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Epidemiology of IPF

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Epidemiology of IPF

IPF is a rare disease

Up to 132,000 people are living with IPF in the US1,2
 
About 50,000 people are diagnosed with IPF annually1,2

1. Raghu G et al. Am J RespirCrit Care Med. 2006;174(7):810-816.
2. U.S. Census Bureau, Pop. Division. Annual Estimate of the Population for the United States, Regions, States, and Puerto Rico: April 1, 2011.

The incidence of IPF increased over a 10-year period

*The drop between 2002 and 2004 occurred due to changes in ICD coding for IPF and do not reflect an actual drop in incidence

3. Raghu G et al. Lancet Respir Med. 2014;2(7):566-572.

The prevalence of IPF increased over a 10-year period

3. Raghu G et al. Lancet Respir Med. 2014;2(7):566-572.

The most common risk factors for IPF are old age, history of smoking, and male gender

For more in-depth information, see About IPF – Risk Factors
 
4. King TE et al. Am J Respir Crit Care Med. 2001;164:1171-1181.
5. Kim DS et al. Proc Am Thorac Soc. 2006;3:285-292.
6. Douglass WW et al. Am J Respir Crit Care Med. 2000; 161:1172-1178.
7. Gribbin J et al. J Thorax. 2006;61:980-985.
8. Coultas DB et al. Am J Respir Crit Care Med. 1994;150(4):967-972.

The most common risk factors for IPF are old age, history of smoking, and male gender

During a recent study, the mean age at diagnosis of IPF was 63.9

4. King TE et al. Am J Respir Crit Care Med. 2001;164:1171-1181.
5. Kim DS et al. Proc Am Thorac Soc. 2006;3:285-292.
6. Douglass WW et al. Am J Respir Crit Care Med. 2000; 161:1172-1178.
7. Gribbin J et al. J Thorax. 2006;61:980-985.
8. Coultas DB et al. Am J Respir Crit Care Med. 1994;150(4):967-972.
9. van Moorsel CH et al. Am J Respir Crit Care Med. 2010;182(11):1419-1425.

The most common risk factors for IPF are old age, history of smoking, and male gender

Males have a higher prevalence of IPF than females.4,6-8

4. King TE et al. Am J Respir Crit Care Med. 2001;164:1171-1181.
5. Kim DS et al. Proc Am Thorac Soc. 2006;3:285-292.
6. Douglass WW et al. Am J Respir Crit Care Med. 2000; 161:1172-1178.
7. Gribbin J et al. J Thorax. 2006;61:980-985. 8. Coultas DB et al. Am J Respir Crit Care Med. 1994;150(4):967-972.
 

The most common risk factors for IPF are old age, history of smoking, and male gender

Smoking history shows a significant association with risk of developing IPF.6

4. King TE et al. Am J Respir Crit Care Med. 2001;164:1171-1181.
5. Kim DS et al. Proc Am Thorac Soc. 2006;3:285-292.
6. Douglass WW et al. Am J Respir Crit Care Med. 2000; 161:1172-1178.
7. Gribbin J et al. J Thorax. 2006;61:980-985.
8. Coultas DB et al. Am J Respir Crit Care Med. 1994;150(4):967-972.

Median survival after IPF diagnosis is only 2-3 years

Only 20%-40% of patients diagnosed with IPF survive 5 years 10-11,13-15
 
50% of patients with IPF survive 2 years

Approximately 30% of patients survive 5 years

10. Flaherty KR et al. Eur Respir J. 2002;19(2):275-283.
11. Nicholson AG et al.Am J Respir Crit Care Med. 2000;162(6):2213-2217.
12. Rudd RM et al. Thorax. 2007;62(1):62-66.
13. Bjoraker JA et al. Am J Respir Crit Care Med. 1998;157(1):199-203.
14. Jegal Y et al. Am J Respir Crit Care Med. 2005;171(6):639-644.
15. Nagai S et al. Sarcoidosis Vasc Diffuse Lung Dis. 1999;16(2):209-214.

IPF has lower 5-year survival than many familiar cancers

Out of many, more common serious diseases, only lung cancer has a lower 5-year survival rate than IPF16,17
 
16. American Cancer Society. Cancer Facts & Figures 2017. Atlanta: American Cancer Society; 2017.
17. Benza RL et al. Chest. 2012;142(2):448-456.

