Introduction to IPF

  • Idiopathic pulmonary fibrosis (IPF) is a devastating disease that is characterized as a progressive, irreversible, and fatal lung disease of unknown cause1,2
  • Progressive fibrosis, which occurs in IPF, leads to permanent, irreversible loss of lung function2
  • Diagnosis involves a systematic evaluation of clinical, physiologic, and radiologic findings1,3,4
  • View the Diagnosis section for more information

IPF Is a Progressive and Fatal Interstitial Lung Disease

  • IPF belongs to a large family of lung diseases known as interstitial lung diseases (ILDs), which are differentiated based on clinical, physiologic, radiologic, and pathologic findings1,4
  • IPF is the clinical syndrome associated with the morphologic pattern of usual interstitial pneumonia (UIP)1,5
  • As an ILD, IPF is the most common idiopathic interstitial pneumonia6
List not exhaustive. Please see Reference 4.

Figure 1. A schematic of commonly seen ILDs that may share similar features to IPF, including the subcategories of idiopathic interstitial pneumonia, connective tissue disease, and hypersensitivity pneumonitis.4
  • Survival with IPF is worse than that of many cancers6,7 and pulmonary arterial hypertension (PAH)8
Figure 2. The 5-year survival rate for IPF is worse than many common cancers and pulmonary arterial hypertension. Image adapted from the American Cancer Society ( SEER Cancer Statistics Review, 1975-2012; du Bois. Eur Respir Rev. 2012;21:141-146; Benza, et al. Chest. 2012;142:448-456; Kistler, et al. BMC Pulm Med. 2014;14:139.
  • The median survival of patients with IPF is approximately 3.8 years9
    • The primary cause of death in patients with IPF is often respiratory failure due to progression of their lung fibrosis1,10
    • Leading causes of death were respiratory and heart disease1,10

Median Survival Time of Patients With IPF

Survival time is from time of initial pulmonary function test evaluation.

Figure 3. Image adapted from Nathan, et al. Chest. 2011;140:221-229.

There Are Patient Characteristics Commonly Seen in Patients With IPF

  • Most patients with IPF are greater than 50 years of age1
  • More men have been reported to have IPF compared with women, with the majority reporting a history of smoking1
  • Many patients with IPF have serious comorbidities, which may include11*:
    • Cardiovascular disease (includes coronary artery disease)
    • Chronic obstructive pulmonary disease (COPD)
    • Gastroesophageal reflux disease (GERD)
    • Lung cancer
    • Metabolic disorders
    • Obstructive sleep apnea
    • Pulmonary arterial hypertension
    • Pulmonary embolism
Listed in alphabetical order
Includes diabetes, hypercholesterolemia/hyperlipidemia, and weight disorders

Prevalence and Incidence of IPF Are Increasing

  • IPF is more common than previously considered12,13
  • The cumulative prevalence of IPF increased from 2001 to 2011 and may be explained by the finding by Raghu, et al 2014 that patients aged over 65 years were living longer in 2011 than in previous years12

Prevalence of IPF Among Medicare Patients Age 65 and Older in the United States

Data based on following criteria: ≥1 year continuous coverage of Medicare Part A and Part B without an ICD-9-CM (idiopathic fibrosing alveolitis) diagnosis of 516.3 before the quarter of the first recorded 516.3 diagnosis.

Figure 4. Image adapted from Raghu, et al. Lancet Respir Med. 2014;5:566-572.
  • The incidence of IPF is projected to increase over time as a result of the large increase in the number of people in older age groups (aged 65-84 years) in the United States13

Projected Incidence of IPF in the United States Between 2015 and 2050

Data based on broad case-finding IPF criteria: evidence of usual interstitial pneumonia (UIP) on surgical lung biopsy specimens or definite or possible UIP pattern on HRCT images.

Figure 5. Image adapted from Fernández Pérez, et al. Chest. 2010;137:129-137.


1. Raghu, et al. Am J Respir Crit Care Med. 2011;183:788-824. 2. Cottin and Maher. Eur Respir Rev. 2015;24:58-64. 3. Ley, et al. Am J Respir Crit Care Med. 2011;183:431-440. 4. Meyer. Transl Respir Med. 2014;2:4-17. 5. Mueller-Mang, et al. RadioGraphics. 2007;27:595-615. 6. du Bois. Eur Respir Rev. 2012;21:141-146. 7. American Cancer Society. SEER Cancer Statistics Review, 1975-2012. 8. Benza, et al. Chest. 2012;142:448-456. 9. Nathan, et al. Chest. 2011;140:221-229. 10. Olson, et al. Am J Respir Crit Care Med. 2007;176:277-284. 11. Raghu, et al. Am J Respir Crit Care Med. 2015;192:e3-e19. 12. Raghu, et al. Lancet Respir Med. 2014;2:566-572. 13. Fernández Pérez, et al. Chest. 2010;137:129-137.

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