and treatment. Eur Respir Rev. 2014;23(131):106–110.
  6. Wijsenbeek MS, Kreuter M, Olson A, et al. Progressive fibrosing interstitial lung diseases: current practice in diagnosis and management. Curr Med Res Opin. 
    2019:1–10.
  7. Cottin V, Hirani N, Hotchkin D, et al. Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150):180076.
  8. Cottin V. Treatment of progressive fibrosing interstitial lung diseases: a milestone in the management of interstitial lung diseases. Eur Respir Rev. 2019;28(153):190109.
  9. Borie R, Le Guen P, Ghanem M, et al. The genetics of interstitial lung diseases. Eur Respir Rev. 2019; 28(153):190053.
  10. Cano-Jimenez E, Rodriguez TV, Martin-Robles I, et al. Diagnostic delay of associated interstitial lung disease increases mortality in rheumatoid arthritis. Sci Rep. 2021;11(1):9184.
  11. Walsh SLF. Multidisciplinary evaluation of interstitial lung diseases: current insights. Eur Respir Rev. 2017;26(144):170002.
  12. De Sadeleer LJ, Meert C, Yserbyt J, et al. Diagnostic ability of a dynamic multidisciplinary discussion in interstitial lung diseases: A retrospective observational study of 938 cases. Chest. 2018;153(6):1416–1423.
  13. Cottin V, Cordier J-F. Velcro crackles: the key for early diagnosis of idiopathic pulmonary fibrosis? Eur Respir J. 2012;40(3):519–521.
  14. Kolb M, Bondue B, Pesci A, et al. Acute exacerbations of progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150):pii:180071.
  15. Ley B, Collard HR, King Jr TE. Clinical course and prediction of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011;183(4):431–440.
  16. Kolb M, Richeldi L, Behr J, et al. Nintedanib in patients with idiopathic pulmonary fibrosis and preserved lung volume. Thorax. 2017 Apr;72(4):340–346.
  17. Torrisi SE, Pavone M, Vancheri A, et al. When to start and when to stop antifibrotic therapies. Eur Respir Rev. 2017;26(145):170053.
  18. Maher TM, Molina-Molina M, Russell AM, et al. Unmet needs in the treatment of idiopathic pulmonary fibrosis-insights from patient chart review in five European countries. BMC Pulm Med. 2017;17(1):124.
  19. Guenther A, Krauss E, Tello S, et al. The European IPF registry (eurIPFreg): baseline characteristics and survival of patients with idiopathic pulmonary fibrosis. Respir Res. 2018;19(1):141.
  20. Jo HE, Glaspole IN, Levin KC, et al. Clinical impact of the interstitial lung disease multidisciplinary service. Respirology. 2016;21:1438–1444.
  21. Behr J, Prasse A, Wirtz H, et al. Survival and course of lung function in the presence or absence of antifibrotic treatment in patients with idiopathic pulmonary fibrosis: long-term results of the INSIGHTS-IPF registry. Eur Respir J. 2020;56(2):1902279.
  22. Kaunisto J, Salomaa E, Hodgson U. Demographics and survival of patients with idiopathic pulmonary fibrosis in the Finnish IPF registry. ERJ Open Res. 
    2019;5:00170–2018.

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