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Nat Rev Dis Primers. 2017 Oct 20;3:17074. doi: 10.1038/nrdp.2017.74.

Idiopathic pulmonary fibrosis.

Author information

Joan and Sanford I. Weill Department of Medicine, Weill Cornell Medical College, New York-Presbyterian Hospital/Weill Cornell Medical Center, 1305 York Avenue, Box 96, Room Y-1059, New York, New York 10021, USA.
Department of Internal Medicine, University of California, San Francisco, California, USA.
Facultad de Ciencias, Universidad Nacional Autonoma de Mexico, Mexico City, Mexico.
Department of Internal Medicine, University of Washington, Seattle, Washington, USA.
Unità Operativa Complessa di Pneumologia, Università Cattolica del Sacro Cuore, Rome, Italy.
Unidad de Investigación, Instituto Nacional de Enfermedades Respiratorias Ismael Cosio Villegas, Mexico City, Mexico.
Interstitial Lung Disease Program, National Jewish Health Denver, Colorado, USA.
Department of Respiratory Medicine and Allergy, Tosei General Hospital, Aichi, Japan.
Interstitial Lung Disease Unit, Royal Brompton Hospital, London, UK.


Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive lung disease characterized by progressive lung scarring and the histological picture of usual interstitial pneumonia (UIP). It is associated with increasing cough and dyspnoea and impaired quality of life. IPF affects ∼3 million people worldwide, with incidence increasing dramatically with age. The diagnostic approach includes the exclusion of other interstitial lung diseases or overlapping conditions and depends on the identification of the UIP pattern, usually with high-resolution CT; lung biopsy might be required in some patients. The UIP pattern is predominantly bilateral, peripheral and with a basal distribution of reticular changes associated with traction bronchiectasis and clusters of subpleural cystic airspaces. The biological processes underlying IPF are thought to reflect an aberrant reparative response to repetitive alveolar epithelial injury in a genetically susceptible ageing individual, although many questions remain on how to define susceptibility. Substantial progress has been made in the understanding of the clinical management of IPF, with the availability of two pharmacotherapeutic agents, pirfenidone and nintedanib, that decrease physiological progression and likely improve progression-free survival. Current efforts are directed at identifying IPF early, potentially relying on combinations of biomarkers that include circulating factors, demographics and imaging data.

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