What is IPF?

Idiopathic pulmonary fibrosis (IPF) is a debilitating and almost uniformly fatal disease. It is characterized anatomically by scarring of the lungs and symptomatically by exertional dyspnea and a dry cough that gradually worsen. Most patients are afflicted with progressive difficulty breathing and shortness of breath. Recent data indicate there may be several distinct disease courses for IPF. About half the patients progress gradually while others appear to experience periods of relative stability interrupted by sudden unpredictable acute exacerbations. In this theory of stepwise IPF progression, survival time from diagnosis is two to five years in patients with IPF.^1,2

Despite recent progress, the diagnosis and management of IPF remain distinct clinical challenges, further complicated by the rapid emergence of new data that challenge conventional wisdom and prompt a reexamination of standard therapeutic approaches. The aim of the PILOT™ initiative is to provide a forum for the open exchange of scientific ideas that results in the timely dissemination of emerging knowledge, facilitates clinical decision making, and improves patient outcomes.

References

1. Martinez FJ. Idiopathic interstitial pneumonias: usual interstitial pneumonia versus nonspecific interstitial pneumonia. Proc Am Thorac Soc. 2006;3:81-95.
2. Martinez FJ, Safrin S, Weycker D, et al. The clinical course of patients with idiopathic pulmonary fibrosis. Ann Intern Med. 2005;142;963-967.