Epidemiology

In Europe and North America, the incidence of idiopathic pulmonary fibrosis (IPF) has been estimated to be between 2.8 and 19 per 100,000 persons per year; incidence in Asia is lower (1.2 to 4.6 per 100,000 persons per year).[6][7][8]

Approximately two-thirds of patients are older than 60 years at diagnosis (mean age at diagnosis is between 60 and 70 years).[1][8]​​​ Among people ages ≥65 years in the US Medicare population, an IPF incidence of 93.7 per 100,000 persons per year has been reported.[9] Prevalence in this population was 494.5 cases per 100,000 persons.[9]

A higher proportion of males than females develop IPF.[8] There is no known ethnic or racial predilection for the development of IPF. The disease is very rare in children.[10]

IPF incidence appears to be increasing with time, even when allowing for aging populations and improved diagnostic sensitivity.[11][12]​​​​ This may be related, in part, to a growing awareness of IPF, particularly among general practitioners, as well as increased CT utilization in older patients.[13][14]

Acute exacerbations

The annual incidence of acute exacerbations without an identifiable cause is estimated to range from 4 to 20 per 100 patient-years.[15] Acute exacerbations are more common in patients with physiologically and functionally advanced disease. One retrospective review found that 35% of IPF patients had at least one episode of rapid deterioration, of whom 55% were considered to have had an acute exacerbation.

Familial pulmonary fibrosis

Present in a subset of patients with IPF. Familial linkage is suggested by an increased prevalence of 10% in first-degree relatives.[16] Although this condition is indistinguishable from the sporadic form, it typically afflicts patients at a younger age (mean age at diagnosis is 55-60 years).[17][18]​​ 

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