Idiopathic Pulmonary Fibrosis

Understanding Idiopathic Pulmonary Fibrosis (IPF)

Idiopathic pulmonary fibrosis (IPF) is a form of progressive pulmonary fibrosis, or abnormal scarring of the lungs. As the scarring progresses, transfer of oxygen into the bloodstream is increasingly impaired, leading to irreversible loss of lung function as well as high morbidity and mortality rates. Average life expectancy is estimated to be three to five years from diagnosis, with approximately two-thirds of patients dying within five years of diagnosis. Survival rates are comparable to those for some of the deadliest cancers. 

​IPF is designated as an orphan disease in the U.S., with U.S. prevalence and incidence of IPF estimated to be ~115,000 and an incidence of 30,000-40,000 new cases per year , respectively. Incidence and mortality are on the rise, and prevalence is expected to increase with the aging population.

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1.Raimundo et al., BMC Pulm Med. (2016); Raghu et al., Eur Respir J (2016); Raghu et al., Lancet Respir Med (2014); Raghu et al., Am J Respir Crit Care Med (2006); Fernandez et al., Chest (2010)

2.Fernández Pérez et al., Chest (2010) 137(1):129-37

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