Prevalence and incidence

One of the main limitations of measuring the historical prevalence and incidence of idiopathic pulmonary fibrosis (IPF) is the absence of a uniform, consistent definition of IPF before a consensus statement was published in 2000, and then updated in 2011.1,2

IPF is responsible for 20–50% of all interstitial lung disease (ILD) cases.3

Its prevalence and incidence have appeared to be increasing over the last few decades.3

Incidence studies from 2000 onwards, in Europe and North America, estimate 3–9 cases of IPF per 100,000 per year.4

Prevalence estimates for IPF have varied from 2–29 cases per 100,000 in the general population.2

Key incidence information for patients with idiopathic pulmonary fibrosis

Figure 1. Key incidence information for patients with IPF (Raghu et al., 2011; Ley and Collard, 2013; Sauleda et al., 2018)
*This may be due to sex differences in historical smoking patterns rather than an inherent sex-related risk for IPF

We asked Professor Elisabeth Bendstrup why she thought that primary care physicians must maintain an index of suspicion for a rare disease such as IPF. Find out her response here

Find out about mortality rates in patients with IPF
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