ILDs are a heterogeneous group of lung disorders that vary in etiology2

There are over 200 types of interstitial lung diseases (ILDs) of known or unknown causes.3,4 Many ILDs are fibrotic.5,6 Fibrotic ILDs are characterized by irreversible scarring of lung tissue, known as pulmonary fibrosis.7,8

ILD lung disorders chart showing types of ILDs flow_chart_mob

Reproduced with permission of the ©ERS 2020. European Respiratory Review 27 (150)180076; DOI: 10.1183/16000617.0076-2018
Published December 21, 2018.

*Eg, asbestosis, silicosis.
  • IPF is one of the most common ILDs and has no known cause2
  • ILD is a common manifestation in autoimmune diseases, such as systemic sclerosis (SSc) and rheumatoid arthritis (RA)10-14

Pulmonary fibrosis can be progressive, leading to worse outcomes6,15

IPF is considered the most typical progressive fibrosing ILD. By definition, all patients with IPF have progressive disease. Pulmonary fibrosis can also be progressive in other ILDs and is associated with a poor prognosis.

Pulmonary fibrosis scars and stiffens tissue, making it harder to breathe7,8,16

fibrotic lung graphic showing distortion of lung architecture and impaired gas exchange fibrotic_lung_mob

Pulmonary fibrosis can take your patients' independence. As fibrosis increases, breathing becomes more difficult and symptoms worsen, which can impact daily living20:

worsening dyspnea icon

Worsening dyspnea and cough

limited ability icon

Limited ability to perform day-to-day activities

emotional well being icon

Strain on emotional well-being

Progressive pulmonary fibrosis can lead to
irreversible lung damage and early mortality,
warranting urgent referral and identification2,7,8

ILD patient 1

It got to the point where I couldn’t even open a bottle of water and I was too fatigued to step foot into the grocery store. Many days, I couldn’t even get off the couch. I had to start reaching out for help.
-Kheesa, living with SSc-ILD

ILD patient 2

Minor tasks had become more difficult. Talking and walking provoked coughing. […] At night, after I climbed the stairs to put our daughter to bed, I had to sit by the side of her bed to catch my breath.
-Maryluz, recipient of a lung transplant for IPF

ILD patient 3

I was sick a lot, had walking pneumonia, breathing issues, wheezing and coughing. It got to the point that I could no longer do yard work, which was something so ingrained in my life.
- Jim, living with RA-ILD


References

  1. Wijsenbeek M, Cottin V. N Engl J Med. 2020;383(10):958-968.
  2. Oldham JM, Noth I. Respir Med. 2014;108(6):819-829.
  3. Cottin V et al. Eur Respir Rev. 2018;27(150):180076.
  4. Ryu JH et al. Mayo Clinic Proc. 2007;82(8):976-986.
  5. Wijsenbeek M et al. Curr Med Res Opin. 2019;35(11):2015-2024.
  6. Cottin V et al. Eur Respir Rev. 2019;28(153):180100.
  7. Wollin L et al. Eur Respir J. 2019;54(3):1900161.
  8. Selman M et al. Ann Intern Med. 2001;134(2):136-151.
  9. Demedts M et al. Eur Respir J. 2001;18(suppl 32):2s-16s.
  10. Fischer A, Distler J. Clin Rheumatol. 2019;38(10):2673-2681.
  11. Geerts S et al. Sarcoidosis Vasc Diffuse Lung Dis. 2017;34(4):326-335.
  12. Shaw M et al. Eur Respir Rev. 2015;24(135):1-16.
  13. Fischer A et al. Arthritis Rheumatol. 2019;71(2):182-195.
  14. Khanna D et al. Am J Respir Crit Care Med. 2020;201(6):650-660.
  15. Wells AU et al. Eur Respir J. 2018;51(5):1800692.
  16. Tonelli R et al. BMC Pulm Med. 2017;17(1):130.
  17. Wells AU, Denton CP. Nat Rev Rheumatol. 2014;10(12):728-739.
  18. Bagnato G, Harari S. Eur Respir Rev. 2015;24(135):102-114.
  19. Kolb M, Vasakova M. Respir Res. 2019;20(1):57.
  20. Swigris JJ et al. Eur Respir Rev. 2018;27(150):180075.
  21. Cosgrove GP et al. BMC Pulm Med. 2018;18(1):9.