Monitoring for ILD progression

Worsening of symptoms or decline in PFT scores can mean that a patient’s pulmonary fibrosis is progressing1

Three patients holding lung scan results in their hands.

A proportion of patients with ILDs may develop a progressive fibrosing phenotype.2–5

Once alternative explanations, such as respiratory tract infection, have been excluded and treated, patients with ILDs meeting the following criteria within 24 months despite treatment in clinical practice can be considered to have progressive fibrosis:6

  • Relative decline of ≥10% in FVC

  • Relative decline of ≥5–<10% in FVC and relative decline of ≥15% in DLco

  • Relative decline of ≥5–<10% in FVC and worsening of respiratory symptoms

  • Relative decline of ≥5–<10% in FVC and increased extent of fibrosis on HRCT

  • Worsening of respiratory symptoms and increased extent of fibrosis on HRCT



Progressive fibrosing ILD has a significant impact on patients, including early mortality:2–5,7

Arrow pointing down in the graph representing a poor survival following an acute exacerbation of IPF.

ILDs can cause progressive deterioration in lung function, respiratory symptoms and quality of life3,4,8–11

Icon of lungs affected by the extent of fibrosis.

If ILD cannot be stabilized within 6–12 months, patients with cHP and NSIP will have ongoing lung function decline and increased mortality6

Progressive fibrosing ILD is characterized by progression of pulmonary fibrosis, worsening of symptoms, decline in lung function and deterioration in health-related quality of life4

Characteristics and potential impacts of the progressive fibrosing phenotype

Find out more about the impacts of the progressive fibrosing phenotype



Time is of the essence when progressive fibrosing ILD is suspected:3

  • Delayed detection of progressive pulmonary fibrosis in ILDs can be deadly: patients have an estimated survival time of 2.5–4 years after detection of progressive fibrosis2–3†

  • Early detection of patients with progressive fibrosing ILD could inform management decisions and patient counseling2



In an online physician survey, it was estimated that it takes 9–12 months for the progressive fibrosing phenotype to be detected after it develops2†

Estimated time periods between initial ILD symptoms and detection of the progressive fibrosing phenotype in patients with different ILDs2†

Average waiting time for ILD diagnosis

Current Medical Research and Opinion, 2019, reprinted by permission of the publisher Informa UK Limited trading as Taylor & Francis Ltd.
Patient journey in non-IPF progressive fibrosing ILDs as estimated by an online physician survey.2†
†  Estimates of patients with non-IPF ILD that develop progressive fibrosis based on an online survey of total 486 physicians (243 pulmonologists, 203 rheumatologists and 40 internists) from the United States, Japan, France, Germany, Italy, Spain and the United Kingdom.2

Multidisciplinary evaluation is the gold standard in the evaluation of ILD severity13

A multidisciplinary team including a pulmonologist, radiologist and histopathologist should be involved in ILD diagnosis

Diagnosis and monitoring of ILDs that may present a progressive fibrosing phenotype3

Diagnosis and monitoring algorithm of progressive fibrosing ILDs

Adapted from: Cottin V, Hirani N, Hotchkin D, et al. Presentation, diagnosis and clionical course of the spectrum of progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150):180076.


Icon of person coughing representing worsening respiratory symptoms.

Worsening respiratory symptoms

HRCT scan icon.


Spirometer icon.


Monitoring ILD progression using PFTs: 

  • Deterioration in PFTs should trigger urgency for further investigation — because pulmonary fibrosis is unpredictable1,14

  • A decline in DLCO is often considered a sign of progressive ILD, particularly when accompanied by a decline in FVC or increased radiological evidence of fibrosis on HRCT over time6


Confirmation of ILD progression using HRCT: 

  • For patients at risk of developing a progressive fibrosing phenotype, HRCT should be evaluated upon worsening of either PFT scores, or respiratory symptoms3,15–17


Other considerations when monitoring for ILD progression

Icon of down graph symbol illustrating seriousness in patients with chronic fibrotic HP.

Acute exacerbation of ILD can be considered an indicator of progressive fibrosis, as lung function irreversibly declines as a result6

Icon representing improvement after pulmonary rehabilitation in patients with ILD

Serial measurement of 6MWD can be helpful in identifying progressive fibrosing ILD, as decline in the 6MWD can provide supportive evidence for progressive fibrosis6



Due to the variable and unpredictable nature of pulmonary fibrosis in SSc, vigilant ongoing monitoring should be conducted, with HRCT repeated upon worsening of  PFT scores, or respiratory symptoms:11,18–21

  • All patients with SSc-ILD need to be followed up every 3–6 months with PFTs, and repeated HRCT as guided by clinical decision19,20


Monitoring algorithm of SSc-ILD19

Algorithm for SSc-ILD screening / diagnosis recommending primary tool HRCT

Algorithm provides brief summary of evidence-based consensus statements (including supplemental Delphi process) for identification and management of SSc-ILD for use in clinical practice. Data from patients with SSc-ILD should be extrapolated to other CTDs with caution.
This article was published in The Lancet Rheumatology, 2(2), Hoffmann-Vold et al, The identification and management of interstitial lung disease in systemic sclerosis: evidence-based European consensus statements, E71-E83, Copyright Elsevier 2022.




Follow-up is mandatory in patients with asymptomatic RA-ILD to identify progressive disease:22

  • Monitoring for progression of RA-ILD using PFTs is recommended every 3–6 months23

What management options should you consider for your patients with fibrotic ILDs?

