What is the mortality rate of idiopathic pulmonary fibrosis (IPF)?

Updated: Jul 16, 2021
  • Author: Alaa Abu Sayf, MD; Chief Editor: Guy W Soo Hoo, MD, MPH  more...
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Answer

Idiopathic pulmonary fibrosis (IPF) portends a poor prognosis. With regard to idiopathic pulmonary fibrosis life expectancy, the estimated mean survival is 2-5 years from the time of diagnosis. [2] Estimated mortality rates are 64.3 deaths per million in men and 58.4 deaths per million in women. [28]

Death rates in patients with idiopathic pulmonary fibrosis increase with increasing age, are consistently higher in men than women, and experience seasonal variation, with the highest death rates occurring in the winter, even when infectious causes are excluded. [11]

Estimates are that 60% of patients with idiopathic pulmonary fibrosis die from their idiopathic pulmonary fibrosis, as opposed to dying with their idiopathic pulmonary fibrosis. Of those patients who die with idiopathic pulmonary fibrosis, most commonly it is after an acute exacerbation of idiopathic pulmonary fibrosis. When an acute exacerbation of idiopathic pulmonary fibrosis is not the cause of death, an increased cardiovascular risk and an increased venous thromboembolic disease risk contribute to the cause of death. The most common causes of death in patients with idiopathic pulmonary fibrosis include acute exacerbations of idiopathic pulmonary fibrosis, acute coronary syndromes, congestive heart failure, lung cancer, infectious causes, and venous thromboembolic disease. [2]

A worse prognosis can be expected based on various clinical parameters, physiologic factors, radiographic findings, histopathologic findings, laboratory findings, and bronchoalveolar lavage findings. du Bois et al evaluated a scoring system to predict individual risk of mortality. They used a Cox proportional hazards model and data from two clinical trials (n = 1,099) to identify independent predictors of 1-year mortality among patients with idiopathic pulmonary fibrosis. The findings demonstrated that 4 readily ascertainable predictors (age, history of respiratory hospitalization within the previous 24 weeks, percent predicted FVC, and 24-week change in FVC) could be used in a scoring system to estimate 1-year mortality. However, this scoring system needs to be validated in other populations of patients with idiopathic pulmonary fibrosis. [29]

Ley et al used competing risks regression modeling to retrospectively screen potential predictors of mortality in a derivation cohort of patients with idiopathic pulmonary fibrosis (n = 228). They identified a model consisting of 4 predictors (sex, age, % predicted FVC, and % predicted DLCO). Based on these 4 predictors, they developed a simple point-score model and staging system that was retrospectively validated in a separate cohort of patients with idiopathic pulmonary fibrosis (n = 330). [30]

Table 1. Scoring for mortality risk in IPF. (Open Table in a new window)

 

Predictor

Points

Sex

Female

0

Male

1

Age (years)

≥60

0

61-65

1

>65

2

FVC (% predicted)

>75

0

50-75

1

< 50

2

DLCO (% predicted)

>55

0

36-55

1

≤35

2

Cannot perform

3

 

Table 2. Staging and mortality risk for IPF. (Open Table in a new window)

Stage

I

II

III

Points

0-3

4-5

6-8

Mortality

 

 

 

1-year

5.6

16.2

39.2

2-year

10.9

29.9

62.1

3-year

16.3

42.1

76.8


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