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Review
. 2018 Nov 29;6(4):110.
doi: 10.3390/medsci6040110.

Idiopathic Pulmonary Fibrosis: Epidemiology, Natural History, Phenotypes

Jaume Sauleda  1   2   3 , Belén Núñez  4   5 , Ernest Sala  6   7   8 , Joan B Soriano  9
Affiliations
Review

Idiopathic Pulmonary Fibrosis: Epidemiology, Natural History, Phenotypes

Jaume Sauleda et al. Med Sci (Basel). .

Abstract

Idiopathic pulmonary fibrosis (IPF) is the most common of the idiopathic interstitial pneumonias. It is characterized by a chronic, progressive, fibrotic interstitial lung disease of unknown cause that occurs primarily in older adults. Its prevalence and incidence have appeared to be increasing over the last decades. Despite its unknown nature, several genetic and environmental factors have been associated with IPF. Moreover, its natural history is variable, but could change depending on the currently suggested phenotypes: rapidly progressive IPF, familial, combined pulmonary fibrosis and emphysema, pulmonary hypertension, and that associated with connective tissue diseases. Early recognition and accurate staging are likely to improve outcomes and induce a prompt initiation of antifibrotics therapy. Treatment is expected to be more effective in the early stages of the disease, while developments in treatment aim to improve the current median survival of 3⁻4 years after diagnosis.

Keywords: comorbidities; evolution; idiopathic pulmonary fibrosis; incidence; prevalence; risk factors.

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Conflict of interest statement

The authors declare no conflicts of interest.

Figures

Figure 1
Figure 1
Worldwide burden of interstitial lung diseases and pulmonary sarcoidosis, measured in disability-adjusted life years (DALYs) per 100,000 in 2016, all ages, both sexes, by country. From https://vizhub.healthdata.org/gbd-compare/ (open web site, accessed on 14 July 2018).
See this image and copyright information in PMC

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