IPF – Epidemiology
- Up to approximately 140,000 people are living with IPF in the US1,2
- About 50,000 people are diagnosed with IPF annually1,2
THE INCIDENCE OF IPF INCREASED OVER A 10-YEAR PERIOD3
Adapted from Raghu G et al3
*The drop between 2002 and 2004 occurred due to changes in ICD coding for IPF and do not reflect an actual drop in incidence.
THE PREVALENCE OF IPF INCREASED OVER A 10-YEAR PERIOD4
Adapted from Raghu G et al4
MEDIAN SURVIVAL AFTER IPF DIAGNOSIS CAN BE AS LOW AS 2-3 YEARS.5-8
- Only 20%-40% of patients diagnosed with IPF survive
5 years5,6,8-10
IPF HAS A WORSE 5-YEAR SURVIVAL RATE THAN COMMON CANCERS.
Only lung cancer has a lower 5-year survival rate than IPF10-12
SURVIVAL TIME IN IPF IS DIFFICULT TO CALCULATE ACCURATELY.
Survival in IPF is confounded by the various time points from which survival can be calculated:13
- Time of first radiologic abnormality
- Time of onset of symptoms
- Time of diagnosis
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 measured14
- First onset of symptoms - median survival was 80.8 months
- First clinic visit - median survival was 35.2 months
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 itself.15
MOST PATIENTS WITH IPF DIE OF RESPIRATORY FAILURE.
Most prospectively recorded IPF-related deaths from 6 published clinical trials were due to respiratory causes.12
Cause of Death:
- Respiratory - 77%
- Non-respiratory - 18%
- Unknown - 5%
MOST RESPIRATORY-RELATED DEATHS ARE DUE TO SUBACUTE DETERIORATION.
These events were defined as worsening over a period of greater than 4 weeks to months.12
Cause of Death:
- Subacute respiratory - 47%
- Acute Respiratory - 30%
- Non-respiratory - 18%
- Unknown - 5%
ACUTE DETERIORATIONS CAUSE A SIGNIFICANT NUMBER OF DEATHS.
These events were defined as sudden worsening less than 4 weeks in duration.12
Cause of Death:
- Subacute respiratory - 47%
- Acute Respiratory - 30%
- Non-respiratory - 18%
- Unknown - 5%
REFERENCES
- Raghu G, Weycker D, Edelsberg J, Bradford WZ, Oster G. Incidence and prevalence of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2006;174(7):810-816. doi:10.1164/rccm.200602-163OC.
- U.S. Census Bureau, Pop. Division. Annual Estimate of the Population for the United States, Regions, States, and Puerto Rico: April 1, 2011.
- Raghu G, Chen S-Y, Yeh W-S, et al. Idiopathic pulmonary fibrosis in US Medicare beneficiaries aged 65 years and older: incidence, prevalence, and survival, 2001-11. Lancet Respir Med. 2014;2(7):566-572. doi: 10.1016/S2213-2600(14)70101-8.
- Raghu G, Chen SY, Hou Q, Yeh WS, Collard HR. Incidence and prevalence of idiopathic pulmonary fibrosis in US adults 18-64 years old. Eur Respir J. 2016;48(1):179-186. doi:10.1183/13993003.01653-2015.
- Flaherty KR, Toews GB, Travis WD, et al. Clinical significance of histological classification of idiopathic interstitial pneumonia. Eur Respir J. 2002;19(2):275-283. doi: 10.1183/09031936.02.00182002.
- Nicholson AG, Colby TV, du Bois RM, et al. The prognostic significance of the histologic pattern of interstitial pneumonia in patients presenting with the clinical entity of cryptogenic fibrosing alveolitis. Am J Respir Crit Care Med. 2000;162(6):2213-2217. doi: 10.1164/ajrccm.162.6.2003049.
- Rudd RM, Prescott RJ, Chalmers JC, et al. British Thoracic Society Study on cryptogenic fibrosing alveolitis: Response to treatment and survival. Thorax. 2007;62(1):62-66. doi: 10.1136/thx.2005.045591.
- Bjoraker JA, Ryu JH, Edwin MK, et al. Prognostic significance of histopathologic subsets in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 1998;157(1):199-203. doi: 10.1164/ajrccm.157.1.9704130.
- Jegal Y, Kim DS, Shim TS, et al. Physiology is a stronger predictor of survival than pathology in fibrotic interstitial pneumonia. Am J Respir Crit Care Med. 2005;171(6):639-644. doi: 10.1164/rccm.200403-331OC.
- Nagai S, Kitaichi M, Hamada K, et al. Hospital-based historical cohort study of 234 histologically proven Japanese patients with IPF. Sarcoidosis Vasc Difluse Lung Dis. 1999;16(2):209-214.
- American Cancer Society. Cancer Facts and Figures 2021. Atlanta, GA: American Cancer Society; 2021.
- Ley B, Collard HR, King TE Jr. Clinical course and prediction of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011;183(4):431-440. doi:10.1164/rccm.201006-0894CI.
- Kim DS, Collard HR, King TE. Classification and natural history of the idiopathic interstitial pneumonias. Proc Am Thorac Soc. 2006;3(4):285-292. doi: 10.1513/pats.200601-005TK.
- King TE, Tooze JA, Schwarz MI, et al. Predicting survival in idiopathic pulmonary fibrosis: scoring system and survival model. Am J Respir Crit Care Med. 2001;164(7):1171-81. doi: 10.1164/ajrccm.164.7.2003140.
- Olson AL, Swigris JJ, Lezotte DC, et al. Mortality from pulmonary fibrosis increased in the United States from 1992 to 2003. Am J Respir Crit Care Med. 2007; 176(3):277-284. doi: 10.1164/rccm.200701-044OC.
