Idiopathic Pulmonary Fibrosis (IPF)

Idiopathic Pulmonary Fibrosis (IPF), a type of Interstitial Lung Disease (ILD) which is characterized by the lining of the lungs becoming thick and stiff with the formation of scarring over time. It is believed that IPF may be caused by an irregular healing process that occurred after an insult to the lung1. Although estimates vary, published data estimate that about 100,000 people in the US live with IPF. The FDA grants Orphan Drug Designation (ODD) status to medicines intended for the treatment, diagnosis or prevention of rare diseases or disorders that affect fewer than 200,000 people in the US2-3.


1. What Is Idiopathic Pulmonary Fibrosis? National Institutes of Health. Accessed 29 March 2022.

2. Lederer, D J and Martinez F J. Idiopathic Pulmonary Fibrosis. N Engl J Med. 2018;378:1811-23.

3. Raghu G, Chen SY, Hou Q, Yeh WS, Collard HR. Incidence and prevalence of idiopathic pulmonary fibrosis in US adults 18-64 years old. Eur Respir J. 2016 Jul;48(1):179-86. doi: 10.1183/13993003.01653-2015. Epub 2016 Apr 28. PMID: 27126689.