  • 6MWD, six-minute walking distance; BAL, bronchoalveolar lavage; cHP, chronic hypersensitivity pneumonitis; CTD-ILD, connective tissue disease-associated interstitial lung disease; DLCO, diffusing capacity of the lung for carbon; FVC, forced vital capacity; HP, hypersensitivity pneumonitis; HRCT, high-resolution computed tomography; HRQoL, health-related quality of life; IIP, idiopathic interstitial pneumonia; ILD, interstitial lung disease; iNSIP, idiopathic non-specific interstitial pneumonia; MDT, multidisciplinary team; NSIP, non-specific interstitial pneumonia; PFT, pulmonary function test; RA-ILD, rheumatoid arthritis-associated interstitial lung disease; SSc, systemic sclerosis; SSc-ILD, systemic sclerosis-associated interstitial lung disease.

  1. Raghu G, Collard HR, Egan JJ, et al; on behalf of the 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(6):788-824.

  2. Wijsenbeek M, Kreuter M, Olson A, et al. Progressive fibrosing interstitial lung diseases: current practice in diagnosis and management. Curr Med Res Opin. 2019;35(11):2015–2024.

  3. 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.

  4. Kolb M, Vašáková M. The natural history of progressive fibrosing interstitial lung diseases. Respir Res. 2019;20(1).

  5. Cottin V, Wollin L, Fischer A, et al. Fibrosing interstitial lung diseases: knowns and unknowns. Eur Respir Rev. 2019b;28(151):pii:180100.

  6. George PM, Spagnolo P, Kreuter M, et al. Progressive fibrosing interstitial lung disease: clinical uncertainties, consensus recommendations, and research priorities. Lancet Respir Med. 2020;8:925–954.

  7. Flaherty KR, Brown KK, Wells AU, et al. Design of the PF-ILD trial: A double-blind, randomised, placebo-controlled phase III trial of nintedanib in patients with progressive fibrosing interstitial lung disease. BMJ Open Resp Res. 2017;4(1):e000212.

  8. 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.

  9. 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.

  10. 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.

  11. Chowaniec M, Skoczyńska M, Sokolik R, Wiland P. Interstitial lung disease in systemic sclerosis: challenges in early diagnosis and management. Reumatologia. 2018;56(4):249–254.

  12. Cottin V, Brown KK. Interstitial lung disease associated with systemic sclerosis (SSc–ILD). Respir Res. 2019a;20(1):13.

  13. Chaudhuri N, Spencer L, Greaves M, et al. A Review of the Multidisciplinary Diagnosis of Interstitial Lung Diseases: A Retrospective Analysis in a Single UK Specialist Centre. J Clin Med. 2016;5(66):1–9.

  14. Martinez FJ, Flaherty K. Pulmonary function testing in idiopathic interstitial pneumonias. Proc Am Thorac Soc. 2006;3(4):315–321.

  15. Roofeh D, Jaafar S, Vummidi D, Khanna D. Management of systemic sclerosis-associated interstitial lung disease. Curr Opin Rheumatol. 2019;31(3):241–249.

  16. Walsh SLF, Devaraj A, Enghelmayer JI, et al. Role of imaging in progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150):pii:180073.

  17. Elicker BM, Kallianos KG, Henry TS. The role of high-resolution computed tomography in the follow-up of diffuse lung disease: Number 2 in the Series “Radiology” Edited by Nicola Sverzellati and Sujal Desai. Eur Respir Rev. 2017;26(144):pii:170008.

  18. Hoffmann-Vold AM, Allanore Y, Alves M, et al. Progressive interstitial lung disease in patients with systemic sclerosis-associated interstitial lung disease in the EUSTAR database. Ann Rheum Dis. 2020;217455.

  19. Hoffmann-Vold AM, Maher TM, Philpot EE, et al. The identification and management of interstitial lung disease in systemic sclerosis: evidence-based European consensus statements. The Lancet Rheumatology. 2020b;2: e71–e83.

  20. Distler O, Assassi S, Cottin V, et al. Predictors of progression in systemic sclerosis patients with interstitial lung disease. Eur Respir J. 2020;55, 1902026.

  21. Cappelli S, Bellando Randone S, Camiciottoli G, et al. Interstitial lung disease in systemic sclerosis: where do we stand? Eur Respir Rev. 2015;24:411–419.

  22. Cassone G, Manfredi A, Vacchi C, et al. Treatment of rheumatoid arthritis-associated interstitial lung disease: lights and shadows. J Clin Med. 2020;9(4):1082.

  23. Iqbal K and Kelly C. Treatment of rheumatoid arthritis-associated interstitial lung disease: a perspective review. Ther Adv Musculoskelet Dis. 2015;7(6):247–267.

  24. Ryerson CJ, Cayou C, Topp F, et al. Pulmonary rehabilitation improves long-term outcomes in interstitial lung disease: a prospective cohort study. Respir Med. 2014;108(1):203-210.

  25. Kreuter M, Bendstrup E, Russell A, et al. Palliative care in interstitial lung disease: living well. Lancet Respir Med. 2017;5(12):968-980.

  26. Maher TM, Wuyts W. Management of Fibrosing Interstitial Lung Diseases. Adv Ther. 2019;doi:10.1007/s12325-019-00992-9. [Epub ahead of print].

  27. Sgalla G, Cerri S, Ferrari R, et al. Mindfulness-based stress reduction in patients with interstitial lung diseases: a pilot, single-centre observational study on safety and efficacy. BMJ Open Respir Res. 2015;2(1):e000065.

  28. Wijsenbeek M, Cottin V. Spectrum of Fibrotic Lung Diseases. N Engl J Med. 2020;383:958–968.

  29. 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.

  30. Robalo-Cordeiro C, Campos P, Carvalho L, et al. Idiopathic pulmonary fibrosis in the era of antifibrotic therapy: Searching for new opportunities grounded in evidence. Rev Port Pneumol. 2017;23(5):287–293.

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