Current and Future Therapies for Idiopathic Pulmonary Fibrosis

Abstract

Idiopathic pulmonary fibrosis (IPF) is a relentless, progressive disease that carries a median survival of 2.8 to 4.5 years from diagnosis. At present there are no licensed therapies for IPF in the United States. This has changed since i wrote the manuscript. However, the last decade has seen a dramatic increase in IPF-related clinical trial activity resulting in the publication of a number of large multicenter studies. These trials have resulted in a number of disappointments, but also 2, at least partial, successes. Both the antioxidant N-acetyl cysteine and the novel antifibrotic compound pirfenidone have been shown to slow disease progression in IPF when compared with placebo. Furthermore, as the natural history of IPF has become better understood, it seems likely that further novel treatment approaches will emerge in the coming few years. Although the key goal of treatment in IPF is to prevent progression of fibroproliferation, it is becoming increasingly clear that treating disease complications such as pulmonary hypertension and palliating symptoms such as dyspnea and cough is vitally important as such approaches improve quality, if not duration, of life for patients with this devastating disease.