Individual fibrotic ILDs are rare, but collectively they affect a considerable number of patients1

Up to 246,000 people in the US are estimated to have an ILD Up to 246,000 people in the US are estimated to have an ILD
chart showing increase icon chart showing increase icon

Epidemiological studies have shown that incidence of interstitial lung disease
(ILD) has been increasing over the last three decades3,4

IPF is one of the most common ILDs2,5-7

Idiopathic pulmonary fibrosis (IPF) is a relentless, progressive, and deadly disease. IPF is estimated to affect about 140,000 people in the US. After diagnosis, median survival is only 3-5 years. Although the cause of IPF is unknown, there are risk factors for IPF, including:

  • Male gender
  • >60 years old
  • History of smoking
  • Microbial agents
  • Environmental exposures
  • Family history

IPF claims ~40,000 US lives every year—as many
as breast cancer8,9

ILD is a common, early, and potentially fatal manifestation of autoimmune diseases10-12

The lungs are a common site of disease involvement in autoimmune diseases, such as rheumatoid arthritis (RA) and systemic sclerosis (SSc). ILD often develops early in the course of disease, and may even be the presenting manifestation. Autoimmune ILDs can have varied clinical presentations and disease courses.

Up to 30% of patients with RA develop ILD. 39% of patients with RA-ILD may die within 5 years Up to 30% of patients with RA develop ILD. 39% of patients with RA-ILD may die within 5 years

Certain risk factors increase the likelihood of developing ILD in RA, including19,20:

  • 4x greater risk in males vs females
  • Typically occurs in people aged 40-50 years
  • History of smoking
  • Rheumatoid factor or anti-CCP antibody
 
Up to 80% of patients with SSc develop ILD. 18% of patients with SSc-ILD may die within 5 years Up to 80% of patients with SSc develop ILD. 18% of patients with SSc-ILD may die within 5 years

Certain risk factors increase the likelihood of developing ILD in SSc, including21-24:

  • Older age
  • Male gender
  • Short disease duration
  • Diagnosis of dcSSc
  • Presence of ATA

Maintain a high level of suspicion for ILD in
patients with known autoimmune diseases

chart showing decrease chart showing decrease Learn about the burden of ILD
exclamation mark graphic exclamation mark graphic Learn about the role of PCPs
book graphic book graphic View resources

ATA, anti-topoisomerase I antibodies; CCP, cyclic citrullinated peptide; dcSSc, diffuse cutaneous systemic sclerosis.

References

  1. Wijsenbeek M, Cottin V. N Engl J Med. 2020;383(10):958-968.
  2. Monthly Population Estimates for the United States: April 1, 2020 to December 1, 2022 (NA-EST2021-POP). US Census Bureau, Population Division; 2021.
  3. Kornum J et al. BMC Pulm Med. 2008;8(24). doi:10.1186/1471-2466-8-24
  4. Navaratnam V et al. Thorax. 2011;66:462-467.
  5. Oldham JM, Noth I. Respir Med. 2014;108(6):819-829.
  6. Raghu G et al. Am J Respir Crit Care Med. 2006;174(7):810-816.
  7. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824.
  8. National Institutes of Health. ARRA impact report: functional genomics of lung diseases. Accessed August 12, 2022. https://report.nih.gov/recovery/impac­treports/PrintPage.aspx?Id=14
  9. US Cancer Statistics Working Group. United States Cancer Statistics: 1999–2012 Incidence and Mortality Web-based Report. (http://nccd.cdc.gov/uscs/) Atlanta (GA): Department of Health and Human Services, Centers for Disease Control and Prevention, and National Cancer Institute; 2015.
  10. Fischer A, Distler J. Clin Rheumatol. 2019;38(10):2673-2681.
  11. Mathai SC, and Danoff SK. BMJ. 2016;352:h6819.
  12. Wells AU et al. Eur Respir J. 2018;51(5):1800692.
  13. Geerts S et al. Sarcoidosis Vasc Diffuse Lung Dis. 2017;34(4):326-335.
  14. Shaw M et al. Eur Respir Rev. 2015;24(135):1-16.
  15. Hyldgaard C et al. Respirology. 2017;22(3):494-500.
  16. Fischer A et al. Arthritis Rheumatol. 2019;71(2):182-195.
  17. Khanna D et al. Am J Respir Crit Care. 2020;201(6):650-660.
  18. Hoffmann-Vold AM et al. Am J Respir Crit Care Med. 2019;200(10):1258-1266.
  19. Esposito AJ et al. Clin Chest Med. 2019;40(3):545-560.
  20. Koo SM et al. Tuberc Respir Dis (Seoul). 2019;82(4)285-297.
  21. Cappelli S et al. Eur Respir Rev. 2015;24(137):411-419.
  22. Steen V. Ann Rheum Dis. 2003;62(2):97-99.
  23. Walker UA et al. Ann Rheum Dis. 2007;66(6):754-763.
  24. Assassi S et al. Arthritis Res Ther. 2010;12(5):R166.