Survival time in IPF is difficult to calculate accurately

Survival in IPF is confounded by the various time points from which survival can be calculated5:

Time of first radiologic abnormality
Time of onset of symptoms
Time of diagnosis
 
5. Kim DS et al. Proc Am Thorac Soc. 2006;3(4):285-292.

Survival time depends on the frame of reference

A prospective analysis of 238 patients with IPF in the United States found that survival depended significantly on the point from which it was measured4
 
4. King TE et al. Am J Respir Crit Care Med. 2001;164:1171-1181

Patients with IPF are likely to die due to their disease

Data from the US Multiple Cause-of-Death mortality database (1992-2003) show that the majority of people with pulmonary fibrosis (94% of whom had IPF) had their death attributed to the disease itself18
 
18. Olson AL et al. Am J Respir Crit Care Med. 2007;176(3):277-284.

Most patients with IPF die of respiratory failure

The vast majority of prospectively recorded IPF-related deaths from 6 published clinical trials were due to respiratory causes19
 
19. Ley B et al. Am J Respir Crit Care Med. 2011;183(4):431-440.

The majority of respiratory-related deaths are due to subacute deterioration

These events were defined as worsening over a period of greater than 4 weeks to months19
 
19. Ley B et al. Am J Respir Crit Care Med. 2011;183(4):431-440.

Acute deteriorations cause a significant number of deaths

These events were defined as sudden worsening less than 4 weeks in duration19

For more information on acute exacerbations in IPF, visit About IPF: AE-IPF

19. Ley B et al. Am J Respir Crit Care Med. 2011;183(4):431-440.

Summary

  • IPF is a rare disease that causes lung fibrosis1
  • The reported prevalence of IPF increased over a 10-year period3
  • Patients with IPF have a poor prognosis10-11,13-15
    • Survival in IPF is closely tied to disease progression4
  • Acute deterioration accounts for a significant amount of deaths in patients with IPF19
1. Raghu G et al. Am J RespirCrit Care Med. 2006;174(7):810-816.
3. Raghu G et al. Lancet Respir Med. 2014;2(7):566-572.
10. Flaherty KR et al.EurRespir J. 2002;19(2):275-283.
4. King TE et al. Am J RespirCrit Care Med. 2001;164:1171-1181.
11. Nicholson AG et al. Am J RespirCrit Care Med. 2000;162(6):2213-2217.
12. Rudd RM et al. Thorax. 2007;62(1):62-66.
13. Bjoraker JA et al. Am J RespirCrit Care Med. 1998;157(1):199-203.
14. Jegal Y et al. Am J RespirCrit Care Med. 2005;171(6):639-644.
15. Nagai S et al. Sarcoidosis Vasc Diffuse Lung Dis. 1999;16(2):209-214.
19. Ley B et al. Am J RespirCrit Care Med. 2011;183(4):431-440.

References

1.Raghu G et al. Am J Respir Crit Care Med2006;174(7):810-816
2.U.S. Census Bureau, Pop. Division. Annual Estimate of the Population for the United States, Regions, States, and Puerto Rico: April 1, 2011
3.Raghu G et al. Lancet Respir Med. 2014;2(7):566-572
4.King TE et al. Am J Respir Crit Care Med. 2001;164:1171-1181
5.Kim DS et al. Proc Am Thorac Soc. 2006;3:285-292
6.Douglass WW et al. Am J Respir Crit Care Med. 2000; 161:1172-1178
7.Gribbin J et al. J Thorax. 2006;61:980-985
8.Coultas DB et al. Am J Respir Crit Care Med1994;150(4):967-972
9.van Moorsel CH et al. Am J Respir Crit Care Med. 2010;182(11):1419-1425
10.Flaherty KR et al. Eur Respir J. 2002;19(2):275-283
11.Nicholson AG et al.Am J Respir Crit Care Med. 2000;162(6):2213-2217
12.Rudd RM et al. Thorax. 2007;62(1):62-66
13.Bjoraker JA et al. Am J Respir Crit Care Med. 1998;157(1):199-203
14.Jegal Y et al. Am J Respir Crit Care Med. 2005;171(6):639-644
15.Nagai S et al. Sarcoidosis Vasc Diffuse Lung Dis. 1999;16(2):209-214
16.American Cancer Society. Cancer Facts & Figures 2017. Atlanta: American Cancer Society; 2017
17.Benza RL et al. Chest. 2012;142(2):448-456
18.Olson AL et al. Am J Respir Crit Care Med. 2007;176(3):277-284
19.19. Ley B et al. Am J Respir Crit Care Med. 2011;183(4):431-440