Summary

Idiopathic pulmonary fibrosis (IPF) is a distinctive form of chronic interstitial lung disease occurring primarily in older adults, more often male [Raghu G et al. Am J Respir Crit Care Med 2011]. The estimated prevalence of IPF is 14 to 43 per 100,000 and an estimated incidence is 7 to 16 per 100,000, depending on how it is defined [Raghu G et al. Am J Respir Crit Care Med 2006]. Prognosis can be predicted by the radiographic extent of the disease, amount of fibrosis, presence of pulmonary hypertension, and comorbidities (emphysema, lung cancer, and coronary artery disease).