Idiopathic Pulmonary Fibrosis

Two antifibrotic medications — nintedanib and pirfenidone — were approved in the fall of 2014 for use in idiopathic pulmonary fibrosis. While these medications are not a cure, they have both been shown to slow the decline of lung funciton over time.

Ongoing studies of other medications for IPF have shown initial promise, but need more research.

Pulmonary rehabilitation is a structured exercise and education program designed to increase endurance, strength, and exercise capacity, as well as to decrease fatigue and shortness of breath, for people with chronic lung disease, including people with IPF.

Additionally, it is important that any other medical problems that are associated with IPF are treated, such as gastroesophageal reflux disease (GERD) and pulmonary hypertension.

Finally, lung transplant may be an effective treatment option for some people with IPF.

Reviewed by health care specialists at UCSF Medical Center.